Materiały źródłowe

• #1 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Surgical resection is the mainstay of therapy for soft tissue sarcomas. […] In some small low-grade tumors of the extremities or trunk, surgery alone can be performed without the use of radiation. […] Patient selection factors may vary among surgeons. In general, surgery alone is considered in patients with low-grade tumors of the extremity or superficial trunk that are 5 cm or smaller in diameter (T1) and have microscopically negative surgical margins. […] When feasible, wide-margin function-sparing surgical excision is the cornerstone of effective treatment for extremity tumors. […] Local control of high-grade soft tissue sarcomas of the trunk and the head and neck can be achieved with surgery in combination with radiation therapy. […] Effective treatment of retroperitoneal sarcomas requires removal of all gross disease while sparing adjacent viscera not invaded by tumor.

• #2 Undifferentiated Pleomorphic Sarcoma: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22435-undifferentiated-pleomorphic-sarcoma

  Your treatment plan is unique to you. Ask your oncologist about your options. You’ll likely have a multidisciplinary team, including a surgeon, a radiation oncologist and a medical oncologist. Each specialist has a specific role, but they all work together to treat you. They can help you determine what’s best in your situation. […] It’s possible to cure UPS, especially during the early stages. There still may be treatment options in later stages, which can prolong your quality of life. Like any type of cancer, early detection and treatment increase your chances for successful outcomes.

• #3 Undifferentiated pleomorphic sarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/undifferentiated-pleomorphic-sarcoma/diagnosis-treatment/drc-20389565

  During intraoperative radiation therapy (IORT) radiation is directed through the surgical incision onto a specific site; in this case a thigh. IORT dosage can be much higher than for standard radiation therapy given from the outside of the body. […] Treatment for undifferentiated pleomorphic sarcoma usually involves surgery to remove the cancer cells. Other options include radiation therapy and drug treatments (systemic therapies), such as chemotherapy, targeted therapy and immunotherapy. Which treatments are best for you will depend on the size and location of your cancer. […] When possible, doctors try to remove the sarcoma completely with surgery. The goal is to remove the cancer and a margin of healthy tissue around it with as minimal an impact as possible. […] Radiation therapy uses high-powered beams of energy, such as X-rays or protons, to kill cancer cells. Radiation therapy can be given as:

• #4 Undifferentiated pleomorphic sarcoma // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/undifferentiated-pleomorphic-sarcoma

  Treatment for undifferentiated pleomorphic sarcoma usually involves surgery to remove the cancer cells. Other options include radiation therapy and drug treatments (systemic therapies), such as chemotherapy, targeted therapy and immunotherapy. Which treatments are best for you will depend on the size and location of your cancer. […] When possible, doctors try to remove the sarcoma completely with surgery. The goal is to remove the cancer and a margin of healthy tissue around it with as minimal an impact as possible. […] Radiation therapy uses high-powered beams of energy, such as X-rays or protons, to kill cancer cells. […] Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. […] Targeted drug treatments focus on specific abnormalities present within cancer cells. […] Immunotherapy uses your immune system to fight cancer.

• #5 Osteosarcoma and UPS of Bone Treatment – NCI

  https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq

  Osteosarcoma and undifferentiated pleomorphic sarcoma (UPS) are treated the same way. […] Treatment options for osteosarcoma and UPS depend on: where the tumor is in the body and if it has spread, the size of the tumor, the grade of the cancer, whether the bones are still growing, your child’s age and overall health, your child’s and family’s goals, such as being able to participate in sports, or concerns about physical appearance, whether the cancer is newly diagnosed or has recurred after treatment. […] There are different types of treatment for children with osteosarcoma or undifferentiated pleomorphic sarcoma (UPS). […] Children with osteosarcoma or UPS should have their treatment planned by a team of health care providers who are experts in treating cancer in children. […] The following types of treatment are used: Surgery, Chemotherapy, Radiation therapy, Samarium.

• #6 Undifferentiated Pleomorphic Sarcoma | Norton Children’s

  https://nortonchildrens.com/services/cancer/conditions/sarcoma/undifferentiated-pleomorphic-sarcoma/

  Undifferentiated pleomorphic sarcoma is a rare cancer that usually forms in soft tissue in the legs, arms or abdomen, but it can form in any soft tissue or bone anywhere in the body. […] Also called malignant fibrous histiocytoma or undifferentiated sarcoma, this aggressive cancer can grow fast and often spreads to other parts of the body, including the lungs. […] The survival rate for undifferentiated pleomorphic sarcoma is 60% at five years and 48% at 10 years, according to a 2019 study. As with all cancers, early detection improves the survival rate. By their nature, long-term survival rates don’t reflect the latest advancements in treatment and may underestimate current survival chances. […] The board-certified and fellowship-trained oncologists at Norton Children’s Cancer Institute come together at regular conferences to share viewpoints from various perspectives that help determine the best course of treatment. It’s like getting second, third and fourth opinions all at once.

• #7

  https://www.orthobullets.com/pathology/8064/undifferentiated-pleomorphic-sarcoma

  Undifferentiated Pleomorphic Sarcoma, previously known as malignant fibrous histiocytoma, is a high-grade, aggressive, malignant fibrogenic tumor. […] Treatment is usually wide-margin surgical excision with radiotherapy. […] Wide surgical resection radiation therapy is the standard of care in most cases. […] Radiation is an important adjunct to surgery decreasing local recurrence. […] Chemotherapy may be administered in some cases. […] Neoadjuvant chemotherapy has shown improved 5-year recurrence-free survival and overall survival in tumors 10 cm. […] 50-60% 5-year survival. […] Amputation is indicated when otherwise unable to resect tumor with negative margins. […] Good prognosis in absence of metastatic disease.

• #8 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Surgical resection is the mainstay of therapy for soft tissue sarcomas. […] In some small low-grade tumors of the extremities or trunk, surgery alone can be performed without the use of radiation. […] Patient selection factors may vary among surgeons. In general, surgery alone is considered in patients with low-grade tumors of the extremity or superficial trunk that are 5 cm or smaller in diameter (T1) and have microscopically negative surgical margins. […] When feasible, wide-margin function-sparing surgical excision is the cornerstone of effective treatment for extremity tumors. […] Local control of high-grade soft tissue sarcomas of the trunk and the head and neck can be achieved with surgery in combination with radiation therapy. […] Effective treatment of retroperitoneal sarcomas requires removal of all gross disease while sparing adjacent viscera not invaded by tumor.

• #9 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  The standard of care for head, neck, trunk, and extremities UPS is en bloc surgical excision with microscopically negative margins, particularly for stage I tumors. This may be achieved by wide local excision with 2 cm margins of uninvolved tissue, albeit some cases may pose greater difficulty due to the involvement of important neurovascular structures. Postoperative radiotherapy (RT) is indicated when margins are close to the tumor ( 1 cm), microscopically positive, involve bone, major blood vessels, or nerves. […] Effective RT should cover a 5 cm margin and can be administered through external beam RT (EBRT) (50 Gy), intraoperative RT (IORT) (10-16 Gy), low-dose brachytherapy (LDR) (45 Gy), or its high-dose equivalent (HDR). […] Broadly, chemotherapy with different antineoplastics is employed for advanced, widespread, or irresectable stages of various STSs.

• #10 Undifferentiated pleomorphic sarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/undifferentiated-pleomorphic-sarcoma/diagnosis-treatment/drc-20389565

  During intraoperative radiation therapy (IORT) radiation is directed through the surgical incision onto a specific site; in this case a thigh. IORT dosage can be much higher than for standard radiation therapy given from the outside of the body. […] Treatment for undifferentiated pleomorphic sarcoma usually involves surgery to remove the cancer cells. Other options include radiation therapy and drug treatments (systemic therapies), such as chemotherapy, targeted therapy and immunotherapy. Which treatments are best for you will depend on the size and location of your cancer. […] When possible, doctors try to remove the sarcoma completely with surgery. The goal is to remove the cancer and a margin of healthy tissue around it with as minimal an impact as possible. […] Radiation therapy uses high-powered beams of energy, such as X-rays or protons, to kill cancer cells. Radiation therapy can be given as:

• #11 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  The standard of care for head, neck, trunk, and extremities UPS is en bloc surgical excision with microscopically negative margins, particularly for stage I tumors. This may be achieved by wide local excision with 2 cm margins of uninvolved tissue, albeit some cases may pose greater difficulty due to the involvement of important neurovascular structures. Postoperative radiotherapy (RT) is indicated when margins are close to the tumor ( 1 cm), microscopically positive, involve bone, major blood vessels, or nerves. […] Effective RT should cover a 5 cm margin and can be administered through external beam RT (EBRT) (50 Gy), intraoperative RT (IORT) (10-16 Gy), low-dose brachytherapy (LDR) (45 Gy), or its high-dose equivalent (HDR). […] Broadly, chemotherapy with different antineoplastics is employed for advanced, widespread, or irresectable stages of various STSs.

• #12 Undifferentiated Pleomorphic Sarcoma – MD Searchlight

  https://mdsearchlight.com/cancer/undifferentiated-pleomorphic-sarcoma/

  The go-to treatment for UPS (Undifferentiated Pleomorphic Sarcoma), a form of cancer, in the head, neck, trunk, and extremities is a type of surgery called en bloc excision, particularly for stage I tumors. In simpler terms, this surgery involves removing the tumor along with some surrounding healthy tissue to ensure no cancer cells are left behind. This method is usually achieved by removing the tumor with a 2 cm margin of healthy tissue. In some cases, this can be challenging due to the presence of important nerves and blood vessels near the tumor. […] After surgery, radiotherapy, which is the use of high-energy rays to destroy cancer cells, is recommended if the tumor is very close to the healthy tissue margin, if any cancer cells are found in the margins, or if the tumor involves important structures like bones, major blood vessels, or nerves. Radiotherapy, which covers a 5 cm margin, can be given through different methods, like the use of an external device (external beam RT), during surgery (intraoperative RT) or by placing radioactive materials inside the body (brachytherapy).

• #13 Undifferentiated pleomorphic sarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/undifferentiated-pleomorphic-sarcoma-1?lang=us

  Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma (MFH), is considered the most common type of soft tissue sarcoma. It has an aggressive biological behavior and a poor prognosis. […] Most undifferentiated pleomorphic sarcomas are high grade (3 and 4) and are aggressive in their biological behavior. They frequently metastasize (30-50% at diagnosis) and locally recur despite aggressive treatment. The overall 5-year survival is between 25-70%. […] Treatment usually consists of aggressive en bloc resection with a wide margin. Supplementary neoadjuvant chemotherapy and radiotherapy is especially useful in reducing the local recurrence rate. Limb-sparing surgery is usually possible.

• #14 Undifferentiated Pleomorphic Sarcoma: Causes, Symptoms and Treatment | Apollo Hospitals

  https://www.apollohospitals.com/diseases-and-conditions/undifferentiated-pleomorphic-sarcoma-causes-symptoms-and-treatment/

  Undifferentiated pleomorphic sarcoma was previously also known as malignant fibrous histiocytoma. This type of sarcoma can be extremely aggressive and may require immediate treatment to stop it from growing. […] Your doctor will select your treatment option based on the location and the size of the lump. The following options are available for the treatment of undifferentiated pleomorphic sarcoma: […] Surgery depends on the location of the sarcoma. If the sarcoma is in the arms or legs, the doctor will completely remove the sarcoma and a part of the healthy tissue to stop it from spreading. However, if cancer has spread widely, surgeons may have to amputate your arm or leg. Radiation therapy or chemotherapy may be performed before the surgery to shrink cancer and avoid amputations. […] If the sarcoma is spreading, your doctor may also subject you to chemotherapy. In chemotherapy, your doctor will prescribe various drugs to treat your cancer. These drugs work by killing the fast-growing cells to prevent the formation of sarcoma or tumors. Chemotherapy for undifferentiated pleomorphic sarcoma is usually prescribed if it recurs after the initial treatment.

• #15 Undifferentiated Pleomorph – Types of Sarcoma Cancer

  https://www.leiomyosarcoma.org/undifferentiated-pleomorph/

  Like most sarcomas, the best way to treat undifferentiated pleomorphic sarcoma is to remove the tumor through surgery. If there are remaining cancer cells in the body, then radiation or chemotherapy is generally used to address it. […] In some cases, though, the tumor may have spread to the point that surgery isn’t an option. As undifferentiated pleomorphic sarcoma usually affects the limbs, surgeons will do their best to remove the tumor while leaving as much of the arm or leg intact. When this isn’t possible, though, it may become necessary to actually amputate the limb, especially if the sarcoma has spread and become bone cancer. […] The good news is that there is a high survival rate for those whose undifferentiated pleomorphic sarcomas are caught and addressed early. Once the cancer has advanced, there’s usually a five-year survival rate.

• #16 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  The standard of care for head, neck, trunk, and extremities UPS is en bloc surgical excision with microscopically negative margins, particularly for stage I tumors. This may be achieved by wide local excision with 2 cm margins of uninvolved tissue, albeit some cases may pose greater difficulty due to the involvement of important neurovascular structures. Postoperative radiotherapy (RT) is indicated when margins are close to the tumor ( 1 cm), microscopically positive, involve bone, major blood vessels, or nerves. […] Effective RT should cover a 5 cm margin and can be administered through external beam RT (EBRT) (50 Gy), intraoperative RT (IORT) (10-16 Gy), low-dose brachytherapy (LDR) (45 Gy), or its high-dose equivalent (HDR). […] Broadly, chemotherapy with different antineoplastics is employed for advanced, widespread, or irresectable stages of various STSs.

• #17 Management of undifferentiated high‐grade pleomorphic sarcoma of parotid region in elderly women

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6406222/

  Wide resection with free margins is required, but a complete excision of head and neck localizations can be challenging due to the infiltrative nature and consequent invasion to critical structures. […] Adjuvant radiation therapy is generally recommended if margins are positive or close, if the lesion is highgrade, in large tumors (more than 5 cm of diameter), or in the presence of metastatic lymph nodes. […] The use of chemotherapy and/or radiotherapy as the primary treatment modality has been less successful; chemoresistance is not uncommon, and at present, there is no specific trial showing significant improvement of survival after adjuvant systemic chemotherapy. […] Overall survival rates for UHPS range from 30% to 74%. Positive or close surgical margins, after excision, correlate with an increased local recurrence rate and reduce overall survival.

• #18 A systematic review of undifferentiated pleomorphic sarcoma of the chest wall – Bennett – Chinese Clinical Oncology

  https://cco.amegroups.org/article/view/119459/html

  Surgical resection is the primary mode of treatment and is often supplemented with radiotherapy and chemotherapy. […] Prognosis is worse in patients with deeper and larger tumors of the chest wall. […] Future research needs to elucidate the necessity of other therapies in the context of margin distance, tumor size, and tumor location. […] The National Cancer Comprehensive indicates that neoadjuvant RT is more effective than its counterpart in treating UPS in the trunk. […] Neoadjuvant or adjuvant chemotherapy and RT, though debated, are often used to supplement surgical resection of UPS. […] Inadequacy of margins with large tumors are often secondary to proximity to deeper, vital structures located within the thoracic cavity and mediastinum. […] It is important to note our review demonstrates characteristics of a limited number of patients.

• #19 Management of undifferentiated high‐grade pleomorphic sarcoma of parotid region in elderly women

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6406222/

  Wide resection with free margins is required, but a complete excision of head and neck localizations can be challenging due to the infiltrative nature and consequent invasion to critical structures. […] Adjuvant radiation therapy is generally recommended if margins are positive or close, if the lesion is highgrade, in large tumors (more than 5 cm of diameter), or in the presence of metastatic lymph nodes. […] The use of chemotherapy and/or radiotherapy as the primary treatment modality has been less successful; chemoresistance is not uncommon, and at present, there is no specific trial showing significant improvement of survival after adjuvant systemic chemotherapy. […] Overall survival rates for UHPS range from 30% to 74%. Positive or close surgical margins, after excision, correlate with an increased local recurrence rate and reduce overall survival.

• #20 Management of undifferentiated high‐grade pleomorphic sarcoma of parotid region in elderly women

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6406222/

  Wide resection with free margins is required, but a complete excision of head and neck localizations can be challenging due to the infiltrative nature and consequent invasion to critical structures. […] Adjuvant radiation therapy is generally recommended if margins are positive or close, if the lesion is highgrade, in large tumors (more than 5 cm of diameter), or in the presence of metastatic lymph nodes. […] The use of chemotherapy and/or radiotherapy as the primary treatment modality has been less successful; chemoresistance is not uncommon, and at present, there is no specific trial showing significant improvement of survival after adjuvant systemic chemotherapy. […] Overall survival rates for UHPS range from 30% to 74%. Positive or close surgical margins, after excision, correlate with an increased local recurrence rate and reduce overall survival.

• #21 Management of undifferentiated high‐grade pleomorphic sarcoma of parotid region in elderly women

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6406222/

  No specific guidelines for the management of this tumor have been established, but surgical excision with free margins associated with neck dissection in advanced cases in a multimodality regimen that currently represents the best chance of disease control. Postoperative radiotherapy improves local control, although neoadjuvant chemotherapy has been less investigated because it is limited by systemic toxicity. Free flap reconstruction makes more extensive resection possible and may improve local tumor control, while providing acceptable cosmetic and functional results.

• #22 Undifferentiated pleomorphic sarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/undifferentiated-pleomorphic-sarcoma/diagnosis-treatment/drc-20389565

  During intraoperative radiation therapy (IORT) radiation is directed through the surgical incision onto a specific site; in this case a thigh. IORT dosage can be much higher than for standard radiation therapy given from the outside of the body. […] Treatment for undifferentiated pleomorphic sarcoma usually involves surgery to remove the cancer cells. Other options include radiation therapy and drug treatments (systemic therapies), such as chemotherapy, targeted therapy and immunotherapy. Which treatments are best for you will depend on the size and location of your cancer. […] When possible, doctors try to remove the sarcoma completely with surgery. The goal is to remove the cancer and a margin of healthy tissue around it with as minimal an impact as possible. […] Radiation therapy uses high-powered beams of energy, such as X-rays or protons, to kill cancer cells. Radiation therapy can be given as:

• #23 Undifferentiated Pleomorphic Sarcoma: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22435-undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma treatment depends on the size of the tumor and whether the cancer has spread. The main treatment for early-stage UPS is typically surgery to remove the growth, most often combined with radiation therapy. But you might need additional cancer treatments, too. […] Surgery for undifferentiated pleomorphic sarcoma involves removing the tumor. The goal is to remove the entire sarcoma and a little bit of the healthy tissue around it (known as the margin). Your surgeon does this to improve the chances of removing all the cancer cells. If cancer cells are at the edge of the removed tumor, there’s a risk that some may be left in your body as well. […] Oncologists may also use additional treatments before or after surgery. These treatments rarely shrink the tumor, but they can help make the tumors safer to remove at surgery or treat any remaining cancer cells after surgery: Chemotherapy, Immunotherapy, Radiation therapy.

• #24 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  The standard of care for head, neck, trunk, and extremities UPS is en bloc surgical excision with microscopically negative margins, particularly for stage I tumors. This may be achieved by wide local excision with 2 cm margins of uninvolved tissue, albeit some cases may pose greater difficulty due to the involvement of important neurovascular structures. Postoperative radiotherapy (RT) is indicated when margins are close to the tumor ( 1 cm), microscopically positive, involve bone, major blood vessels, or nerves. […] Effective RT should cover a 5 cm margin and can be administered through external beam RT (EBRT) (50 Gy), intraoperative RT (IORT) (10-16 Gy), low-dose brachytherapy (LDR) (45 Gy), or its high-dose equivalent (HDR). […] Broadly, chemotherapy with different antineoplastics is employed for advanced, widespread, or irresectable stages of various STSs.

• #25 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  The standard of care for head, neck, trunk, and extremities UPS is en bloc surgical excision with microscopically negative margins, particularly for stage I tumors. This may be achieved by wide local excision with 2 cm margins of uninvolved tissue, albeit some cases may pose greater difficulty due to the involvement of important neurovascular structures. Postoperative radiotherapy (RT) is indicated when margins are close to the tumor ( 1 cm), microscopically positive, involve bone, major blood vessels, or nerves. […] Effective RT should cover a 5 cm margin and can be administered through external beam RT (EBRT) (50 Gy), intraoperative RT (IORT) (10-16 Gy), low-dose brachytherapy (LDR) (45 Gy), or its high-dose equivalent (HDR). […] Broadly, chemotherapy with different antineoplastics is employed for advanced, widespread, or irresectable stages of various STSs.

• #26 Management of undifferentiated high‐grade pleomorphic sarcoma of parotid region in elderly women

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6406222/

  Wide resection with free margins is required, but a complete excision of head and neck localizations can be challenging due to the infiltrative nature and consequent invasion to critical structures. […] Adjuvant radiation therapy is generally recommended if margins are positive or close, if the lesion is highgrade, in large tumors (more than 5 cm of diameter), or in the presence of metastatic lymph nodes. […] The use of chemotherapy and/or radiotherapy as the primary treatment modality has been less successful; chemoresistance is not uncommon, and at present, there is no specific trial showing significant improvement of survival after adjuvant systemic chemotherapy. […] Overall survival rates for UHPS range from 30% to 74%. Positive or close surgical margins, after excision, correlate with an increased local recurrence rate and reduce overall survival.

• #27 Management of undifferentiated high‐grade pleomorphic sarcoma of parotid region in elderly women

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6406222/

  Wide resection with free margins is required, but a complete excision of head and neck localizations can be challenging due to the infiltrative nature and consequent invasion to critical structures. […] Adjuvant radiation therapy is generally recommended if margins are positive or close, if the lesion is highgrade, in large tumors (more than 5 cm of diameter), or in the presence of metastatic lymph nodes. […] The use of chemotherapy and/or radiotherapy as the primary treatment modality has been less successful; chemoresistance is not uncommon, and at present, there is no specific trial showing significant improvement of survival after adjuvant systemic chemotherapy. […] Overall survival rates for UHPS range from 30% to 74%. Positive or close surgical margins, after excision, correlate with an increased local recurrence rate and reduce overall survival.

• #28 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  The standard of care for head, neck, trunk, and extremities UPS is en bloc surgical excision with microscopically negative margins, particularly for stage I tumors. This may be achieved by wide local excision with 2 cm margins of uninvolved tissue, albeit some cases may pose greater difficulty due to the involvement of important neurovascular structures. Postoperative radiotherapy (RT) is indicated when margins are close to the tumor ( 1 cm), microscopically positive, involve bone, major blood vessels, or nerves. […] Effective RT should cover a 5 cm margin and can be administered through external beam RT (EBRT) (50 Gy), intraoperative RT (IORT) (10-16 Gy), low-dose brachytherapy (LDR) (45 Gy), or its high-dose equivalent (HDR). […] Broadly, chemotherapy with different antineoplastics is employed for advanced, widespread, or irresectable stages of various STSs.

• #29 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  The standard of care for head, neck, trunk, and extremities UPS is en bloc surgical excision with microscopically negative margins, particularly for stage I tumors. This may be achieved by wide local excision with 2 cm margins of uninvolved tissue, albeit some cases may pose greater difficulty due to the involvement of important neurovascular structures. Postoperative radiotherapy (RT) is indicated when margins are close to the tumor ( 1 cm), microscopically positive, involve bone, major blood vessels, or nerves. […] Effective RT should cover a 5 cm margin and can be administered through external beam RT (EBRT) (50 Gy), intraoperative RT (IORT) (10-16 Gy), low-dose brachytherapy (LDR) (45 Gy), or its high-dose equivalent (HDR). […] Broadly, chemotherapy with different antineoplastics is employed for advanced, widespread, or irresectable stages of various STSs.

• #30 Undifferentiated pleomorphic sarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/undifferentiated-pleomorphic-sarcoma/diagnosis-treatment/drc-20389565

  During intraoperative radiation therapy (IORT) radiation is directed through the surgical incision onto a specific site; in this case a thigh. IORT dosage can be much higher than for standard radiation therapy given from the outside of the body. […] Treatment for undifferentiated pleomorphic sarcoma usually involves surgery to remove the cancer cells. Other options include radiation therapy and drug treatments (systemic therapies), such as chemotherapy, targeted therapy and immunotherapy. Which treatments are best for you will depend on the size and location of your cancer. […] When possible, doctors try to remove the sarcoma completely with surgery. The goal is to remove the cancer and a margin of healthy tissue around it with as minimal an impact as possible. […] Radiation therapy uses high-powered beams of energy, such as X-rays or protons, to kill cancer cells. Radiation therapy can be given as:

• #31 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  The standard of care for head, neck, trunk, and extremities UPS is en bloc surgical excision with microscopically negative margins, particularly for stage I tumors. This may be achieved by wide local excision with 2 cm margins of uninvolved tissue, albeit some cases may pose greater difficulty due to the involvement of important neurovascular structures. Postoperative radiotherapy (RT) is indicated when margins are close to the tumor ( 1 cm), microscopically positive, involve bone, major blood vessels, or nerves. […] Effective RT should cover a 5 cm margin and can be administered through external beam RT (EBRT) (50 Gy), intraoperative RT (IORT) (10-16 Gy), low-dose brachytherapy (LDR) (45 Gy), or its high-dose equivalent (HDR). […] Broadly, chemotherapy with different antineoplastics is employed for advanced, widespread, or irresectable stages of various STSs.

• #32 Undifferentiated pleomorphic sarcoma – VALINTERMED treatment in Valencia

  https://valintermed.com/en/medlibrary/undifferentiated-pleomorphic-sarcoma/

  Treatment of undifferentiated pleomorphic sarcoma is based on the extent of tumor spread and the overall clinical picture. It may include: […] General treatment: Chemotherapy and radiotherapy may be used as adjuvant therapy after surgery. […] Pharmacological treatment: In some cases, the range of use of immunotherapy or targeted therapy may be discussed depending on the molecular abnormalities. […] Surgical treatment: Surgery to remove the tumor is the standard of treatment, often requiring radical intervention to achieve complete resection. […] Other treatments: Innovative approaches such as localized chemotherapy or the use of stereotactic radiotherapy may be considered in cases of recurrence. […] How is undifferentiated pleomorphic sarcoma treated? Treatment may include surgery, chemotherapy, and radiation therapy, depending on the stage and location of the tumor.

• #33 Undifferentiated pleomorphic sarcoma – Care at Mayo Clinic – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/undifferentiated-pleomorphic-sarcoma/care-at-mayo-clinic/mac-20389586

  Mayo Clinic doctors will work with you to review all of your treatment options and choose the treatment that best suits your needs and goals. […] The range of possible treatments for people with undifferentiated pleomorphic sarcoma includes several types of surgery including minimally invasive surgery and limb-sparing surgery as well as the latest radiation therapy techniques, including intensity-modulated radiation therapy, proton beam therapy, brachytherapy and intraoperative radiation therapy (IORT). […] A full range of systemic therapies, including chemotherapy and biological therapy also are available.

• #34 Undifferentiated Pleomorphic Sarcoma: Causes, Symptoms and Treatment | Apollo Hospitals

  https://www.apollohospitals.com/diseases-and-conditions/undifferentiated-pleomorphic-sarcoma-causes-symptoms-and-treatment/

  Undifferentiated pleomorphic sarcoma was previously also known as malignant fibrous histiocytoma. This type of sarcoma can be extremely aggressive and may require immediate treatment to stop it from growing. […] Your doctor will select your treatment option based on the location and the size of the lump. The following options are available for the treatment of undifferentiated pleomorphic sarcoma: […] Surgery depends on the location of the sarcoma. If the sarcoma is in the arms or legs, the doctor will completely remove the sarcoma and a part of the healthy tissue to stop it from spreading. However, if cancer has spread widely, surgeons may have to amputate your arm or leg. Radiation therapy or chemotherapy may be performed before the surgery to shrink cancer and avoid amputations. […] If the sarcoma is spreading, your doctor may also subject you to chemotherapy. In chemotherapy, your doctor will prescribe various drugs to treat your cancer. These drugs work by killing the fast-growing cells to prevent the formation of sarcoma or tumors. Chemotherapy for undifferentiated pleomorphic sarcoma is usually prescribed if it recurs after the initial treatment.

• #35

  https://www.sydneysarcomaunit.com.au/undifferentiated-pleomorphic-sarcoma

  Surgery […] Doctors try to surgically remove the sarcoma completely whenever possible. The goal is to remove the tumour as well as a margin of healthy tissue around it with the least amount of damage possible. […] Before surgery, various therapies such as radiation therapy and chemotherapy may be used to enable surgical removal of the tumour without amputating the affected limb. […] Radiation therapy uses high-powered beams of energy, such as X-rays or protons, to kill cancer cells. The use of radiotherapy for these tumours is common. […] Radiotherapy (either before or after surgery) is useful in the management of sarcomas. It has been shown to reduce the chance of the tumour re-occurring locally after surgical resection. […] Chemotherapy is a medicinal treatment that kills cancer cells by using chemicals. It can be taken as a pill, injected into a vein (intravenously), or both.

• #36 Undifferentiated Pleomorphic Sarcoma | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/undifferentiated-pleomorphic-sarcoma/

  Surgery is the first treatment method for UPS. The surgeon will remove the tumour and will aim to take out an area of normal tissue too this is known as taking a margin. This is to make sure that all of the cancer has been removed. […] This treatment uses high-energy radiation to destroy cancer cells. It can be used either before or after surgery. When used before surgery, radiotherapy aims to make the tumour smaller so that it can be more easily removed. When used after surgery, radiotherapy aims to kill off any remaining cancer cells that have not been removed by surgery. […] This treatment uses anti-cancer drugs to destroy cancer cells. It is sometimes used in patients that have a high risk of the cancer coming back or if it has spread to other parts of the body, also known as metastasis.

• #37 Undifferentiated pleomorphic sarcoma of Buttock – Macmillan Online Community

  https://community.macmillan.org.uk/cancer_types/soft-tissue-sarcomas-forum/f/diagnosis-and-treatment/258860/undifferentiated-pleomorphic-sarcoma-of-buttock

  my husband is 66 and in June last year he said he had a bit of an ache in top half of his leg – said he felt like hed been sitting on a coach seat for too long. […] Surgeon told us that she would have preferred to do radiotherapy before surgery but team thought it needed removing asap. […] He has now just finished 6 weeks of radiotherapy post surgery. […] We have already been told because of several factors grade, size and aggressive nature of the sarcoma there is a strong possibility of recurrence and/either mestatase within 2 years. […] I too had 6 weeks radiotherapy […] The hospital because of the type your husband had will keep close eye on him because of its rarity and how aggressive they can be. I’m glad he has finished his treatment and is on the road to recovery.

• #38 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  In the setting of distant metastasis, surgery may be associated with long-term disease-free survival in patients with pulmonary metastasis and optimal underlying disease biology. […] Complete staging and treatment planning by a multidisciplinary team of cancer specialists is required to determine the optimal treatment for patients with soft tissue sarcoma. […] The role of chemotherapy is not well defined. […] There is evidence that favorable clinical outcomes may be associated with referral to a specialized sarcoma treatment center. […] Chemotherapy remains controversial, and its potential benefits should be considered in the context of the short- and long-term toxicities of the chemotherapy regimen. […] The role of adjuvant chemotherapy remains controversial. […] Neoadjuvant chemotherapy with or without radiation therapy has shown response rates of 17% to 32%, 10-year RFS rates of up to 58%, and 10-year OS rates of up to 64%.

• #39 Undifferentiated pleomorphic sarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Undifferentiated_pleomorphic_sarcoma

  In place or combined with surgery and/or radiotherapy, severe and/or metastatic cases of UPS are commonly treated with epirubicin plus ifosfamide; doxorubicin alone or combined with ifosfamide, olaratumab, trabectedin, gemcitabine, or docetaxel; cyclophosphamide, vincristine, doxorubicin, plus dacarbazine or cisplatin; cyclophosphamide, doxorubicin, plus dacarbazine; high dose methotrexate; or etoposide, ifosfamide, and cisplatin. […] However, other studies report that the addition of radiotherapy and/or chemotherapy to surgical resection does not improve recurrence or overall survival rates; addition of radiotherapy to surgery improves local control of UPS tumors but not disease-free survival rates (i.e. time from treatment to recurrence of disease); and adjuvant chemotherapy and radiotherapy have no significant effects on local recurrence-free survival rates, metastasis-free survival times, and overall survival rates.

• #40 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Anthracyclines remain the first-line class of systemic therapy in managing most locally advanced and metastatic soft tissue sarcoma. […] The combination of gemcitabine and docetaxel is used as second-line therapy in treating patients with soft tissue sarcoma. […] Immune checkpoint inhibitors have shown some activity in selected soft tissue sarcoma subtypes, but their use cannot be routinely recommended. […] Treatment options for recurrent soft tissue sarcoma include surgery with or without radiation therapy, chemotherapy and targeted therapy, and immune checkpoint inhibitor therapy.

• #41 A case of undifferentiated pleomorphic sarcoma in esophagus after multiple cancer treatments of surgery and chemoradiotherapy | Surgical Case Reports | Full Text

  https://surgicalcasereports.springeropen.com/articles/10.1186/s40792-022-01560-0

  Undifferentiated pleomorphic sarcoma (UPS) in the esophagus is extremely rare. Therefore, there are few reports of UPS in the esophagus (UPSE). We present a case of UPSE after multiple cancer treatments. […] Currently, complete resection is the only treatment option for UPSE. An optimal treatment strategy using chemotherapy or radiotherapy should be established. […] The standard treatment for UPS is complete resection, and a wide margin is required, if feasible. […] However, in the case of intra-abdominal or gastrointestinal origin tumor, wide margins may be difficult to achieve to preserve organ’s function, and NCCN guidelines also suggest that complete resection with negative margins is necessary. […] Chemotherapy for soft tissue sarcoma can be considered when the tumor is advanced or unresectable.

• #42

  https://www.sydneysarcomaunit.com.au/undifferentiated-pleomorphic-sarcoma

  Chemotherapy is sometimes given before surgery to shrink cancer and make it easier to remove during surgery. Chemotherapy is also used after surgery to improve cancer survival. […] Chemotherapy and radiation can be used together. […] Targeted chemotherapy treatments are designed to target specific abnormalities found in cancer cells. Targeted medication treatments can kill cancer cells by blocking these abnormalities. […] Immunotherapy is a cancer treatment that makes use of your immune system. […] Immunotherapy is often reserved for patients with advanced cancer.

• #43 Undifferentiated pleomorphic sarcomas of the proximal femur, A case study

  https://meddocsonline.org/journal-of-case-reports-and-medical-images/Undifferentiated-pleomorphic-sarcomas-of-the-proximal-femur-A-case-study.html

  Pleomorphic sarcomas are a subtype of soft tissue sarcomas occurring most commonly in the proximal thigh, with a male preponderance, common in 7th and 8th decades of life. […] Though the grade, and level of local invasion, metastasis, and post surgical margins guide the tumor management, treatment modality is basically subdivided into surgery chemotherapy and radiation therapy. Risk of recurrence is present as with any tumor, so regular follow up, and evaluations are recommended. […] The treatment modalities are three fold, as in all cases of a bone malignancies, consist of excision, with wide margins the rest of treatment including chemotherapy and radiation therapy depends on the grade of the tumour, local invasion and metastasis. Lymph node involvement must be carefully assessed, not only for dissection but also to make sure no spread has occurred. Recurrence occurs in about half of the patients, common within the first 2 years of treatment. Increased risk of recurrence includes factors such -a mass size greater than 5 cm, a high-grade histology, a deep anatomical location of the tumour, poor resection of the original tumour. […] Even after appropriate treatment, the risk of recurrence is high, especially within a period of 2 years; therefore, regular follow-ups with imaging such as an MRI are mandatory.

• #44 A primary undifferentiated pleomorphic sarcoma of the lumbosacral region harboring a LMNA-NTRK1 gene fusion with durable clinical response to crizotinib: a case report | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4749-z

  Perioperative chemotherapy was recently reported to be beneficial in terms of overall survival, and doxorubicin as a single agent or in combination with ifosfamide is the first choice of chemotherapy in cases of UPS metastasis. […] The observed effectiveness of crizotinib treatment provides clinical validation of this molecular target. […] Molecularly targeted therapy of these rare but aggressive lesions represents a novel treatment option that may lead to fewer toxic side effects and better clinical outcomes. […] Crizotinib is a multi-active kinase inhibitor that blocks TrkA autophosphorylation and cell growth in cells expressing NTRK1 fusion proteins. […] Based on the report of a minor response to crizotinib in a case of non-small cell lung cancer harboring a NTRK1 fusion as well as preclinical data, we started oral administration of crizotinib (450 mg QD) in the UPS patient described in this report. Over the follow-up period, the patient did not experience intolerable adverse effects from treatment and continued crizotinib monotherapy with no evidence of disease for more than 18 months as of July 2018.

• #45 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Anthracyclines remain the first-line class of systemic therapy in managing most locally advanced and metastatic soft tissue sarcoma. […] The combination of gemcitabine and docetaxel is used as second-line therapy in treating patients with soft tissue sarcoma. […] Immune checkpoint inhibitors have shown some activity in selected soft tissue sarcoma subtypes, but their use cannot be routinely recommended. […] Treatment options for recurrent soft tissue sarcoma include surgery with or without radiation therapy, chemotherapy and targeted therapy, and immune checkpoint inhibitor therapy.

• #46 Treatment of High-Grade Undifferentiated Pleomorphic Sarcoma in the Retroperitoneum

  https://www.targetedonc.com/view/treatment-of-high-grade-undifferentiated-pleomorphic-sarcoma-in-the-retroperitoneum

  The prognosis for sarcomas really depends on the treatment modalities that can be used and the treatment modalities that are used. […] The standard treatment options for patients who present with unresectable soft tissue sarcomas is to use chemotherapeutic agents. For the first-line therapy, one would use a doxorubicin-based therapy and, preferably, probably in this situation, one would use the addition of olaratumab. […] If we look historically at the data, there are other combinations that can be utilized, including doxorubicin with ifosfamide or single-agent doxorubicin.

• #47 Treatment of High-Grade Undifferentiated Pleomorphic Sarcoma in the Retroperitoneum

  https://www.targetedonc.com/view/treatment-of-high-grade-undifferentiated-pleomorphic-sarcoma-in-the-retroperitoneum

  The prognosis for sarcomas really depends on the treatment modalities that can be used and the treatment modalities that are used. […] The standard treatment options for patients who present with unresectable soft tissue sarcomas is to use chemotherapeutic agents. For the first-line therapy, one would use a doxorubicin-based therapy and, preferably, probably in this situation, one would use the addition of olaratumab. […] If we look historically at the data, there are other combinations that can be utilized, including doxorubicin with ifosfamide or single-agent doxorubicin.

• #48 Undifferentiated pleomorphic sarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Undifferentiated_pleomorphic_sarcoma

  In place or combined with surgery and/or radiotherapy, severe and/or metastatic cases of UPS are commonly treated with epirubicin plus ifosfamide; doxorubicin alone or combined with ifosfamide, olaratumab, trabectedin, gemcitabine, or docetaxel; cyclophosphamide, vincristine, doxorubicin, plus dacarbazine or cisplatin; cyclophosphamide, doxorubicin, plus dacarbazine; high dose methotrexate; or etoposide, ifosfamide, and cisplatin. […] However, other studies report that the addition of radiotherapy and/or chemotherapy to surgical resection does not improve recurrence or overall survival rates; addition of radiotherapy to surgery improves local control of UPS tumors but not disease-free survival rates (i.e. time from treatment to recurrence of disease); and adjuvant chemotherapy and radiotherapy have no significant effects on local recurrence-free survival rates, metastasis-free survival times, and overall survival rates.

• #49 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Anthracyclines remain the first-line class of systemic therapy in managing most locally advanced and metastatic soft tissue sarcoma. […] The combination of gemcitabine and docetaxel is used as second-line therapy in treating patients with soft tissue sarcoma. […] Immune checkpoint inhibitors have shown some activity in selected soft tissue sarcoma subtypes, but their use cannot be routinely recommended. […] Treatment options for recurrent soft tissue sarcoma include surgery with or without radiation therapy, chemotherapy and targeted therapy, and immune checkpoint inhibitor therapy.

• #50 Undifferentiated Pleomorphic Sarcoma — Cancer Survivors Network

  https://csn.cancer.org/discussion/325162/undifferentiated-pleomorphic-sarcoma

  My husband was diagnosed in early 2021. He began Gemcitabine and Docitaxel. He has had ten rounds. […] I know we need to ask our oncologist whether the side effects from treatment are worth the few months that my husband’s life could be extended. […] The only thing we can do is go back to chemo. […] Because I’ve had so much chemo, I could not do any more chemotherapy. So my doctors in New York recommended I do immunotherapy. I’ve done a year of Keytruda. So far I have no tumors. […] If the chemo is not working for your husband, look into a place that’s doing immunotherapy; the side effects are nothing like the chemo.

• #51 Undifferentiated pleomorphic sarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Undifferentiated_pleomorphic_sarcoma

  In place or combined with surgery and/or radiotherapy, severe and/or metastatic cases of UPS are commonly treated with epirubicin plus ifosfamide; doxorubicin alone or combined with ifosfamide, olaratumab, trabectedin, gemcitabine, or docetaxel; cyclophosphamide, vincristine, doxorubicin, plus dacarbazine or cisplatin; cyclophosphamide, doxorubicin, plus dacarbazine; high dose methotrexate; or etoposide, ifosfamide, and cisplatin. […] However, other studies report that the addition of radiotherapy and/or chemotherapy to surgical resection does not improve recurrence or overall survival rates; addition of radiotherapy to surgery improves local control of UPS tumors but not disease-free survival rates (i.e. time from treatment to recurrence of disease); and adjuvant chemotherapy and radiotherapy have no significant effects on local recurrence-free survival rates, metastasis-free survival times, and overall survival rates.

• #52 Reddit – The heart of the internet

  https://www.reddit.com/r/sarcoma/comments/1ei9spu/trabectedin_as_a_chemo_therapy_for/

  My father, 62(M), has metastatic undifferentiated pleomorphic sarcoma (UPS) in his lungs. […] His oncologist has recommended using Trabectedin as chemotherapy. The drug will be administered for 24 hours I think every 21 days. […] He is scheduled to start chemotherapy in 2-3 weeks.

• #53 Successful Treatment via Chemotherapy for Undifferentiated Pleomorphic Sarcoma of Back with Pulmonary Metastasis: A Case Report

  https://www.jstage.jst.go.jp/article/nishiseisai/60/4/60_4_661/_article/-char/en

  Successful Treatment via Chemotherapy for Undifferentiated Pleomorphic Sarcoma of Back with Pulmonary Metastasis: A Case Report […] Herein, we present a 61-year-old male with undifferentiated pleomorphic sarcoma, so-called malignant fibrous hisiocytoma (MFH) of the back. Local recurrence and multiple pulmonary metastases developed two months after definitive surgery. Local and distant lesions responded completely to chemotherapy consisting of mesna, doxorubicin, ifosfamide, and dacarbazine (MAID). Radiation therapy at a dose of 60 Gy was added to the site of local recurrence. The patient’s conditions improved and no recurrence was seen even five months after completion of treatment.

• #54 Undifferentiated pleomorphic sarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Undifferentiated_pleomorphic_sarcoma

  In place or combined with surgery and/or radiotherapy, severe and/or metastatic cases of UPS are commonly treated with epirubicin plus ifosfamide; doxorubicin alone or combined with ifosfamide, olaratumab, trabectedin, gemcitabine, or docetaxel; cyclophosphamide, vincristine, doxorubicin, plus dacarbazine or cisplatin; cyclophosphamide, doxorubicin, plus dacarbazine; high dose methotrexate; or etoposide, ifosfamide, and cisplatin. […] However, other studies report that the addition of radiotherapy and/or chemotherapy to surgical resection does not improve recurrence or overall survival rates; addition of radiotherapy to surgery improves local control of UPS tumors but not disease-free survival rates (i.e. time from treatment to recurrence of disease); and adjuvant chemotherapy and radiotherapy have no significant effects on local recurrence-free survival rates, metastasis-free survival times, and overall survival rates.

• #55 Undifferentiated Pleomorphic Sarcoma: Causes, Symptoms and Treatment | Apollo Hospitals

  https://www.apollohospitals.com/diseases-and-conditions/undifferentiated-pleomorphic-sarcoma-causes-symptoms-and-treatment/

  Undifferentiated pleomorphic sarcoma was previously also known as malignant fibrous histiocytoma. This type of sarcoma can be extremely aggressive and may require immediate treatment to stop it from growing. […] Your doctor will select your treatment option based on the location and the size of the lump. The following options are available for the treatment of undifferentiated pleomorphic sarcoma: […] Surgery depends on the location of the sarcoma. If the sarcoma is in the arms or legs, the doctor will completely remove the sarcoma and a part of the healthy tissue to stop it from spreading. However, if cancer has spread widely, surgeons may have to amputate your arm or leg. Radiation therapy or chemotherapy may be performed before the surgery to shrink cancer and avoid amputations. […] If the sarcoma is spreading, your doctor may also subject you to chemotherapy. In chemotherapy, your doctor will prescribe various drugs to treat your cancer. These drugs work by killing the fast-growing cells to prevent the formation of sarcoma or tumors. Chemotherapy for undifferentiated pleomorphic sarcoma is usually prescribed if it recurs after the initial treatment.

• #56 Osteosarcoma and Undifferentiated Pleomorphic Sarcoma of Bone Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Chicago Colorectal

  https://chicagocolorectal.com/patient-education/healthwise?DOCHWID=ncicdr0000062698

  Patients with undifferentiated pleomorphic sarcoma (UPS) of bone are treated according to osteosarcoma treatment protocols. The outcome for patients with resectable UPS is similar to the outcome for patients with osteosarcoma. As with osteosarcoma, patients with favorable necrosis (≥90% necrosis) have a longer survival than do those with an inferior necrosis (<90% necrosis). Many patients with UPS will need preoperative chemotherapy to achieve a wide local excision. [...] Treatment options for patients with osteosarcoma or undifferentiated pleomorphic sarcoma (UPS) of bone with metastatic disease at diagnosis include the following: Chemotherapy. [...] The treatment options for UPS of bone with metastasis at initial presentation are the same as the treatment for osteosarcoma with metastasis. Patients with unresectable or metastatic UPS have a very poor outcome.

• #57 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  In the setting of distant metastasis, surgery may be associated with long-term disease-free survival in patients with pulmonary metastasis and optimal underlying disease biology. […] Complete staging and treatment planning by a multidisciplinary team of cancer specialists is required to determine the optimal treatment for patients with soft tissue sarcoma. […] The role of chemotherapy is not well defined. […] There is evidence that favorable clinical outcomes may be associated with referral to a specialized sarcoma treatment center. […] Chemotherapy remains controversial, and its potential benefits should be considered in the context of the short- and long-term toxicities of the chemotherapy regimen. […] The role of adjuvant chemotherapy remains controversial. […] Neoadjuvant chemotherapy with or without radiation therapy has shown response rates of 17% to 32%, 10-year RFS rates of up to 58%, and 10-year OS rates of up to 64%.

• #58 Undifferentiated pleomorphic sarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Undifferentiated_pleomorphic_sarcoma

  In place or combined with surgery and/or radiotherapy, severe and/or metastatic cases of UPS are commonly treated with epirubicin plus ifosfamide; doxorubicin alone or combined with ifosfamide, olaratumab, trabectedin, gemcitabine, or docetaxel; cyclophosphamide, vincristine, doxorubicin, plus dacarbazine or cisplatin; cyclophosphamide, doxorubicin, plus dacarbazine; high dose methotrexate; or etoposide, ifosfamide, and cisplatin. […] However, other studies report that the addition of radiotherapy and/or chemotherapy to surgical resection does not improve recurrence or overall survival rates; addition of radiotherapy to surgery improves local control of UPS tumors but not disease-free survival rates (i.e. time from treatment to recurrence of disease); and adjuvant chemotherapy and radiotherapy have no significant effects on local recurrence-free survival rates, metastasis-free survival times, and overall survival rates.

• #59

  https://www.orthobullets.com/pathology/8064/undifferentiated-pleomorphic-sarcoma

  Undifferentiated Pleomorphic Sarcoma, previously known as malignant fibrous histiocytoma, is a high-grade, aggressive, malignant fibrogenic tumor. […] Treatment is usually wide-margin surgical excision with radiotherapy. […] Wide surgical resection radiation therapy is the standard of care in most cases. […] Radiation is an important adjunct to surgery decreasing local recurrence. […] Chemotherapy may be administered in some cases. […] Neoadjuvant chemotherapy has shown improved 5-year recurrence-free survival and overall survival in tumors 10 cm. […] 50-60% 5-year survival. […] Amputation is indicated when otherwise unable to resect tumor with negative margins. […] Good prognosis in absence of metastatic disease.

• #60 Treatment of Soft Tissue Sarcomas, by Stage | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/treating/by-stage.html

  For some types of soft tissue sarcomas, treatment with newer targeted drugs or immunotherapy might also be an option. […] If the sarcoma comes back in the same area where it started, it may be treated with surgery. Radiation therapy may be given after surgery, especially if radiation wasn’t part of the treatment of the original tumor. If the sarcoma returns in a distant part of the body, chemo, targeted therapy, or immunotherapy drugs may be options.

• #61 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Anthracyclines remain the first-line class of systemic therapy in managing most locally advanced and metastatic soft tissue sarcoma. […] The combination of gemcitabine and docetaxel is used as second-line therapy in treating patients with soft tissue sarcoma. […] Immune checkpoint inhibitors have shown some activity in selected soft tissue sarcoma subtypes, but their use cannot be routinely recommended. […] Treatment options for recurrent soft tissue sarcoma include surgery with or without radiation therapy, chemotherapy and targeted therapy, and immune checkpoint inhibitor therapy.

• #62 Mayo Clinic Health Library – Undifferentiated pleomorphic sarcoma | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20344525

  Radiation therapy uses high-powered beams of energy, such as X-rays or protons, to kill cancer cells. […] Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. It can be administered by pill or through a vein (intravenously), or both. […] Targeted drug treatments focus on specific abnormalities present within cancer cells. […] Immunotherapy uses your immune system to fight cancer. […] During intraoperative radiation therapy (IORT) radiation is directed through the surgical incision onto a specific site; in this case a thigh.

• #63 Successful pazopanib treatment of undifferentiated pleomorphic sarcoma with coamplification of PDGFRA, VEGFR2 and KIT: A case report

  https://www.spandidos-publications.com/10.3892/mco.2024.2767

  Undifferentiated pleomorphic sarcoma (UPS) is a high-grade, aggressive soft tissue sarcoma (STS) with a poor prognosis, and no definitive or effective treatment is currently available for it. […] Pazopanib, an orally available multiple tyrosine kinase inhibitor, has been approved for the treatment of advanced STS. […] The patient exhibited a marked and sustained response to pazopanib. […] Palliative radiation therapy was administered to the left femur and second/fifth thoracic vertebrae to prevent fractures. […] After radiation therapy, the patient achieved a partial response after eight courses of doxorubicin. […] Hence, pazopanib was initiated as a second-line treatment. […] Notably, the retroperitoneal tumour shrank, and no new lesions developed for 3 years after the initiation of pazopanib treatment.

• #64 Successful pazopanib treatment of undifferentiated pleomorphic sarcoma with coamplification of PDGFRA, VEGFR2 and KIT: A case report

  https://www.spandidos-publications.com/10.3892/mco.2024.2767

  This response suggests that the coamplification of PDGFRA, VEGFR2 and KIT may predict favourable outcomes in response to pazopanib. […] Pazopanib is an oral tyrosine kinase inhibitor (TKI) with activity against vascular endothelial growth factor receptor 1 (VEGFR1), VEGFR2, VEGFR3, platelet-derived growth factor receptor A (PDGFRA), PDGFRB, and stem cell factor receptor (KIT). […] Pazopanib has been approved for the treatment of advanced STSs. […] Therefore, we treated the patient with pazopanib with second-line therapy. […] Interestingly, CR was achieved and maintained for 3 years with pazopanib. […] The present case suggests that STSs with amplified PDGFRA, VEGFR2, and KIT are rare; however, patients with these features may benefit from pazopanib therapy. […] Therefore, further studies are required to confirm the benefits of pazopanib in patients with coamplification of PDGFRA, VEGFR2, and KIT, and to establish predictive markers for pazopanib sensitivity.

• #65 Treatment of High-Grade Undifferentiated Pleomorphic Sarcoma in the Retroperitoneum

  https://www.targetedonc.com/view/treatment-of-high-grade-undifferentiated-pleomorphic-sarcoma-in-the-retroperitoneum

  The prognosis for sarcomas really depends on the treatment modalities that can be used and the treatment modalities that are used. […] The standard treatment options for patients who present with unresectable soft tissue sarcomas is to use chemotherapeutic agents. For the first-line therapy, one would use a doxorubicin-based therapy and, preferably, probably in this situation, one would use the addition of olaratumab. […] If we look historically at the data, there are other combinations that can be utilized, including doxorubicin with ifosfamide or single-agent doxorubicin.

• #66

  https://ncs.navigatingcare.com/library/bone-osteo/learn/treatment-of-recurrent-osteosarcoma-and-undifferentiated-pleomorphic-sarcoma-ups-of-bone

  Treatment of recurrent osteosarcoma and UPS of bone may include the following: Surgery to remove cancer in all areas where it has spread. Chemotherapy and targeted therapy (sorafenib, regorafenib, lenvatinib, or everolimus). Samarium and radiation therapy. […] Treatment depends on the area and type of recurrence, for example: For tumors that have recurred in the same bone where the cancer started: Surgery. Radiation therapy. […] For tumors that have recurred in the lung only: Surgery. Chemotherapy. Targeted therapy. A clinical trial comparing two different types of surgery (thoracotomy and video-assisted thoracoscopic surgery) in treating patients with osteosarcoma that has spread to the lung. […] For tumors that have recurred in bones other than where the cancer started: Surgery. Samarium with or without stem cells, as palliative treatment to relieve pain and improve the quality of life. […] For tumors that have recurred twice: Surgery to remove the cancer and/or chemotherapy. […] Clinical trials for treatment of recurrent osteosarcoma and UPS of bone may include the following: A clinical trial of a new immune cell therapy.

• #67 A primary undifferentiated pleomorphic sarcoma of the lumbosacral region harboring a LMNA-NTRK1 gene fusion with durable clinical response to crizotinib: a case report | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4749-z

  Perioperative chemotherapy was recently reported to be beneficial in terms of overall survival, and doxorubicin as a single agent or in combination with ifosfamide is the first choice of chemotherapy in cases of UPS metastasis. […] The observed effectiveness of crizotinib treatment provides clinical validation of this molecular target. […] Molecularly targeted therapy of these rare but aggressive lesions represents a novel treatment option that may lead to fewer toxic side effects and better clinical outcomes. […] Crizotinib is a multi-active kinase inhibitor that blocks TrkA autophosphorylation and cell growth in cells expressing NTRK1 fusion proteins. […] Based on the report of a minor response to crizotinib in a case of non-small cell lung cancer harboring a NTRK1 fusion as well as preclinical data, we started oral administration of crizotinib (450 mg QD) in the UPS patient described in this report. Over the follow-up period, the patient did not experience intolerable adverse effects from treatment and continued crizotinib monotherapy with no evidence of disease for more than 18 months as of July 2018.

• #68 A primary undifferentiated pleomorphic sarcoma of the lumbosacral region harboring a LMNA-NTRK1 gene fusion with durable clinical response to crizotinib: a case report | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4749-z

  This case provides robust evidence for the importance of molecular evaluation in cases of these rare but aggressive lesions and stresses the need for the development of drugs for better molecularly targeted STS treatment, especially when standard-of-care options have been exhausted or treatment options are unavailable.

• #69 Undifferentiated pleomorphic sarcoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/undifferentiated-pleomorphic-sarcoma/diagnosis-treatment/drc-20389565

  Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. It can be administered by pill or through a vein (intravenously), or both. […] For undifferentiated pleomorphic sarcoma, targeted therapy drugs may be combined with chemotherapy. […] Immunotherapy uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that help them hide. Immunotherapy works by interfering with that process.

• #70 Undifferentiated pleomorphic sarcoma | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/undifferentiated-pleomorphic-sarcoma

  Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die. […] Immunotherapy uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that help them hide. Immunotherapy works by interfering with that process.

• #71 Moving the Needle in Undifferentiated Pleomorphic Sarcoma: Overview of Paradigm

  https://www.onclive.com/view/moving-the-needle-in-undifferentiated-pleomorphic-sarcoma-overview-of-paradigm

  In this episode, experts discuss the latest advancements in the multimodal treatment of undifferentiated pleomorphic sarcoma of the extremities. […] Recently, immunotherapy, radiation therapy, and surgery are emerging as a growing and promising combination for the treatment of UPS of the extremities. Pembrolizumab [Keytruda], an antiPD-1 immune checkpoint inhibitor, has shown efficacy in various malignancies, including soft tissue sarcomas. The Stand Up to Cancer Sarcoma Alliance for Research through Collaboration [SARC] 032 trial demonstrated that the addition of pembrolizumab to preoperative radiotherapy and surgery significantly improved disease-free survival in patients with stage III UPS of the extremities compared with radiation and surgery alone. […] The multimodal approach addresses both local and systemic disease control, offering a comprehensive treatment strategy for stage III UPS of the extremities. The combination of pembrolizumab, radiotherapy, and surgery represents a significant advancement in the management of this aggressive sarcoma subtype.

• #72 Undifferentiated pleomorphic sarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Undifferentiated_pleomorphic_sarcoma

  Recent studies have treated UPS by targeting the immune system with pembrolizumab. […] It is clear that new treatment strategies as well as further studies on the efficacy of pembrolizumab and similarly acting immunotherapy drugs used with or without radiotherapy and/or chemotherapy over longer time periods are needed to evaluate their usefulness in treating UPS.

• #73 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  Stages II and III should involve multidisciplinary action to discuss the appropriateness of pre- or postoperative chemoradiation. Unresectable cases can be approached with chemotherapy, chemoradiation, or regional limb therapy. […] For stage IV patients, there is insufficient data to support a specific plan, and expert oncologists in STSs must be consulted. […] Beyond traditional chemotherapy for UPS, immune-checkpoint inhibition with pembrolizumab (anti-PD1), nivolumab (anti-PD1), and ipilimumab (anti-CTLA4) is currently under investigation. […] Similar principles apply to internal, retroperitoneal, or intraabdominal UPS management. Excision with negative margins, with or without IORT, is the treatment of choice for resectable disease, whilst chemotherapy, chemoradiation, or HDR are preferred for unresectable cases.

• #74 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  Stages II and III should involve multidisciplinary action to discuss the appropriateness of pre- or postoperative chemoradiation. Unresectable cases can be approached with chemotherapy, chemoradiation, or regional limb therapy. […] For stage IV patients, there is insufficient data to support a specific plan, and expert oncologists in STSs must be consulted. […] Beyond traditional chemotherapy for UPS, immune-checkpoint inhibition with pembrolizumab (anti-PD1), nivolumab (anti-PD1), and ipilimumab (anti-CTLA4) is currently under investigation. […] Similar principles apply to internal, retroperitoneal, or intraabdominal UPS management. Excision with negative margins, with or without IORT, is the treatment of choice for resectable disease, whilst chemotherapy, chemoradiation, or HDR are preferred for unresectable cases.

• #75 Moving the Needle in Undifferentiated Pleomorphic Sarcoma: Overview of Paradigm

  https://www.onclive.com/view/moving-the-needle-in-undifferentiated-pleomorphic-sarcoma-overview-of-paradigm

  In this episode, experts discuss the latest advancements in the multimodal treatment of undifferentiated pleomorphic sarcoma of the extremities. […] Recently, immunotherapy, radiation therapy, and surgery are emerging as a growing and promising combination for the treatment of UPS of the extremities. Pembrolizumab [Keytruda], an antiPD-1 immune checkpoint inhibitor, has shown efficacy in various malignancies, including soft tissue sarcomas. The Stand Up to Cancer Sarcoma Alliance for Research through Collaboration [SARC] 032 trial demonstrated that the addition of pembrolizumab to preoperative radiotherapy and surgery significantly improved disease-free survival in patients with stage III UPS of the extremities compared with radiation and surgery alone. […] The multimodal approach addresses both local and systemic disease control, offering a comprehensive treatment strategy for stage III UPS of the extremities. The combination of pembrolizumab, radiotherapy, and surgery represents a significant advancement in the management of this aggressive sarcoma subtype.

• #76 Pembrolizumab Plus Standard Therapy Improves Outcomes in Stage III Soft Tissue Sarcoma

  https://www.oncnursingnews.com/view/pembrolizumab-plus-standard-therapy-improves-outcomes-in-stage-iii-soft-tissue-sarcoma

  Pembrolizumab, added to preoperative radiation and surgery, prolonged disease-free survival in patients with soft tissue sarcoma of the extremity. […] Adding pembrolizumab standard treatment (radiation and surgery) significantly improved outcomes for patients with advanced soft tissue sarcoma in the limbs. […] Findings from SU2C-SARC032 indicate that pembrolizumab is a promising new treatment option for these patients and suggest a path for even greater therapeutic effect by further optimizing immunotherapy. […] Based on these findings, as well as prior data demonstrating the synergistic effects of radiation therapy and immune checkpoint blockade on local and distant tumor response, the SU2C-SARC032 trial investigators hypothesized that adding pembrolizumab to preoperative radiation therapy and surgery could improve DFS for patients with stage III soft tissue sarcoma. […] Therefore, for patients with resectable, intermediate-grade or high-grade undifferentiated pleomorphic sarcoma or liposarcoma measuring more than 5 cm, SU2C-SARC032 establishes pembrolizumab as a new treatment option.

• #77 Neoadjuvant Immunotherapy May Improve Long Term Survival in Patients With Soft Tissue Sarcoma – The ASCO Post

  https://ascopost.com/news/february-2024/neoadjuvant-immunotherapy-may-improve-long-term-survival-in-patients-with-soft-tissue-sarcoma/

  Patients with soft-tissue sarcoma treated with neoadjuvant immunotherapy had very little residual tumor at the time of surgery and promising long-term survival, according to phase II trial results published by Roland et al in Nature Cancer. […] Currently, surgery is the only potential cure for those with resectable soft-tissue sarcoma; however, many patients experience cancer recurrence within 5 years. Radiation therapy and chemotherapy prior to surgery are often used to reduce the risk of disease recurrence. […] The researchers discovered that following treatment with a combination of immunotherapy and radiation and subsequent surgical removal of the residual mass, 90% of the patients with undifferentiated pleomorphic sarcoma had less than 15% viable tumor cells remainingan improvement over the amount of residual tumor cells typically seen following radiation alone. Further, the 2-year overall survival rate following neoadjuvant immunotherapy was 90% in the patients with undifferentiated pleomorphic sarcoma and 82% in the patients with resectable retroperitoneal dedifferentiated liposarcoma.

• #78 Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/s13569-020-00133-9

  Conventional cytotoxic agents and pazopanib are approved for advanced soft tissue sarcomas but have low response rates and modest survival benefits. […] Recently, immune checkpoint inhibitors have shown clinically meaningful activity. The combination of pazopanib and immunotherapy has shown synergism in various other malignancies but has not been fully explored in advanced soft tissue sarcomas. […] In view of significant symptoms, she was treated with pazopanib in combination with pembrolizumab. She had remarkable radiological and clinical improvement, with a manageable toxicity profile and an ongoing response at ten months of therapy. […] Undifferentiated pleomorphic sarcoma is an immunologically active subtype of soft tissue sarcoma, which is particularly amenable to immune checkpoint inhibitors. Pazopanib with immune checkpoint inhibitors is a well-tolerated, yet hitherto underexplored combination that may offer significant clinical benefit in advanced sarcomas; this finding warrants further evaluation in clinical trials.

• #79 Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/s13569-020-00133-9

  Given the need to achieve a rapid tumor response, and evidence for the combination therapy resulting in meaningful results with acceptable toxicity, we were enticed to offer it to our patient after a discussion of the potential risks and benefits. […] Although each agent’s individual contribution to the good response observed in this patient can only be confirmed or refuted in a randomized trial, the improvement was most likely attributable to the immune checkpoint inhibitor since objective responses with single agent pazopanib are uncommon and typically not well-sustained. […] Considering that this dual therapy is feasible, well tolerated and can result in sustained response, these findings merit further evaluation in prospectively designed randomized clinical trials.

• #80 A Randomized Phase III Trial of Doxorubicin + Pembrolizumab Versus Doxorubicin Alone for the Treatment of Undifferentiated Pleomorphic Sarcoma (UPS) and Related Poorly Differentiated Sarcomas | Dana-Farber Cancer Institute

  https://www.dana-farber.org/clinical-trials/24-411

  This phase II trial compares the effect of immunotherapy (pembrolizumab) plus chemotherapy (doxorubicin) to chemotherapy (doxorubicin) alone in treating patients with undifferentiated pleomorphic sarcoma (UPS) or a related poorly differentiated sarcoma that has spread from where it first started (primary site) to other places in the body (metastatic) or that cannot be removed by surgery (unresectable). […] Adding immunotherapy (pembrolizumab) to the standard chemotherapy (doxorubicin) may help patients with metastatic or unresectable UPS or a related poorly differentiated sarcoma live longer without having disease progression.

• #81 Undifferentiated Pleomorphic Sarcoma/Myxofibrosarcoma/Other Fibrosarcomas | Springer Publishing

  https://connect.springerpub.com/content/book/978-0-8261-4853-7/part/part02/chapter/ch20

  Undifferentiated pleomorphic sarcoma (UPS) encompasses malignant neoplasms of mesenchymal origin, which demonstrate cellular pleomorphism and which lack evidence of cellular differentiation. […] For localized disease, the treatment of high-grade tumors includes a multimodality approach (perioperative chemotherapy, radiation therapy, and surgery), followed by close surveillance with scans and follow-up visits. […] For metastatic disease, patients will require systemic therapies as well as radiation therapy and surgery in selected patients. […] This chapter reviews clinical presentation, diagnostic approach, and general therapeutic approach for UPS, and discusses metastatic disease, atypical fibroxanthoma, and fibrosarcoma subtypes.

• #82 A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

  https://www.mdpi.com/2077-0383/13/13/3684

  This detailed review focuses on retroperitoneal undifferentiated pleomorphic sarcoma (UPS), a particularly aggressive soft-tissue sarcoma that poses unique diagnostic and therapeutic challenges due to its rarity and complex presentation. […] In terms of treatment, this paper advocates a multidisciplinary approach that combines surgery, radiotherapy and chemotherapy and tailors it to individual patients to optimize treatment outcomes. […] This review highlights case studies that illustrate the effectiveness of surgical intervention in the treatment of these tumors and emphasize the importance of achieving clear surgical margins to prevent recurrence. […] A multidisciplinary approach is crucial for the effective treatment of retroperitoneal UPS due to the complex nature and rarity of the disease. This approach involves the collaboration of various specialists, including surgical oncologists, medical oncologists, radiologists, pathologists, and radiation oncologists.

• #83 Osteosarcoma and UPS of Bone Treatment – NCI

  https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq

  Treatment of newly diagnosed localized osteosarcoma and UPS of bone may include: surgery to remove the tumor, chemotherapy before or after surgery to remove the tumor, radiation therapy if surgery cannot be done or if the tumor was not completely removed by surgery. […] Treatment of newly diagnosed osteosarcoma and UPS that has spread to the lung may include chemotherapy followed by surgery to remove the primary cancer and more chemotherapy. […] Treatment of newly diagnosed osteosarcoma and UPS that has spread to a distant bone or to both the lung and a distant bone may include: Chemotherapy followed by surgery to remove the primary cancer. […] Treatment depends on where the cancer recurred and whether it has recurred more than once. […] For tumors that have recurred in the same bone where the cancer started, treatment is surgery.

• #84 Undifferentiated Pleomorphic Sarcoma/Myxofibrosarcoma/Other Fibrosarcomas | Springer Publishing

  https://connect.springerpub.com/content/book/978-0-8261-4853-7/part/part02/chapter/ch20

  Undifferentiated pleomorphic sarcoma (UPS) encompasses malignant neoplasms of mesenchymal origin, which demonstrate cellular pleomorphism and which lack evidence of cellular differentiation. […] For localized disease, the treatment of high-grade tumors includes a multimodality approach (perioperative chemotherapy, radiation therapy, and surgery), followed by close surveillance with scans and follow-up visits. […] For metastatic disease, patients will require systemic therapies as well as radiation therapy and surgery in selected patients. […] This chapter reviews clinical presentation, diagnostic approach, and general therapeutic approach for UPS, and discusses metastatic disease, atypical fibroxanthoma, and fibrosarcoma subtypes.

• #85 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  Stages II and III should involve multidisciplinary action to discuss the appropriateness of pre- or postoperative chemoradiation. Unresectable cases can be approached with chemotherapy, chemoradiation, or regional limb therapy. […] For stage IV patients, there is insufficient data to support a specific plan, and expert oncologists in STSs must be consulted. […] Beyond traditional chemotherapy for UPS, immune-checkpoint inhibition with pembrolizumab (anti-PD1), nivolumab (anti-PD1), and ipilimumab (anti-CTLA4) is currently under investigation. […] Similar principles apply to internal, retroperitoneal, or intraabdominal UPS management. Excision with negative margins, with or without IORT, is the treatment of choice for resectable disease, whilst chemotherapy, chemoradiation, or HDR are preferred for unresectable cases.

• #86 Osteosarcoma and UPS of Bone Treatment – NCI

  https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq

  For tumors that have recurred in the lung only, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy. […] For tumors that have recurred in bones other than where the cancer started, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy, samarium with or without stem cells, as palliative treatment to relieve pain and improve quality of life.

• #87

  https://ncs.navigatingcare.com/library/bone-osteo/learn/treatment-of-recurrent-osteosarcoma-and-undifferentiated-pleomorphic-sarcoma-ups-of-bone

  Treatment of recurrent osteosarcoma and UPS of bone may include the following: Surgery to remove cancer in all areas where it has spread. Chemotherapy and targeted therapy (sorafenib, regorafenib, lenvatinib, or everolimus). Samarium and radiation therapy. […] Treatment depends on the area and type of recurrence, for example: For tumors that have recurred in the same bone where the cancer started: Surgery. Radiation therapy. […] For tumors that have recurred in the lung only: Surgery. Chemotherapy. Targeted therapy. A clinical trial comparing two different types of surgery (thoracotomy and video-assisted thoracoscopic surgery) in treating patients with osteosarcoma that has spread to the lung. […] For tumors that have recurred in bones other than where the cancer started: Surgery. Samarium with or without stem cells, as palliative treatment to relieve pain and improve the quality of life. […] For tumors that have recurred twice: Surgery to remove the cancer and/or chemotherapy. […] Clinical trials for treatment of recurrent osteosarcoma and UPS of bone may include the following: A clinical trial of a new immune cell therapy.

• #88 Moving the Needle in Undifferentiated Pleomorphic Sarcoma: Overview of Paradigm

  https://www.onclive.com/view/moving-the-needle-in-undifferentiated-pleomorphic-sarcoma-overview-of-paradigm

  In this episode, experts discuss the latest advancements in the multimodal treatment of undifferentiated pleomorphic sarcoma of the extremities. […] Recently, immunotherapy, radiation therapy, and surgery are emerging as a growing and promising combination for the treatment of UPS of the extremities. Pembrolizumab [Keytruda], an antiPD-1 immune checkpoint inhibitor, has shown efficacy in various malignancies, including soft tissue sarcomas. The Stand Up to Cancer Sarcoma Alliance for Research through Collaboration [SARC] 032 trial demonstrated that the addition of pembrolizumab to preoperative radiotherapy and surgery significantly improved disease-free survival in patients with stage III UPS of the extremities compared with radiation and surgery alone. […] The multimodal approach addresses both local and systemic disease control, offering a comprehensive treatment strategy for stage III UPS of the extremities. The combination of pembrolizumab, radiotherapy, and surgery represents a significant advancement in the management of this aggressive sarcoma subtype.

• #89 Pembrolizumab Plus Standard Therapy Improves Outcomes in Stage III Soft Tissue Sarcoma

  https://www.oncnursingnews.com/view/pembrolizumab-plus-standard-therapy-improves-outcomes-in-stage-iii-soft-tissue-sarcoma

  Pembrolizumab, added to preoperative radiation and surgery, prolonged disease-free survival in patients with soft tissue sarcoma of the extremity. […] Adding pembrolizumab standard treatment (radiation and surgery) significantly improved outcomes for patients with advanced soft tissue sarcoma in the limbs. […] Findings from SU2C-SARC032 indicate that pembrolizumab is a promising new treatment option for these patients and suggest a path for even greater therapeutic effect by further optimizing immunotherapy. […] Based on these findings, as well as prior data demonstrating the synergistic effects of radiation therapy and immune checkpoint blockade on local and distant tumor response, the SU2C-SARC032 trial investigators hypothesized that adding pembrolizumab to preoperative radiation therapy and surgery could improve DFS for patients with stage III soft tissue sarcoma. […] Therefore, for patients with resectable, intermediate-grade or high-grade undifferentiated pleomorphic sarcoma or liposarcoma measuring more than 5 cm, SU2C-SARC032 establishes pembrolizumab as a new treatment option.

• #90 Moving the Needle in Undifferentiated Pleomorphic Sarcoma: Overview of Paradigm

  https://www.onclive.com/view/moving-the-needle-in-undifferentiated-pleomorphic-sarcoma-overview-of-paradigm

  In this episode, experts discuss the latest advancements in the multimodal treatment of undifferentiated pleomorphic sarcoma of the extremities. […] Recently, immunotherapy, radiation therapy, and surgery are emerging as a growing and promising combination for the treatment of UPS of the extremities. Pembrolizumab [Keytruda], an antiPD-1 immune checkpoint inhibitor, has shown efficacy in various malignancies, including soft tissue sarcomas. The Stand Up to Cancer Sarcoma Alliance for Research through Collaboration [SARC] 032 trial demonstrated that the addition of pembrolizumab to preoperative radiotherapy and surgery significantly improved disease-free survival in patients with stage III UPS of the extremities compared with radiation and surgery alone. […] The multimodal approach addresses both local and systemic disease control, offering a comprehensive treatment strategy for stage III UPS of the extremities. The combination of pembrolizumab, radiotherapy, and surgery represents a significant advancement in the management of this aggressive sarcoma subtype.

• #91 A Randomized Phase III Trial of Doxorubicin + Pembrolizumab Versus Doxorubicin Alone for the Treatment of Undifferentiated Pleomorphic Sarcoma (UPS) and Related Poorly Differentiated Sarcomas | Dana-Farber Cancer Institute

  https://www.dana-farber.org/clinical-trials/24-411

  This phase II trial compares the effect of immunotherapy (pembrolizumab) plus chemotherapy (doxorubicin) to chemotherapy (doxorubicin) alone in treating patients with undifferentiated pleomorphic sarcoma (UPS) or a related poorly differentiated sarcoma that has spread from where it first started (primary site) to other places in the body (metastatic) or that cannot be removed by surgery (unresectable). […] Adding immunotherapy (pembrolizumab) to the standard chemotherapy (doxorubicin) may help patients with metastatic or unresectable UPS or a related poorly differentiated sarcoma live longer without having disease progression.

• #92 Undifferentiated Pleomorphic Sarcoma: Long-Term Follow-Up from a Large | CMAR

  https://www.dovepress.com/undifferentiated-pleomorphic-sarcoma-long-term-follow-up-from-a-large–peer-reviewed-fulltext-article-CMAR

  Our study aimed to describe the clinical features of undifferentiated pleomorphic sarcoma (UPS) and identify the predictors of poor outcomes. […] Aggressive surgical resection remained the principal treatment. Local recurrence occurred in 13-42% of patients and distant metastasis could be found in 31-35% of patients despite aggressive surgery. […] Currently, it is urgent to clarify and update the prognosis of patients suffering from UPS with a long period of follow-up owing to changes in classification criteria. […] Primary treatment of UPS should be conducted by experts in large sarcoma center. Wide surgical margin provides sufficient control of the disease recurrence. […] In our study, tumor presentation, tumor size and important structures involved were independent prognostic factors associated with OS, which justified the early and primary treatment was very important.

• #93 Treatment of Soft Tissue Sarcomas, by Stage | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/treating/by-stage.html

  For some types of soft tissue sarcomas, treatment with newer targeted drugs or immunotherapy might also be an option. […] If the sarcoma comes back in the same area where it started, it may be treated with surgery. Radiation therapy may be given after surgery, especially if radiation wasn’t part of the treatment of the original tumor. If the sarcoma returns in a distant part of the body, chemo, targeted therapy, or immunotherapy drugs may be options.

• #94 Osteosarcoma and UPS of Bone Treatment – NCI

  https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq

  Treatment of newly diagnosed localized osteosarcoma and UPS of bone may include: surgery to remove the tumor, chemotherapy before or after surgery to remove the tumor, radiation therapy if surgery cannot be done or if the tumor was not completely removed by surgery. […] Treatment of newly diagnosed osteosarcoma and UPS that has spread to the lung may include chemotherapy followed by surgery to remove the primary cancer and more chemotherapy. […] Treatment of newly diagnosed osteosarcoma and UPS that has spread to a distant bone or to both the lung and a distant bone may include: Chemotherapy followed by surgery to remove the primary cancer. […] Treatment depends on where the cancer recurred and whether it has recurred more than once. […] For tumors that have recurred in the same bone where the cancer started, treatment is surgery.

• #95 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Anthracyclines remain the first-line class of systemic therapy in managing most locally advanced and metastatic soft tissue sarcoma. […] The combination of gemcitabine and docetaxel is used as second-line therapy in treating patients with soft tissue sarcoma. […] Immune checkpoint inhibitors have shown some activity in selected soft tissue sarcoma subtypes, but their use cannot be routinely recommended. […] Treatment options for recurrent soft tissue sarcoma include surgery with or without radiation therapy, chemotherapy and targeted therapy, and immune checkpoint inhibitor therapy.

• #96 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  In the setting of distant metastasis, surgery may be associated with long-term disease-free survival in patients with pulmonary metastasis and optimal underlying disease biology. […] Complete staging and treatment planning by a multidisciplinary team of cancer specialists is required to determine the optimal treatment for patients with soft tissue sarcoma. […] The role of chemotherapy is not well defined. […] There is evidence that favorable clinical outcomes may be associated with referral to a specialized sarcoma treatment center. […] Chemotherapy remains controversial, and its potential benefits should be considered in the context of the short- and long-term toxicities of the chemotherapy regimen. […] The role of adjuvant chemotherapy remains controversial. […] Neoadjuvant chemotherapy with or without radiation therapy has shown response rates of 17% to 32%, 10-year RFS rates of up to 58%, and 10-year OS rates of up to 64%.

• #97 Osteosarcoma and UPS of Bone Treatment – NCI

  https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq

  For tumors that have recurred in the lung only, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy. […] For tumors that have recurred in bones other than where the cancer started, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy, samarium with or without stem cells, as palliative treatment to relieve pain and improve quality of life.

• #98

  https://ncs.navigatingcare.com/library/bone-osteo/learn/treatment-of-recurrent-osteosarcoma-and-undifferentiated-pleomorphic-sarcoma-ups-of-bone

  Treatment of recurrent osteosarcoma and UPS of bone may include the following: Surgery to remove cancer in all areas where it has spread. Chemotherapy and targeted therapy (sorafenib, regorafenib, lenvatinib, or everolimus). Samarium and radiation therapy. […] Treatment depends on the area and type of recurrence, for example: For tumors that have recurred in the same bone where the cancer started: Surgery. Radiation therapy. […] For tumors that have recurred in the lung only: Surgery. Chemotherapy. Targeted therapy. A clinical trial comparing two different types of surgery (thoracotomy and video-assisted thoracoscopic surgery) in treating patients with osteosarcoma that has spread to the lung. […] For tumors that have recurred in bones other than where the cancer started: Surgery. Samarium with or without stem cells, as palliative treatment to relieve pain and improve the quality of life. […] For tumors that have recurred twice: Surgery to remove the cancer and/or chemotherapy. […] Clinical trials for treatment of recurrent osteosarcoma and UPS of bone may include the following: A clinical trial of a new immune cell therapy.

• #99

  https://ncs.navigatingcare.com/library/bone-osteo/learn/treatment-of-recurrent-osteosarcoma-and-undifferentiated-pleomorphic-sarcoma-ups-of-bone

  Treatment of recurrent osteosarcoma and UPS of bone may include the following: Surgery to remove cancer in all areas where it has spread. Chemotherapy and targeted therapy (sorafenib, regorafenib, lenvatinib, or everolimus). Samarium and radiation therapy. […] Treatment depends on the area and type of recurrence, for example: For tumors that have recurred in the same bone where the cancer started: Surgery. Radiation therapy. […] For tumors that have recurred in the lung only: Surgery. Chemotherapy. Targeted therapy. A clinical trial comparing two different types of surgery (thoracotomy and video-assisted thoracoscopic surgery) in treating patients with osteosarcoma that has spread to the lung. […] For tumors that have recurred in bones other than where the cancer started: Surgery. Samarium with or without stem cells, as palliative treatment to relieve pain and improve the quality of life. […] For tumors that have recurred twice: Surgery to remove the cancer and/or chemotherapy. […] Clinical trials for treatment of recurrent osteosarcoma and UPS of bone may include the following: A clinical trial of a new immune cell therapy.

• #100

  https://ncs.navigatingcare.com/library/bone-osteo/learn/treatment-of-recurrent-osteosarcoma-and-undifferentiated-pleomorphic-sarcoma-ups-of-bone

  Treatment of recurrent osteosarcoma and UPS of bone may include the following: Surgery to remove cancer in all areas where it has spread. Chemotherapy and targeted therapy (sorafenib, regorafenib, lenvatinib, or everolimus). Samarium and radiation therapy. […] Treatment depends on the area and type of recurrence, for example: For tumors that have recurred in the same bone where the cancer started: Surgery. Radiation therapy. […] For tumors that have recurred in the lung only: Surgery. Chemotherapy. Targeted therapy. A clinical trial comparing two different types of surgery (thoracotomy and video-assisted thoracoscopic surgery) in treating patients with osteosarcoma that has spread to the lung. […] For tumors that have recurred in bones other than where the cancer started: Surgery. Samarium with or without stem cells, as palliative treatment to relieve pain and improve the quality of life. […] For tumors that have recurred twice: Surgery to remove the cancer and/or chemotherapy. […] Clinical trials for treatment of recurrent osteosarcoma and UPS of bone may include the following: A clinical trial of a new immune cell therapy.

• #101 Osteosarcoma and UPS of Bone Treatment – NCI

  https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq

  For tumors that have recurred in the lung only, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy. […] For tumors that have recurred in bones other than where the cancer started, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy, samarium with or without stem cells, as palliative treatment to relieve pain and improve quality of life.

• #102 Osteosarcoma and UPS of Bone Treatment – NCI

  https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq

  For tumors that have recurred in the lung only, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy. […] For tumors that have recurred in bones other than where the cancer started, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy, samarium with or without stem cells, as palliative treatment to relieve pain and improve quality of life.

• #103 Osteosarcoma Treatment (PDQ®): Treatment – Patient Information [NCI] | The Children’s Hospital at Montefiore

  https://www.cham.org/health-library/article?id=ncicdr0000062949

  Treatment of newly diagnosed osteosarcoma and UPS that has spread to the lung may include chemotherapy followed by surgery to remove the primary cancer and more chemotherapy. Then, the doctor will remove the cancer in the lung and give additional chemotherapy. […] Treatment of newly diagnosed osteosarcoma and UPS that has spread to a distant bone or to both the lung and a distant bone may include: Chemotherapy followed by surgery to remove the primary cancer. More chemotherapy is given after surgery. This may be followed by surgery to remove cancer that has spread to other areas of the body. […] For tumors that have recurred in the same bone where the cancer started, treatment is surgery. […] For tumors that have recurred in the lung only, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy. […] For tumors that have recurred in bones other than where the cancer started, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy, samarium with or without stem cells, as palliative treatment to relieve pain and improve quality of life.

• #104 Osteosarcoma Treatment (PDQ®): Treatment – Patient Information [NCI] | The Children’s Hospital at Montefiore

  https://www.cham.org/health-library/article?id=ncicdr0000062949

  Treatment of newly diagnosed osteosarcoma and UPS that has spread to the lung may include chemotherapy followed by surgery to remove the primary cancer and more chemotherapy. Then, the doctor will remove the cancer in the lung and give additional chemotherapy. […] Treatment of newly diagnosed osteosarcoma and UPS that has spread to a distant bone or to both the lung and a distant bone may include: Chemotherapy followed by surgery to remove the primary cancer. More chemotherapy is given after surgery. This may be followed by surgery to remove cancer that has spread to other areas of the body. […] For tumors that have recurred in the same bone where the cancer started, treatment is surgery. […] For tumors that have recurred in the lung only, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy. […] For tumors that have recurred in bones other than where the cancer started, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy, samarium with or without stem cells, as palliative treatment to relieve pain and improve quality of life.

• #105 Osteosarcoma Treatment (PDQ®): Treatment – Patient Information [NCI] | The Children’s Hospital at Montefiore

  https://www.cham.org/health-library/article?id=ncicdr0000062949

  Treatment of newly diagnosed osteosarcoma and UPS that has spread to the lung may include chemotherapy followed by surgery to remove the primary cancer and more chemotherapy. Then, the doctor will remove the cancer in the lung and give additional chemotherapy. […] Treatment of newly diagnosed osteosarcoma and UPS that has spread to a distant bone or to both the lung and a distant bone may include: Chemotherapy followed by surgery to remove the primary cancer. More chemotherapy is given after surgery. This may be followed by surgery to remove cancer that has spread to other areas of the body. […] For tumors that have recurred in the same bone where the cancer started, treatment is surgery. […] For tumors that have recurred in the lung only, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy. […] For tumors that have recurred in bones other than where the cancer started, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy, samarium with or without stem cells, as palliative treatment to relieve pain and improve quality of life.

• #106 Osteosarcoma Treatment (PDQ®): Treatment – Patient Information [NCI] | The Children’s Hospital at Montefiore

  https://www.cham.org/health-library/article?id=ncicdr0000062949

  Treatment of newly diagnosed osteosarcoma and UPS that has spread to the lung may include chemotherapy followed by surgery to remove the primary cancer and more chemotherapy. Then, the doctor will remove the cancer in the lung and give additional chemotherapy. […] Treatment of newly diagnosed osteosarcoma and UPS that has spread to a distant bone or to both the lung and a distant bone may include: Chemotherapy followed by surgery to remove the primary cancer. More chemotherapy is given after surgery. This may be followed by surgery to remove cancer that has spread to other areas of the body. […] For tumors that have recurred in the same bone where the cancer started, treatment is surgery. […] For tumors that have recurred in the lung only, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy. […] For tumors that have recurred in bones other than where the cancer started, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy, samarium with or without stem cells, as palliative treatment to relieve pain and improve quality of life.

• #107 High-Grade Pleomorphic Sarcomas Treated with Immune Checkpoint Blockade: The MD Anderson Cancer Center Experience

  https://www.mdpi.com/2072-6694/16/9/1763

  Undifferentiated pleomorphic sarcomas (UPSs) represent 10–20% of all soft tissue sarcomas (STSs) and have quickly emerged as one of the more immune-sensitive types. […] This is a retrospective, observational study of all patients with metastatic high-grade pleomorphic sarcomas treated with FDA-approved ICB at MD Anderson Cancer Center intended to describe the efficacy and toxicity of ICB in this particular group of patients. […] The mainstay of advanced/metastatic treatment for STSs in general and UPSs in particular consists of systemic therapies, with doxorubicin-based and gemcitabine-based treatments used as front-line treatment, with a median progression-free survival (PFS) of around 6 months and a median overall survival (OS) of around 18–20 months. […] UPS has quickly emerged as one of the more immune-sensitive types of STS, which was identified during the first immune checkpoint blockade (ICB) clinical trials in patients with advanced and pretreated STS with an objective response rate (ORR) of 20–40% and a median PFS of 3 months.

• #108 High-Grade Pleomorphic Sarcomas Treated with Immune Checkpoint Blockade: The MD Anderson Cancer Center Experience

  https://www.mdpi.com/2072-6694/16/9/1763

  Undifferentiated pleomorphic sarcomas (UPSs) represent 10–20% of all soft tissue sarcomas (STSs) and have quickly emerged as one of the more immune-sensitive types. […] This is a retrospective, observational study of all patients with metastatic high-grade pleomorphic sarcomas treated with FDA-approved ICB at MD Anderson Cancer Center intended to describe the efficacy and toxicity of ICB in this particular group of patients. […] The mainstay of advanced/metastatic treatment for STSs in general and UPSs in particular consists of systemic therapies, with doxorubicin-based and gemcitabine-based treatments used as front-line treatment, with a median progression-free survival (PFS) of around 6 months and a median overall survival (OS) of around 18–20 months. […] UPS has quickly emerged as one of the more immune-sensitive types of STS, which was identified during the first immune checkpoint blockade (ICB) clinical trials in patients with advanced and pretreated STS with an objective response rate (ORR) of 20–40% and a median PFS of 3 months.

• #109 Successful pazopanib treatment of undifferentiated pleomorphic sarcoma with coamplification of PDGFRA, VEGFR2 and KIT: A case report

  https://www.spandidos-publications.com/10.3892/mco.2024.2767

  Undifferentiated pleomorphic sarcoma (UPS) is a high-grade, aggressive soft tissue sarcoma (STS) with a poor prognosis, and no definitive or effective treatment is currently available for it. […] Pazopanib, an orally available multiple tyrosine kinase inhibitor, has been approved for the treatment of advanced STS. […] The patient exhibited a marked and sustained response to pazopanib. […] Palliative radiation therapy was administered to the left femur and second/fifth thoracic vertebrae to prevent fractures. […] After radiation therapy, the patient achieved a partial response after eight courses of doxorubicin. […] Hence, pazopanib was initiated as a second-line treatment. […] Notably, the retroperitoneal tumour shrank, and no new lesions developed for 3 years after the initiation of pazopanib treatment.

• #110 Successful pazopanib treatment of undifferentiated pleomorphic sarcoma with coamplification of PDGFRA, VEGFR2 and KIT: A case report

  https://www.spandidos-publications.com/10.3892/mco.2024.2767

  This response suggests that the coamplification of PDGFRA, VEGFR2 and KIT may predict favourable outcomes in response to pazopanib. […] Pazopanib is an oral tyrosine kinase inhibitor (TKI) with activity against vascular endothelial growth factor receptor 1 (VEGFR1), VEGFR2, VEGFR3, platelet-derived growth factor receptor A (PDGFRA), PDGFRB, and stem cell factor receptor (KIT). […] Pazopanib has been approved for the treatment of advanced STSs. […] Therefore, we treated the patient with pazopanib with second-line therapy. […] Interestingly, CR was achieved and maintained for 3 years with pazopanib. […] The present case suggests that STSs with amplified PDGFRA, VEGFR2, and KIT are rare; however, patients with these features may benefit from pazopanib therapy. […] Therefore, further studies are required to confirm the benefits of pazopanib in patients with coamplification of PDGFRA, VEGFR2, and KIT, and to establish predictive markers for pazopanib sensitivity.

• #111 High-Grade Pleomorphic Sarcomas Treated with Immune Checkpoint Blockade: The MD Anderson Cancer Center Experience

  https://www.mdpi.com/2072-6694/16/9/1763

  Undifferentiated pleomorphic sarcomas (UPSs) represent 10–20% of all soft tissue sarcomas (STSs) and have quickly emerged as one of the more immune-sensitive types. […] This is a retrospective, observational study of all patients with metastatic high-grade pleomorphic sarcomas treated with FDA-approved ICB at MD Anderson Cancer Center intended to describe the efficacy and toxicity of ICB in this particular group of patients. […] The mainstay of advanced/metastatic treatment for STSs in general and UPSs in particular consists of systemic therapies, with doxorubicin-based and gemcitabine-based treatments used as front-line treatment, with a median progression-free survival (PFS) of around 6 months and a median overall survival (OS) of around 18–20 months. […] UPS has quickly emerged as one of the more immune-sensitive types of STS, which was identified during the first immune checkpoint blockade (ICB) clinical trials in patients with advanced and pretreated STS with an objective response rate (ORR) of 20–40% and a median PFS of 3 months.

• #112 Undifferentiated Pleomorphic Sarcoma — Cancer Survivors Network

  https://csn.cancer.org/discussion/325162/undifferentiated-pleomorphic-sarcoma

  My husband was diagnosed in early 2021. He began Gemcitabine and Docitaxel. He has had ten rounds. […] I know we need to ask our oncologist whether the side effects from treatment are worth the few months that my husband’s life could be extended. […] The only thing we can do is go back to chemo. […] Because I’ve had so much chemo, I could not do any more chemotherapy. So my doctors in New York recommended I do immunotherapy. I’ve done a year of Keytruda. So far I have no tumors. […] If the chemo is not working for your husband, look into a place that’s doing immunotherapy; the side effects are nothing like the chemo.

• #113 Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/s13569-020-00133-9

  Conventional cytotoxic agents and pazopanib are approved for advanced soft tissue sarcomas but have low response rates and modest survival benefits. […] Recently, immune checkpoint inhibitors have shown clinically meaningful activity. The combination of pazopanib and immunotherapy has shown synergism in various other malignancies but has not been fully explored in advanced soft tissue sarcomas. […] In view of significant symptoms, she was treated with pazopanib in combination with pembrolizumab. She had remarkable radiological and clinical improvement, with a manageable toxicity profile and an ongoing response at ten months of therapy. […] Undifferentiated pleomorphic sarcoma is an immunologically active subtype of soft tissue sarcoma, which is particularly amenable to immune checkpoint inhibitors. Pazopanib with immune checkpoint inhibitors is a well-tolerated, yet hitherto underexplored combination that may offer significant clinical benefit in advanced sarcomas; this finding warrants further evaluation in clinical trials.

• #114 Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/s13569-020-00133-9

  Given the need to achieve a rapid tumor response, and evidence for the combination therapy resulting in meaningful results with acceptable toxicity, we were enticed to offer it to our patient after a discussion of the potential risks and benefits. […] Although each agent’s individual contribution to the good response observed in this patient can only be confirmed or refuted in a randomized trial, the improvement was most likely attributable to the immune checkpoint inhibitor since objective responses with single agent pazopanib are uncommon and typically not well-sustained. […] Considering that this dual therapy is feasible, well tolerated and can result in sustained response, these findings merit further evaluation in prospectively designed randomized clinical trials.

• #115 High-Grade Pleomorphic Sarcomas Treated with Immune Checkpoint Blockade: The MD Anderson Cancer Center Experience

  https://www.mdpi.com/2072-6694/16/9/1763

  While there is an increasing number of ICB-based clinical trials with UPS patients, few real-world data on the use of ICB in UPS patients and other high-grade pleomorphic STS patients are available. […] The median PFS was 2.9 months and 3.8 months in the UPS group and in the other high-grade pleomorphic sarcoma group, respectively. […] The best response to ICB treatment was significantly associated with PFS (p = 0.0012): the median PFS was 40.7 months and 5.6 months in patients whose tumors responded (PR/CR) and stabilized (SD) per RECIST 1.1, respectively, compared to 2.2 months in non-responders. […] Real-world retrospective data demonstrate a median PFS of 2.9 months for patients with UPSs and other high-grade pleomorphic sarcomas treated with ICB in the metastatic setting, which is consistent with the published literature. The optimal sequencing of RT and prior lines of systemic therapy needs to be further evaluated.

• #116 Undifferentiated Pleomorphic Sarcoma | Orthopedic Surgery | Patient Care | Montefiore Einstein

  https://montefioreeinstein.org/patient-care/services/orthopedics/specialties/orthopedic-oncology/pleomorphic-sarcoma

  Treatment for undifferentiated pleomorphic sarcoma generally involves surgery. When the tumor is small and found in just one part of the body, surgery is sometimes enough and no additional treatments are required. […] If the tumor is larger than five centimeters, the combined use of surgery and radiation therapy offers patients better control of their disease, meaning a lower risk of local recurrence. A variety of radiation techniques can be used, and the exact method of delivery is tailored to a patients individual circumstances and clinical needs. If the tumor is large or if it has metastasized, treatment will often also include chemotherapy. […] Novel therapies for undifferentiated pleomorphic sarcoma are being explored, often within the context of ongoing clinical trials. These include a wide variety of targeted agents designed to affect one or more specific pathways. The role of immunotherapy is being currently explored. […] Following the completion of treatment, patients typically require close monitoring for a period of five years. This includes physical examinations and periodic imaging studies. Many patients continue to follow up even after five years, though at a reduced rate.

• #117 Long Duration Pembrolizumab for Metastatic Undifferentiated Pleomorphic Soft Tissue Sarcoma With Multimodality Therapy | Reuben | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/4237/3601

  Patients with undifferentiated pleomorphic sarcoma (UPS) of soft tissue have responsiveness to immunotherapy treatment. […] This report is unique owing to the probable longest reporting of successful continuous immunotherapy for metastatic UPS over 6.5 years and 109 cycles. […] A robust response was seen but occasionally augmented by adding external beam radiation therapy (XRT). Treatment was tolerated without adverse effects. […] For similar patients, a lengthy treatment course, beyond that utilized for other malignancies, can be considered. This is likely to be safe if it is tolerated and without early adverse effects. […] Other treatment modalities such as palliative surgery and XRT are described which may also be required for management of mixed responses. […] Recently immunotherapy has been shown to provide responses in soft tissue sarcoma.

• #118 Long Duration Pembrolizumab for Metastatic Undifferentiated Pleomorphic Soft Tissue Sarcoma With Multimodality Therapy | Reuben | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/4237/3601

  While immunotherapy is an encouraging advancement, only certain sarcoma subtypes appear to respond. […] No guidelines yet support or refute chronic, long-term immunotherapy for sarcoma management. […] Lengthy or prolonged immunotherapy could thus be a consideration when efficacy continues to be seen over many years. […] Herein a case is described with prolonged treatment and substantial continued response and tolerability to pembrolizumab immunotherapy for a patient with undifferentiated pleomorphic sarcoma (UPS). […] Multidisciplinary care with palliative surgery and XRT occurred for occasional, non-responding metastases. […] A prolonged use of immunotherapy for the treatment of metastatic UPS is presented. […] It is also shown that despite two separate lung metastases which lost an immune response, others continued to respond and did so for many years after immunotherapy was started.

• #119 Long Duration Pembrolizumab for Metastatic Undifferentiated Pleomorphic Soft Tissue Sarcoma With Multimodality Therapy | Reuben | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/4237/3601

  The consideration of prolonged immunotherapy is a concept for future researchers to consider based upon patient experiences such as this one. […] A trial could be designed without a customary cap of 2 years of immunotherapy, at least for patients who demonstrate ongoing responses to most of their metastatic disease. […] This report will hopefully assist in developing a body of evidence pertaining to outcomes of prolonged immunotherapy use for sarcoma patients.

• #120 Immunotherapy before surgery leads to promising long-term survival in sarcoma patients | MD Anderson Cancer Center

  https://www.mdanderson.org/newsroom/immunotherapy-before-surgery-leads-to-promising-long-term-survival.h00-159695178.html

  This is the first study investigating the use of neoadjuvant immunotherapy in patients with soft-tissue sarcoma before curative intent surgery, according to Roland. […] The researchers found that the presence of intratumoral B cells were associated with improved OS. […] We know from previous research the importance of the presence of B cells in a tumor to predict immunotherapy responses, and we found in this study that patients with higher levels of B cells in their tumors were more likely to respond. […] Trial participants experienced no increased risk of surgery complications and no new side effects were identified. Adverse effects observed were expected and manageable. The most common side effects were rash, fatigue and diarrhea.

• #121 Successful pazopanib treatment of undifferentiated pleomorphic sarcoma with coamplification of PDGFRA, VEGFR2 and KIT: A case report

  https://www.spandidos-publications.com/10.3892/mco.2024.2767

  This response suggests that the coamplification of PDGFRA, VEGFR2 and KIT may predict favourable outcomes in response to pazopanib. […] Pazopanib is an oral tyrosine kinase inhibitor (TKI) with activity against vascular endothelial growth factor receptor 1 (VEGFR1), VEGFR2, VEGFR3, platelet-derived growth factor receptor A (PDGFRA), PDGFRB, and stem cell factor receptor (KIT). […] Pazopanib has been approved for the treatment of advanced STSs. […] Therefore, we treated the patient with pazopanib with second-line therapy. […] Interestingly, CR was achieved and maintained for 3 years with pazopanib. […] The present case suggests that STSs with amplified PDGFRA, VEGFR2, and KIT are rare; however, patients with these features may benefit from pazopanib therapy. […] Therefore, further studies are required to confirm the benefits of pazopanib in patients with coamplification of PDGFRA, VEGFR2, and KIT, and to establish predictive markers for pazopanib sensitivity.

• #122 A primary undifferentiated pleomorphic sarcoma of the lumbosacral region harboring a LMNA-NTRK1 gene fusion with durable clinical response to crizotinib: a case report | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4749-z

  Perioperative chemotherapy was recently reported to be beneficial in terms of overall survival, and doxorubicin as a single agent or in combination with ifosfamide is the first choice of chemotherapy in cases of UPS metastasis. […] The observed effectiveness of crizotinib treatment provides clinical validation of this molecular target. […] Molecularly targeted therapy of these rare but aggressive lesions represents a novel treatment option that may lead to fewer toxic side effects and better clinical outcomes. […] Crizotinib is a multi-active kinase inhibitor that blocks TrkA autophosphorylation and cell growth in cells expressing NTRK1 fusion proteins. […] Based on the report of a minor response to crizotinib in a case of non-small cell lung cancer harboring a NTRK1 fusion as well as preclinical data, we started oral administration of crizotinib (450 mg QD) in the UPS patient described in this report. Over the follow-up period, the patient did not experience intolerable adverse effects from treatment and continued crizotinib monotherapy with no evidence of disease for more than 18 months as of July 2018.

• #123 A Randomized Phase III Trial of Doxorubicin + Pembrolizumab Versus Doxorubicin Alone for the Treatment of Undifferentiated Pleomorphic Sarcoma (UPS) and Related Poorly Differentiated Sarcomas | Dana-Farber Cancer Institute

  https://www.dana-farber.org/clinical-trials/24-411

  This phase II trial compares the effect of immunotherapy (pembrolizumab) plus chemotherapy (doxorubicin) to chemotherapy (doxorubicin) alone in treating patients with undifferentiated pleomorphic sarcoma (UPS) or a related poorly differentiated sarcoma that has spread from where it first started (primary site) to other places in the body (metastatic) or that cannot be removed by surgery (unresectable). […] Adding immunotherapy (pembrolizumab) to the standard chemotherapy (doxorubicin) may help patients with metastatic or unresectable UPS or a related poorly differentiated sarcoma live longer without having disease progression.

• #124 Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/s13569-020-00133-9

  Conventional cytotoxic agents and pazopanib are approved for advanced soft tissue sarcomas but have low response rates and modest survival benefits. […] Recently, immune checkpoint inhibitors have shown clinically meaningful activity. The combination of pazopanib and immunotherapy has shown synergism in various other malignancies but has not been fully explored in advanced soft tissue sarcomas. […] In view of significant symptoms, she was treated with pazopanib in combination with pembrolizumab. She had remarkable radiological and clinical improvement, with a manageable toxicity profile and an ongoing response at ten months of therapy. […] Undifferentiated pleomorphic sarcoma is an immunologically active subtype of soft tissue sarcoma, which is particularly amenable to immune checkpoint inhibitors. Pazopanib with immune checkpoint inhibitors is a well-tolerated, yet hitherto underexplored combination that may offer significant clinical benefit in advanced sarcomas; this finding warrants further evaluation in clinical trials.

• #125 Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report | Clinical Sarcoma Research | Full Text

  https://clinicalsarcomaresearch.biomedcentral.com/articles/10.1186/s13569-020-00133-9

  Given the need to achieve a rapid tumor response, and evidence for the combination therapy resulting in meaningful results with acceptable toxicity, we were enticed to offer it to our patient after a discussion of the potential risks and benefits. […] Although each agent’s individual contribution to the good response observed in this patient can only be confirmed or refuted in a randomized trial, the improvement was most likely attributable to the immune checkpoint inhibitor since objective responses with single agent pazopanib are uncommon and typically not well-sustained. […] Considering that this dual therapy is feasible, well tolerated and can result in sustained response, these findings merit further evaluation in prospectively designed randomized clinical trials.

• #126 A case of undifferentiated pleomorphic sarcoma in esophagus after multiple cancer treatments of surgery and chemoradiotherapy | Surgical Case Reports | Full Text

  https://surgicalcasereports.springeropen.com/articles/10.1186/s40792-022-01560-0

  Moreover, clinical studies of immune checkpoint inhibitor regimens such as pembrolizumab, nivolumab, and ipilimumab are being investigated. […] As mentioned above, chemotherapy for UPS may be useful in the future, but the optimal regimen remains unclear. […] There is no evidence of any effect of chemotherapy or radiotherapy on UPSE. […] In our case, postoperative adjuvant therapy was not prescribed after adequate discussion among multidisciplinary professionals and family members. […] The prognosis for UPS remains poor. […] However, a standard treatment for UPSE has not been established yet; therefore, further knowledge of UPSE is required. […] We report a rare case of UPSE, that was successfully treated with surgical resection. Currently, complete resection is the only treatment option for UPSE. An optimal treatment strategy using chemotherapy or radiotherapy should be established.

• #127

  https://ncs.navigatingcare.com/library/bone-osteo/learn/treatment-of-recurrent-osteosarcoma-and-undifferentiated-pleomorphic-sarcoma-ups-of-bone

  Treatment of recurrent osteosarcoma and UPS of bone may include the following: Surgery to remove cancer in all areas where it has spread. Chemotherapy and targeted therapy (sorafenib, regorafenib, lenvatinib, or everolimus). Samarium and radiation therapy. […] Treatment depends on the area and type of recurrence, for example: For tumors that have recurred in the same bone where the cancer started: Surgery. Radiation therapy. […] For tumors that have recurred in the lung only: Surgery. Chemotherapy. Targeted therapy. A clinical trial comparing two different types of surgery (thoracotomy and video-assisted thoracoscopic surgery) in treating patients with osteosarcoma that has spread to the lung. […] For tumors that have recurred in bones other than where the cancer started: Surgery. Samarium with or without stem cells, as palliative treatment to relieve pain and improve the quality of life. […] For tumors that have recurred twice: Surgery to remove the cancer and/or chemotherapy. […] Clinical trials for treatment of recurrent osteosarcoma and UPS of bone may include the following: A clinical trial of a new immune cell therapy.

• #128 Undifferentiated Pleomorphic Sarcoma

  https://sarcomaoncology.com/types-of-sarcoma/soft-tissue-sarcomas/uncertain-differentiation-undifferentiated-pleomorphic-sarcoma/

  Radiation therapy is used to kill or shrink tumor cells using high-energy radiation beams. A radiation oncologist can perform either external or internal radiation, depending on the location of the tumor. […] Chemotherapy uses strong medication to stop the growth of tumor cells. Your oncologist may also choose to perform chemotherapy before surgery. […] Clinical trials use new treatment approaches for uncertain differentiation sarcomas. You may need clinical trials or more cutting-edge personalized treatments when standard treatments are not effective. […] The treatment plan for this type of sarcoma is based on the tumor stage, prior treatments (if any), growth of the tumor, and biopsy analysis.

• #129 Undifferentiated Pleomorphic Sarcoma (UPS) – Rare Cancers Australia

  https://www.rarecancers.org.au/knowledgebase/cancer-types/undifferentiated-pleomorphic-sarcoma-ups/

  If a UPS is detected, it will be staged and graded based on size, metastasis, and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you. […] Treatment options for UPS may include: Surgery, potentially including: En bloc resection, Curettage, Limb-sparing surgery, Amputation, Bone graft. […] Radiation therapy, a treatment that uses controlled doses of radiation to damage or kill cancer cells. […] Chemotherapy, a cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells. […] Targeted therapy, medication that targets specific molecular features of cancer cells. […] Immunotherapy, a treatment that uses a person’s immune system to fight cancer. […] Clinical trials, research studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases. […] Palliative care, a variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.

• #130 Successful pazopanib treatment of undifferentiated pleomorphic sarcoma with coamplification of PDGFRA, VEGFR2 and KIT: A case report

  https://www.spandidos-publications.com/10.3892/mco.2024.2767

  Undifferentiated pleomorphic sarcoma (UPS) is a high-grade, aggressive soft tissue sarcoma (STS) with a poor prognosis, and no definitive or effective treatment is currently available for it. […] Pazopanib, an orally available multiple tyrosine kinase inhibitor, has been approved for the treatment of advanced STS. […] The patient exhibited a marked and sustained response to pazopanib. […] Palliative radiation therapy was administered to the left femur and second/fifth thoracic vertebrae to prevent fractures. […] After radiation therapy, the patient achieved a partial response after eight courses of doxorubicin. […] Hence, pazopanib was initiated as a second-line treatment. […] Notably, the retroperitoneal tumour shrank, and no new lesions developed for 3 years after the initiation of pazopanib treatment.

• #131 Osteosarcoma Treatment (PDQ®): Treatment – Patient Information [NCI] | The Children’s Hospital at Montefiore

  https://www.cham.org/health-library/article?id=ncicdr0000062949

  Treatment of newly diagnosed osteosarcoma and UPS that has spread to the lung may include chemotherapy followed by surgery to remove the primary cancer and more chemotherapy. Then, the doctor will remove the cancer in the lung and give additional chemotherapy. […] Treatment of newly diagnosed osteosarcoma and UPS that has spread to a distant bone or to both the lung and a distant bone may include: Chemotherapy followed by surgery to remove the primary cancer. More chemotherapy is given after surgery. This may be followed by surgery to remove cancer that has spread to other areas of the body. […] For tumors that have recurred in the same bone where the cancer started, treatment is surgery. […] For tumors that have recurred in the lung only, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy. […] For tumors that have recurred in bones other than where the cancer started, treatment may include: surgery to remove the tumor, chemotherapy, radiation therapy, samarium with or without stem cells, as palliative treatment to relieve pain and improve quality of life.

• #132 Long Duration Pembrolizumab for Metastatic Undifferentiated Pleomorphic Soft Tissue Sarcoma With Multimodality Therapy | Reuben | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/4237/3601

  While immunotherapy is an encouraging advancement, only certain sarcoma subtypes appear to respond. […] No guidelines yet support or refute chronic, long-term immunotherapy for sarcoma management. […] Lengthy or prolonged immunotherapy could thus be a consideration when efficacy continues to be seen over many years. […] Herein a case is described with prolonged treatment and substantial continued response and tolerability to pembrolizumab immunotherapy for a patient with undifferentiated pleomorphic sarcoma (UPS). […] Multidisciplinary care with palliative surgery and XRT occurred for occasional, non-responding metastases. […] A prolonged use of immunotherapy for the treatment of metastatic UPS is presented. […] It is also shown that despite two separate lung metastases which lost an immune response, others continued to respond and did so for many years after immunotherapy was started.

• #133 Long Duration Pembrolizumab for Metastatic Undifferentiated Pleomorphic Soft Tissue Sarcoma With Multimodality Therapy | Reuben | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/4237/3601

  While immunotherapy is an encouraging advancement, only certain sarcoma subtypes appear to respond. […] No guidelines yet support or refute chronic, long-term immunotherapy for sarcoma management. […] Lengthy or prolonged immunotherapy could thus be a consideration when efficacy continues to be seen over many years. […] Herein a case is described with prolonged treatment and substantial continued response and tolerability to pembrolizumab immunotherapy for a patient with undifferentiated pleomorphic sarcoma (UPS). […] Multidisciplinary care with palliative surgery and XRT occurred for occasional, non-responding metastases. […] A prolonged use of immunotherapy for the treatment of metastatic UPS is presented. […] It is also shown that despite two separate lung metastases which lost an immune response, others continued to respond and did so for many years after immunotherapy was started.

• #134 Undifferentiated pleomorphic sarcoma | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20344525/

  Radiation therapy uses high-powered beams of energy, such as X-rays or protons, to kill cancer cells. […] Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. It can be administered by pill or through a vein (intravenously), or both. […] Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die. […] Immunotherapy uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that help them hide. Immunotherapy works by interfering with that process. […] No alternative treatments have been found helpful in treating undifferentiated pleomorphic sarcoma. But some complementary and alternative treatments may relieve the symptoms you experience due to cancer or cancer treatment.

• #135 Undifferentiated pleomorphic sarcoma – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/undifferentiated-pleomorphic-sarcoma/

  Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die. […] Immunotherapy uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that help them hide. Immunotherapy works by interfering with that process. […] No alternative treatments have been found helpful in treating undifferentiated pleomorphic sarcoma. But some complementary and alternative treatments may relieve the symptoms you experience due to cancer or cancer treatment.

• #136 Undifferentiated Pleomorphic Sarcoma | Orthopedic Surgery | Patient Care | Montefiore Einstein

  https://montefioreeinstein.org/patient-care/services/orthopedics/specialties/orthopedic-oncology/pleomorphic-sarcoma

  Treatment for undifferentiated pleomorphic sarcoma generally involves surgery. When the tumor is small and found in just one part of the body, surgery is sometimes enough and no additional treatments are required. […] If the tumor is larger than five centimeters, the combined use of surgery and radiation therapy offers patients better control of their disease, meaning a lower risk of local recurrence. A variety of radiation techniques can be used, and the exact method of delivery is tailored to a patients individual circumstances and clinical needs. If the tumor is large or if it has metastasized, treatment will often also include chemotherapy. […] Novel therapies for undifferentiated pleomorphic sarcoma are being explored, often within the context of ongoing clinical trials. These include a wide variety of targeted agents designed to affect one or more specific pathways. The role of immunotherapy is being currently explored. […] Following the completion of treatment, patients typically require close monitoring for a period of five years. This includes physical examinations and periodic imaging studies. Many patients continue to follow up even after five years, though at a reduced rate.

• #137 Undifferentiated Pleomorphic Sarcoma | Orthopedic Surgery | Patient Care | Montefiore Einstein

  https://montefioreeinstein.org/patient-care/services/orthopedics/specialties/orthopedic-oncology/pleomorphic-sarcoma

  Treatment for undifferentiated pleomorphic sarcoma generally involves surgery. When the tumor is small and found in just one part of the body, surgery is sometimes enough and no additional treatments are required. […] If the tumor is larger than five centimeters, the combined use of surgery and radiation therapy offers patients better control of their disease, meaning a lower risk of local recurrence. A variety of radiation techniques can be used, and the exact method of delivery is tailored to a patients individual circumstances and clinical needs. If the tumor is large or if it has metastasized, treatment will often also include chemotherapy. […] Novel therapies for undifferentiated pleomorphic sarcoma are being explored, often within the context of ongoing clinical trials. These include a wide variety of targeted agents designed to affect one or more specific pathways. The role of immunotherapy is being currently explored. […] Following the completion of treatment, patients typically require close monitoring for a period of five years. This includes physical examinations and periodic imaging studies. Many patients continue to follow up even after five years, though at a reduced rate.

• #138 Undifferentiated pleomorphic sarcomas of the proximal femur, A case study

  https://meddocsonline.org/journal-of-case-reports-and-medical-images/Undifferentiated-pleomorphic-sarcomas-of-the-proximal-femur-A-case-study.html

  Pleomorphic sarcomas are a subtype of soft tissue sarcomas occurring most commonly in the proximal thigh, with a male preponderance, common in 7th and 8th decades of life. […] Though the grade, and level of local invasion, metastasis, and post surgical margins guide the tumor management, treatment modality is basically subdivided into surgery chemotherapy and radiation therapy. Risk of recurrence is present as with any tumor, so regular follow up, and evaluations are recommended. […] The treatment modalities are three fold, as in all cases of a bone malignancies, consist of excision, with wide margins the rest of treatment including chemotherapy and radiation therapy depends on the grade of the tumour, local invasion and metastasis. Lymph node involvement must be carefully assessed, not only for dissection but also to make sure no spread has occurred. Recurrence occurs in about half of the patients, common within the first 2 years of treatment. Increased risk of recurrence includes factors such -a mass size greater than 5 cm, a high-grade histology, a deep anatomical location of the tumour, poor resection of the original tumour. […] Even after appropriate treatment, the risk of recurrence is high, especially within a period of 2 years; therefore, regular follow-ups with imaging such as an MRI are mandatory.

• #139 Undifferentiated Pleomorphic Sarcoma | Orthopedic Surgery | Patient Care | Montefiore Einstein

  https://montefioreeinstein.org/patient-care/services/orthopedics/specialties/orthopedic-oncology/pleomorphic-sarcoma

  Treatment for undifferentiated pleomorphic sarcoma generally involves surgery. When the tumor is small and found in just one part of the body, surgery is sometimes enough and no additional treatments are required. […] If the tumor is larger than five centimeters, the combined use of surgery and radiation therapy offers patients better control of their disease, meaning a lower risk of local recurrence. A variety of radiation techniques can be used, and the exact method of delivery is tailored to a patients individual circumstances and clinical needs. If the tumor is large or if it has metastasized, treatment will often also include chemotherapy. […] Novel therapies for undifferentiated pleomorphic sarcoma are being explored, often within the context of ongoing clinical trials. These include a wide variety of targeted agents designed to affect one or more specific pathways. The role of immunotherapy is being currently explored. […] Following the completion of treatment, patients typically require close monitoring for a period of five years. This includes physical examinations and periodic imaging studies. Many patients continue to follow up even after five years, though at a reduced rate.

• #140 Undifferentiated Pleomorphic Sarcoma | Norton Children’s

  https://nortonchildrens.com/services/cancer/conditions/sarcoma/undifferentiated-pleomorphic-sarcoma/

  Our physicians are active researchers, conducting clinical trials and publishing their discoveries to advance medical knowledge. […] By staying at the forefront of research, our physicians are experienced with newly approved therapies and can provide access to trials of experimental treatments. […] Undifferentiated pleomorphic sarcoma typically is diagnosed after more common cancers have been ruled out. In addition to a physical exam, the physician may recommend imaging scans, such as CT scan or MRI, to get a detailed look at the mass, its structure and whether it is connected to other tissues. […] A definitive diagnosis is made with a biopsy. A sample of the tumor is removed either with a needle or surgically and sent to a lab for evaluation. […] Experience with soft tissue sarcomas is important in getting a correct diagnosis. Biopsies need to be performed in such a way that won’t complicate future surgery to remove the tumor. Undifferentiated pleomorphic sarcoma has the best chance of being cured when it’s treated by pediatric cancer specialists. Young adults may benefit from treatment at a children’s hospital instead of an adult-service hospital because of the care team’s expertise specifically in childhood diseases.

• #141 Undifferentiated pleomorphic sarcomas of the proximal femur, A case study

  https://meddocsonline.org/journal-of-case-reports-and-medical-images/Undifferentiated-pleomorphic-sarcomas-of-the-proximal-femur-A-case-study.html

  Pleomorphic sarcomas are a subtype of soft tissue sarcomas occurring most commonly in the proximal thigh, with a male preponderance, common in 7th and 8th decades of life. […] Though the grade, and level of local invasion, metastasis, and post surgical margins guide the tumor management, treatment modality is basically subdivided into surgery chemotherapy and radiation therapy. Risk of recurrence is present as with any tumor, so regular follow up, and evaluations are recommended. […] The treatment modalities are three fold, as in all cases of a bone malignancies, consist of excision, with wide margins the rest of treatment including chemotherapy and radiation therapy depends on the grade of the tumour, local invasion and metastasis. Lymph node involvement must be carefully assessed, not only for dissection but also to make sure no spread has occurred. Recurrence occurs in about half of the patients, common within the first 2 years of treatment. Increased risk of recurrence includes factors such -a mass size greater than 5 cm, a high-grade histology, a deep anatomical location of the tumour, poor resection of the original tumour. […] Even after appropriate treatment, the risk of recurrence is high, especially within a period of 2 years; therefore, regular follow-ups with imaging such as an MRI are mandatory.

• #142 Undifferentiated sarcoma – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/undifferentiated-sarcoma/

  Living with UPS requires a proactive approach to treatment and lifestyle management. Early detection and treatment are crucial for improving prognosis and quality of life. […] Regular follow-up appointments are essential, as UPS can recur after treatment. Patients should maintain open communication with their healthcare providers to monitor their condition and adjust treatment plans as necessary. […] Research continues to explore prognostic factors and treatment options for UPS. The development of a nomogram for predicting three- and five-year overall survival rates offers a tool for clinicians and patients to make informed decisions. […] While surgery and radiotherapy are standard treatments, ongoing studies aim to refine these approaches to improve patient outcomes. […] Undifferentiated pleomorphic sarcoma (UPS) presents significant treatment challenges due to its aggressive nature and potential for metastasis. The cornerstone of treatment is surgical excision, aiming for wide or radical removal of the tumor to minimize recurrence risks. Radiation therapy, including advanced methods like proton therapy and intraoperative radiation therapy, complements surgery by targeting residual cancer cells while sparing healthy tissue. Chemotherapy is employed in advanced cases, with drugs such as anthracyclines and ifosfamide being commonly used. Targeted therapies and immunotherapy, including pembrolizumab, are emerging as promising options by addressing specific cancer cell abnormalities and enhancing the immune response. […] Regular monitoring and follow-up are crucial for early detection of recurrence and maintaining quality of life.

• #143 Undifferentiated pleomorphic sarcomas of the proximal femur, A case study

  https://meddocsonline.org/journal-of-case-reports-and-medical-images/Undifferentiated-pleomorphic-sarcomas-of-the-proximal-femur-A-case-study.html

  Pleomorphic sarcomas are a subtype of soft tissue sarcomas occurring most commonly in the proximal thigh, with a male preponderance, common in 7th and 8th decades of life. […] Though the grade, and level of local invasion, metastasis, and post surgical margins guide the tumor management, treatment modality is basically subdivided into surgery chemotherapy and radiation therapy. Risk of recurrence is present as with any tumor, so regular follow up, and evaluations are recommended. […] The treatment modalities are three fold, as in all cases of a bone malignancies, consist of excision, with wide margins the rest of treatment including chemotherapy and radiation therapy depends on the grade of the tumour, local invasion and metastasis. Lymph node involvement must be carefully assessed, not only for dissection but also to make sure no spread has occurred. Recurrence occurs in about half of the patients, common within the first 2 years of treatment. Increased risk of recurrence includes factors such -a mass size greater than 5 cm, a high-grade histology, a deep anatomical location of the tumour, poor resection of the original tumour. […] Even after appropriate treatment, the risk of recurrence is high, especially within a period of 2 years; therefore, regular follow-ups with imaging such as an MRI are mandatory.

• #144 Undifferentiated pleomorphic sarcomas of the proximal femur, A case study

  https://meddocsonline.org/journal-of-case-reports-and-medical-images/Undifferentiated-pleomorphic-sarcomas-of-the-proximal-femur-A-case-study.html

  Pleomorphic sarcomas are a subtype of soft tissue sarcomas occurring most commonly in the proximal thigh, with a male preponderance, common in 7th and 8th decades of life. […] Though the grade, and level of local invasion, metastasis, and post surgical margins guide the tumor management, treatment modality is basically subdivided into surgery chemotherapy and radiation therapy. Risk of recurrence is present as with any tumor, so regular follow up, and evaluations are recommended. […] The treatment modalities are three fold, as in all cases of a bone malignancies, consist of excision, with wide margins the rest of treatment including chemotherapy and radiation therapy depends on the grade of the tumour, local invasion and metastasis. Lymph node involvement must be carefully assessed, not only for dissection but also to make sure no spread has occurred. Recurrence occurs in about half of the patients, common within the first 2 years of treatment. Increased risk of recurrence includes factors such -a mass size greater than 5 cm, a high-grade histology, a deep anatomical location of the tumour, poor resection of the original tumour. […] Even after appropriate treatment, the risk of recurrence is high, especially within a period of 2 years; therefore, regular follow-ups with imaging such as an MRI are mandatory.

• #145 Undifferentiated pleomorphic sarcomas of the proximal femur, A case study

  https://meddocsonline.org/journal-of-case-reports-and-medical-images/Undifferentiated-pleomorphic-sarcomas-of-the-proximal-femur-A-case-study.html

  Pleomorphic sarcomas are a subtype of soft tissue sarcomas occurring most commonly in the proximal thigh, with a male preponderance, common in 7th and 8th decades of life. […] Though the grade, and level of local invasion, metastasis, and post surgical margins guide the tumor management, treatment modality is basically subdivided into surgery chemotherapy and radiation therapy. Risk of recurrence is present as with any tumor, so regular follow up, and evaluations are recommended. […] The treatment modalities are three fold, as in all cases of a bone malignancies, consist of excision, with wide margins the rest of treatment including chemotherapy and radiation therapy depends on the grade of the tumour, local invasion and metastasis. Lymph node involvement must be carefully assessed, not only for dissection but also to make sure no spread has occurred. Recurrence occurs in about half of the patients, common within the first 2 years of treatment. Increased risk of recurrence includes factors such -a mass size greater than 5 cm, a high-grade histology, a deep anatomical location of the tumour, poor resection of the original tumour. […] Even after appropriate treatment, the risk of recurrence is high, especially within a period of 2 years; therefore, regular follow-ups with imaging such as an MRI are mandatory.

• #146 Undifferentiated pleomorphic sarcomas of the proximal femur, A case study

  https://meddocsonline.org/journal-of-case-reports-and-medical-images/Undifferentiated-pleomorphic-sarcomas-of-the-proximal-femur-A-case-study.html

  Pleomorphic sarcomas are a subtype of soft tissue sarcomas occurring most commonly in the proximal thigh, with a male preponderance, common in 7th and 8th decades of life. […] Though the grade, and level of local invasion, metastasis, and post surgical margins guide the tumor management, treatment modality is basically subdivided into surgery chemotherapy and radiation therapy. Risk of recurrence is present as with any tumor, so regular follow up, and evaluations are recommended. […] The treatment modalities are three fold, as in all cases of a bone malignancies, consist of excision, with wide margins the rest of treatment including chemotherapy and radiation therapy depends on the grade of the tumour, local invasion and metastasis. Lymph node involvement must be carefully assessed, not only for dissection but also to make sure no spread has occurred. Recurrence occurs in about half of the patients, common within the first 2 years of treatment. Increased risk of recurrence includes factors such -a mass size greater than 5 cm, a high-grade histology, a deep anatomical location of the tumour, poor resection of the original tumour. […] Even after appropriate treatment, the risk of recurrence is high, especially within a period of 2 years; therefore, regular follow-ups with imaging such as an MRI are mandatory.

• #147 Management of undifferentiated high‐grade pleomorphic sarcoma of parotid region in elderly women

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6406222/

  Wide resection with free margins is required, but a complete excision of head and neck localizations can be challenging due to the infiltrative nature and consequent invasion to critical structures. […] Adjuvant radiation therapy is generally recommended if margins are positive or close, if the lesion is highgrade, in large tumors (more than 5 cm of diameter), or in the presence of metastatic lymph nodes. […] The use of chemotherapy and/or radiotherapy as the primary treatment modality has been less successful; chemoresistance is not uncommon, and at present, there is no specific trial showing significant improvement of survival after adjuvant systemic chemotherapy. […] Overall survival rates for UHPS range from 30% to 74%. Positive or close surgical margins, after excision, correlate with an increased local recurrence rate and reduce overall survival.

• #148 Undifferentiated pleomorphic sarcoma of Buttock – Macmillan Online Community

  https://community.macmillan.org.uk/cancer_types/soft-tissue-sarcomas-forum/f/diagnosis-and-treatment/258860/undifferentiated-pleomorphic-sarcoma-of-buttock

  my husband is 66 and in June last year he said he had a bit of an ache in top half of his leg – said he felt like hed been sitting on a coach seat for too long. […] Surgeon told us that she would have preferred to do radiotherapy before surgery but team thought it needed removing asap. […] He has now just finished 6 weeks of radiotherapy post surgery. […] We have already been told because of several factors grade, size and aggressive nature of the sarcoma there is a strong possibility of recurrence and/either mestatase within 2 years. […] I too had 6 weeks radiotherapy […] The hospital because of the type your husband had will keep close eye on him because of its rarity and how aggressive they can be. I’m glad he has finished his treatment and is on the road to recovery.

• #149 Treatment of Soft Tissue Sarcomas, by Stage | American Cancer Society

  https://www.cancer.org/cancer/types/soft-tissue-sarcoma/treating/by-stage.html

  The best chance to cure a soft tissue sarcoma is to remove it with surgery, so surgery is part of the treatment for all soft tissue sarcomas whenever possible. It’s important that your surgeon and other doctors are experienced in the treatment of sarcomas. These tumors are hard to treat and require both experience and expertise. Studies have shown that people with sarcomas tend to have better outcomes when they’re treated at specialized cancer centers that have experience in sarcoma treatment. […] But in recent years, as doctors have learned more about the differences between the types, newer targeted therapy and immunotherapy drugs have become important treatment options for some types of advanced soft tissue sarcomas. […] For all stage II and III sarcomas, removing the tumor with surgery is the main treatment. Lymph nodes will also be removed if they might contain cancer. Radiation may be given after surgery.

• #150 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  In the setting of distant metastasis, surgery may be associated with long-term disease-free survival in patients with pulmonary metastasis and optimal underlying disease biology. […] Complete staging and treatment planning by a multidisciplinary team of cancer specialists is required to determine the optimal treatment for patients with soft tissue sarcoma. […] The role of chemotherapy is not well defined. […] There is evidence that favorable clinical outcomes may be associated with referral to a specialized sarcoma treatment center. […] Chemotherapy remains controversial, and its potential benefits should be considered in the context of the short- and long-term toxicities of the chemotherapy regimen. […] The role of adjuvant chemotherapy remains controversial. […] Neoadjuvant chemotherapy with or without radiation therapy has shown response rates of 17% to 32%, 10-year RFS rates of up to 58%, and 10-year OS rates of up to 64%.

• #151 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Surgical resection is the mainstay of therapy for soft tissue sarcomas. […] In some small low-grade tumors of the extremities or trunk, surgery alone can be performed without the use of radiation. […] Patient selection factors may vary among surgeons. In general, surgery alone is considered in patients with low-grade tumors of the extremity or superficial trunk that are 5 cm or smaller in diameter (T1) and have microscopically negative surgical margins. […] When feasible, wide-margin function-sparing surgical excision is the cornerstone of effective treatment for extremity tumors. […] Local control of high-grade soft tissue sarcomas of the trunk and the head and neck can be achieved with surgery in combination with radiation therapy. […] Effective treatment of retroperitoneal sarcomas requires removal of all gross disease while sparing adjacent viscera not invaded by tumor.

• #152 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK570612/

  The standard of care for head, neck, trunk, and extremities UPS is en bloc surgical excision with microscopically negative margins, particularly for stage I tumors. This may be achieved by wide local excision with 2 cm margins of uninvolved tissue, albeit some cases may pose greater difficulty due to the involvement of important neurovascular structures. Postoperative radiotherapy (RT) is indicated when margins are close to the tumor ( 1 cm), microscopically positive, involve bone, major blood vessels, or nerves. […] Effective RT should cover a 5 cm margin and can be administered through external beam RT (EBRT) (50 Gy), intraoperative RT (IORT) (10-16 Gy), low-dose brachytherapy (LDR) (45 Gy), or its high-dose equivalent (HDR). […] Broadly, chemotherapy with different antineoplastics is employed for advanced, widespread, or irresectable stages of various STSs.

• #153 Soft Tissue Sarcoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq

  Anthracyclines remain the first-line class of systemic therapy in managing most locally advanced and metastatic soft tissue sarcoma. […] The combination of gemcitabine and docetaxel is used as second-line therapy in treating patients with soft tissue sarcoma. […] Immune checkpoint inhibitors have shown some activity in selected soft tissue sarcoma subtypes, but their use cannot be routinely recommended. […] Treatment options for recurrent soft tissue sarcoma include surgery with or without radiation therapy, chemotherapy and targeted therapy, and immune checkpoint inhibitor therapy.

• #154 High-Grade Pleomorphic Sarcomas Treated with Immune Checkpoint Blockade: The MD Anderson Cancer Center Experience

  https://www.mdpi.com/2072-6694/16/9/1763

  Undifferentiated pleomorphic sarcomas (UPSs) represent 10–20% of all soft tissue sarcomas (STSs) and have quickly emerged as one of the more immune-sensitive types. […] This is a retrospective, observational study of all patients with metastatic high-grade pleomorphic sarcomas treated with FDA-approved ICB at MD Anderson Cancer Center intended to describe the efficacy and toxicity of ICB in this particular group of patients. […] The mainstay of advanced/metastatic treatment for STSs in general and UPSs in particular consists of systemic therapies, with doxorubicin-based and gemcitabine-based treatments used as front-line treatment, with a median progression-free survival (PFS) of around 6 months and a median overall survival (OS) of around 18–20 months. […] UPS has quickly emerged as one of the more immune-sensitive types of STS, which was identified during the first immune checkpoint blockade (ICB) clinical trials in patients with advanced and pretreated STS with an objective response rate (ORR) of 20–40% and a median PFS of 3 months.

• #155 Moving the Needle in Undifferentiated Pleomorphic Sarcoma: Overview of Paradigm

  https://www.onclive.com/view/moving-the-needle-in-undifferentiated-pleomorphic-sarcoma-overview-of-paradigm

  In this episode, experts discuss the latest advancements in the multimodal treatment of undifferentiated pleomorphic sarcoma of the extremities. […] Recently, immunotherapy, radiation therapy, and surgery are emerging as a growing and promising combination for the treatment of UPS of the extremities. Pembrolizumab [Keytruda], an antiPD-1 immune checkpoint inhibitor, has shown efficacy in various malignancies, including soft tissue sarcomas. The Stand Up to Cancer Sarcoma Alliance for Research through Collaboration [SARC] 032 trial demonstrated that the addition of pembrolizumab to preoperative radiotherapy and surgery significantly improved disease-free survival in patients with stage III UPS of the extremities compared with radiation and surgery alone. […] The multimodal approach addresses both local and systemic disease control, offering a comprehensive treatment strategy for stage III UPS of the extremities. The combination of pembrolizumab, radiotherapy, and surgery represents a significant advancement in the management of this aggressive sarcoma subtype.

• #156 Pembrolizumab Plus Standard Therapy Improves Outcomes in Stage III Soft Tissue Sarcoma

  https://www.oncnursingnews.com/view/pembrolizumab-plus-standard-therapy-improves-outcomes-in-stage-iii-soft-tissue-sarcoma

  Pembrolizumab, added to preoperative radiation and surgery, prolonged disease-free survival in patients with soft tissue sarcoma of the extremity. […] Adding pembrolizumab standard treatment (radiation and surgery) significantly improved outcomes for patients with advanced soft tissue sarcoma in the limbs. […] Findings from SU2C-SARC032 indicate that pembrolizumab is a promising new treatment option for these patients and suggest a path for even greater therapeutic effect by further optimizing immunotherapy. […] Based on these findings, as well as prior data demonstrating the synergistic effects of radiation therapy and immune checkpoint blockade on local and distant tumor response, the SU2C-SARC032 trial investigators hypothesized that adding pembrolizumab to preoperative radiation therapy and surgery could improve DFS for patients with stage III soft tissue sarcoma. […] Therefore, for patients with resectable, intermediate-grade or high-grade undifferentiated pleomorphic sarcoma or liposarcoma measuring more than 5 cm, SU2C-SARC032 establishes pembrolizumab as a new treatment option.

• #157 Successful pazopanib treatment of undifferentiated pleomorphic sarcoma with coamplification of PDGFRA, VEGFR2 and KIT: A case report

  https://www.spandidos-publications.com/10.3892/mco.2024.2767

  This response suggests that the coamplification of PDGFRA, VEGFR2 and KIT may predict favourable outcomes in response to pazopanib. […] Pazopanib is an oral tyrosine kinase inhibitor (TKI) with activity against vascular endothelial growth factor receptor 1 (VEGFR1), VEGFR2, VEGFR3, platelet-derived growth factor receptor A (PDGFRA), PDGFRB, and stem cell factor receptor (KIT). […] Pazopanib has been approved for the treatment of advanced STSs. […] Therefore, we treated the patient with pazopanib with second-line therapy. […] Interestingly, CR was achieved and maintained for 3 years with pazopanib. […] The present case suggests that STSs with amplified PDGFRA, VEGFR2, and KIT are rare; however, patients with these features may benefit from pazopanib therapy. […] Therefore, further studies are required to confirm the benefits of pazopanib in patients with coamplification of PDGFRA, VEGFR2, and KIT, and to establish predictive markers for pazopanib sensitivity.

• #158 A primary undifferentiated pleomorphic sarcoma of the lumbosacral region harboring a LMNA-NTRK1 gene fusion with durable clinical response to crizotinib: a case report | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4749-z

  This case provides robust evidence for the importance of molecular evaluation in cases of these rare but aggressive lesions and stresses the need for the development of drugs for better molecularly targeted STS treatment, especially when standard-of-care options have been exhausted or treatment options are unavailable.

• #159 Undifferentiated Pleomorphic Sarcoma | Norton Children’s

  https://nortonchildrens.com/services/cancer/conditions/sarcoma/undifferentiated-pleomorphic-sarcoma/

  Our physicians are active researchers, conducting clinical trials and publishing their discoveries to advance medical knowledge. […] By staying at the forefront of research, our physicians are experienced with newly approved therapies and can provide access to trials of experimental treatments. […] Undifferentiated pleomorphic sarcoma typically is diagnosed after more common cancers have been ruled out. In addition to a physical exam, the physician may recommend imaging scans, such as CT scan or MRI, to get a detailed look at the mass, its structure and whether it is connected to other tissues. […] A definitive diagnosis is made with a biopsy. A sample of the tumor is removed either with a needle or surgically and sent to a lab for evaluation. […] Experience with soft tissue sarcomas is important in getting a correct diagnosis. Biopsies need to be performed in such a way that won’t complicate future surgery to remove the tumor. Undifferentiated pleomorphic sarcoma has the best chance of being cured when it’s treated by pediatric cancer specialists. Young adults may benefit from treatment at a children’s hospital instead of an adult-service hospital because of the care team’s expertise specifically in childhood diseases.

• #160 Undifferentiated Pleomorphic Sarcoma: Long-Term Follow-Up from a Large | CMAR

  https://www.dovepress.com/undifferentiated-pleomorphic-sarcoma-long-term-follow-up-from-a-large–peer-reviewed-fulltext-article-CMAR

  Our study aimed to describe the clinical features of undifferentiated pleomorphic sarcoma (UPS) and identify the predictors of poor outcomes. […] Aggressive surgical resection remained the principal treatment. Local recurrence occurred in 13-42% of patients and distant metastasis could be found in 31-35% of patients despite aggressive surgery. […] Currently, it is urgent to clarify and update the prognosis of patients suffering from UPS with a long period of follow-up owing to changes in classification criteria. […] Primary treatment of UPS should be conducted by experts in large sarcoma center. Wide surgical margin provides sufficient control of the disease recurrence. […] In our study, tumor presentation, tumor size and important structures involved were independent prognostic factors associated with OS, which justified the early and primary treatment was very important.

• #161 Undifferentiated Pleomorphic Sarcoma | Norton Children’s

  https://nortonchildrens.com/services/cancer/conditions/sarcoma/undifferentiated-pleomorphic-sarcoma/

  Undifferentiated pleomorphic sarcoma is a rare cancer that usually forms in soft tissue in the legs, arms or abdomen, but it can form in any soft tissue or bone anywhere in the body. […] Also called malignant fibrous histiocytoma or undifferentiated sarcoma, this aggressive cancer can grow fast and often spreads to other parts of the body, including the lungs. […] The survival rate for undifferentiated pleomorphic sarcoma is 60% at five years and 48% at 10 years, according to a 2019 study. As with all cancers, early detection improves the survival rate. By their nature, long-term survival rates don’t reflect the latest advancements in treatment and may underestimate current survival chances. […] The board-certified and fellowship-trained oncologists at Norton Children’s Cancer Institute come together at regular conferences to share viewpoints from various perspectives that help determine the best course of treatment. It’s like getting second, third and fourth opinions all at once.

• #162 Undifferentiated Pleomorphic Sarcoma – SFA

  https://curesarcoma.org/sarcoma-subtypes/undifferentiated-pleomorphic-sarcoma/

  Patients with UPS of bone are generally treated with chemotherapy and complete en bloc resection. […] Patients with localized disease and adequate therapy have a 5-year survival rate of 50–67%. […] Pulmonary metastases are common, occurring in approximately 35–50% of cases. […] Secondary UPS and metastatic disease are associated with a poorer prognosis. […] Incomplete expression of myogenic markers is not thought to affect the prognosis.

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