Materiały źródłowe

• #1 Undifferentiated pleomorphic sarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/undifferentiated-pleomorphic-sarcoma/symptoms-causes/syc-20389554

  Undifferentiated pleomorphic sarcoma (UPS) is a rare type of cancer that begins mostly in the soft tissues of the body. […] Treatment for UPS depends on the location of the cancer, but often involves surgery, radiation and drug treatments. […] Make an appointment with a doctor if you develop any persistent signs or symptoms that worry you. […] It’s not clear what causes undifferentiated pleomorphic sarcoma. […] Doctors know this cancer begins when a cell develops changes in its DNA. […] Factors that may increase the risk of undifferentiated pleomorphic sarcoma include older age and previous radiation therapy. […] Most people who develop undifferentiated pleomorphic sarcoma have no known risk factors, and many people who have risk factors never develop the cancer.

• #2 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  It can be exceedingly difficult to distinguish a UPS from a sarcomatoid carcinoma. […] Depending on definitional criteria, UPS still accounts for a significant proportion of sarcomas occurring in late adult life. […] UPS is characteristically a tumor of late adult life, with most cases occurring in persons between the ages of 50 and 70 years. […] The classic form of UPS has a highly variable morphologic pattern and shows frequent transitions from storiform to pleomorphic areas, although the emphasis in most tumors is on haphazardly arranged pleomorphic zones. […] The role of immunohistochemistry in the diagnosis of UPS has traditionally been an ancillary one, primarily serving as a means to exclude other pleomorphic tumors. […] More recently, a number of studies using comparative genomic hybridization have evaluated UPS and compared the findings to those of other pleomorphic sarcomas. […] The vast majority of UPS are high-grade lesions having a local recurrence rate ranging from 19-31%, a metastatic rate of 31-35%, and a 5-year survival of 65-70%. […] In summary, pleomorphic malignant neoplasms are commonly encountered by surgical pathologists.

• #3 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  Undifferentiated pleomorphic sarcoma (UPS), also termed pleomorphic myofibrosarcoma, high-grade myofibroblastic sarcoma, and high-grade myofibrosarcoma, is characterized by the World Health Organization (WHO) as a rare, poorly differentiated neoplasm (i.e., an abnormal growth of cells that have an unclear identity and/or cell of origin). WHO classified it as one of the undifferentiated/unclassified sarcomas in the category of tumors of uncertain differentiation. […] UPS is considered a diagnosis that defies formal sub-classification after thorough histologic, immunohistochemical, and ultrastructural examinations fail to identify the type of cells involved. […] The diagnosis of UPS depends on finding non-specific, undifferentiated tumor cells that have features suggestive of UPS and not features of other tumor types that also consist of pleomorphic, undifferentiated cells. The features primarily involve the expression of certain proteins by the tumor cells. The identifying proteins for UPS tumor cells are given in the preceding section.

• #4 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

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

  The undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a high-grade soft-tissue sarcoma (STS). […] At present, UPS is diagnosed by excluding other well-classified STSs. […] Histopathology examination is essential for the diagnosis of UPS. […] The definitive diagnosis of UPS is confirmed by excluding other malignancies with a panel of immunohistochemical markers. […] Once the diagnosis has been confirmed with histopathology and immunohistochemistry, evaluation and workup include imaging of the primary tumor, lymph nodes, and distant metastases. […] 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. […] The most common differential diagnoses of UPS are other types of STSs, which must be distinguished by clinical history, physical examination, and immunohistochemistry markers. […] Trunk and extremities UPS is staged according to the TNM and histologic grade (G). […] Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. […] Follow-up visits are important to detect local recurrences or metastasis.

• #5

  https://link.springer.com/article/10.1007/s10353-009-0474-9

  BACKGROUND: For many years malignant fibrous histiocytoma („MFH”) was accepted to be the most common soft tissue sarcoma in adults, its histiogenesis, however, remained controversial. […] RESULTS: Within the last two decades several studies demonstrated that „MFH” has been used as a „waste paper basket” for several better classifiable, poorly differentiated soft tissue sarcomas. Therefore, reclassification of these tumors could led to better prognostification. […] CONCLUSIONS: In the 21st century with the insight of immunohistochemistry as well as improved cytogenetic and molecular diagnostic the term high-grade undifferentiated pleomorphic sarcoma (so-called „MFH”) should be restricted to a group of soft tissue malignancies without a specific line of differentiation. Better classification of various high-grade sarcomas may contribute to more specific treatment options and hence lead to improved survival for patients.

• #6 Undifferentiated Pleomorphic Sarcoma

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

  Uncertain differentiation refers to a group of rare soft tissue sarcomas that do not resemble other sarcoma types or normal cells under a microscope. These types of sarcoma are usually hard to diagnose. […] We treat various subtypes of uncertain differentiation, including undifferentiated pleomorphic sarcoma, a unique high-grade aggressive sarcoma because of its cellular makeup. […] Undifferentiated pleomorphic sarcoma, formerly called malignant fibrous histiocytoma or MFH cancer, is a high-grade aggressive sarcoma that typically occurs in the arms or legs. […] The name comes from the way the tumor cells appear under the microscope. Undifferentiated means the tumor cells lack any clear resemblance to normal cells and it is difficult to tell where in the body they originated from their appearance.

• #7 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. […] One of the most common symptoms is a growing lump in the leg that can become painful. The cancer also may be found in the arms and abdomen, or anywhere in the body. Fever and weight loss also can be symptoms. Like many childhood and adolescent cancers, undifferentiated pleomorphic sarcoma can have vague symptoms that can be easily overlooked or attributed to other causes. An accurate diagnosis requires oncologists with skill, experience and knowledge of the latest discoveries. Consulting with a pediatric cancer specialist offers the best opportunity for early diagnosis and successful treatment.

• #8 Undifferentiated Pleomorphic Sarcoma

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

  Undifferentiated pleomorphic sarcoma is a malignant sarcoma that grows quickly and spreads to other parts of the body, including the lungs. […] Diagnosis of uncertain differentiation sarcoma depends on where the tumor has formed in your body. Your doctor may ask for the following tests: […] In a biopsy, a sample tissue from the tumor site is taken to determine the nature of the tumor, such as its size and whether it is benign or malignant. […] The treatment for uncertain differentiation sarcoma comprises a personalized multimodal approach. […] 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.

• #9 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  It can be exceedingly difficult to distinguish a UPS from a sarcomatoid carcinoma. […] Depending on definitional criteria, UPS still accounts for a significant proportion of sarcomas occurring in late adult life. […] UPS is characteristically a tumor of late adult life, with most cases occurring in persons between the ages of 50 and 70 years. […] The classic form of UPS has a highly variable morphologic pattern and shows frequent transitions from storiform to pleomorphic areas, although the emphasis in most tumors is on haphazardly arranged pleomorphic zones. […] The role of immunohistochemistry in the diagnosis of UPS has traditionally been an ancillary one, primarily serving as a means to exclude other pleomorphic tumors. […] More recently, a number of studies using comparative genomic hybridization have evaluated UPS and compared the findings to those of other pleomorphic sarcomas. […] The vast majority of UPS are high-grade lesions having a local recurrence rate ranging from 19-31%, a metastatic rate of 31-35%, and a 5-year survival of 65-70%. […] In summary, pleomorphic malignant neoplasms are commonly encountered by surgical pathologists.

• #10 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. […] One of the most common symptoms is a growing lump in the leg that can become painful. The cancer also may be found in the arms and abdomen, or anywhere in the body. Fever and weight loss also can be symptoms. Like many childhood and adolescent cancers, undifferentiated pleomorphic sarcoma can have vague symptoms that can be easily overlooked or attributed to other causes. An accurate diagnosis requires oncologists with skill, experience and knowledge of the latest discoveries. Consulting with a pediatric cancer specialist offers the best opportunity for early diagnosis and successful treatment.

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

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

  Diagnosis for undifferentiated pleomorphic sarcoma usually starts with a review of your symptoms and a physical examination. This cancer is often diagnosed after other types of cancer have been ruled out. […] Tests and procedures may include: […] To make a definitive diagnosis, your doctor collects a sample of the tumor tissue and sends it to a lab for testing. […] In the lab, doctors trained in analyzing body tissues (pathologists) examine the sample to determine the types of cells involved and whether the cells are likely to be aggressive. This information helps rule out other types of cancer and guides your treatment. […] Determining the type of biopsy needed and the specifics of how it should be performed requires careful planning by the medical team. […] Undifferentiated pleomorphic sarcoma is rare and often requires complex care. It’s best treated by someone who has significant experience with it, which often means an academic or multispecialized cancer center. […] What kinds of tests do I need to confirm the diagnosis? Do these tests require any special preparation? […] What stage is the sarcoma? […] What treatments are available for undifferentiated pleomorphic sarcoma, and which do you recommend?

• #12 Undifferentiated Pleomorphic Sarcoma | Sarcoma UK

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

  Undifferentiated pleomorphic sarcoma, also known as UPS, is one of the most common types of sarcoma. […] A specialist doctor will diagnose UPS through a series of tests. These may include: […] UPS is a diagnosis of exclusion. This means that the tumour shows no identifiable characteristics for the doctors to be able to diagnose it as a specific sarcoma type. A doctor will look to see if the biopsy looks like a specific sarcoma subtype and if it doesn’t, then it will be diagnosed as UPS.

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

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

  Diagnosis for undifferentiated pleomorphic sarcoma usually starts with a review of your symptoms and a physical examination. This cancer is often diagnosed after other types of cancer have been ruled out. […] Tests and procedures may include: […] To make a definitive diagnosis, your doctor collects a sample of the tumor tissue and sends it to a lab for testing. […] In the lab, doctors trained in analyzing body tissues (pathologists) examine the sample to determine the types of cells involved and whether the cells are likely to be aggressive. This information helps rule out other types of cancer and guides your treatment. […] Determining the type of biopsy needed and the specifics of how it should be performed requires careful planning by the medical team. […] Undifferentiated pleomorphic sarcoma is rare and often requires complex care. It’s best treated by someone who has significant experience with it, which often means an academic or multispecialized cancer center. […] What kinds of tests do I need to confirm the diagnosis? Do these tests require any special preparation? […] What stage is the sarcoma? […] What treatments are available for undifferentiated pleomorphic sarcoma, and which do you recommend?

• #14 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. […] Your healthcare provider will start by reviewing your health and biological family history. Theyll also ask questions about your symptoms, like when they started and whether theyve changed over time. Theyll also do: […] A physical exam. Theyll check the size of the lump and feel its location and how hard it is. […] Imaging tests. These typically include X-rays, an ultrasound and/or an MRI. […] Biopsy. Your provider will take a small sample of tissue and send it to a pathologist for testing. This involves looking at the sample under a microscope. […] Its 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.

• #15 Undifferentiated pleomorphic sarcoma | ABC Medical Center

  https://centromedicoabc.com/en/padecimientos/undifferentiated-pleomorphic-sarcoma/

  The specialist, in addition to analyzing your clinical history and symptoms, will perform a physical examination in which they will pay special attention to the affected area to try to detect the size and depth of the tumor, as well as identify if it is connected to nearby tissues and if there is inflammation or damage to the nerves. […] Also, the following tests will be requested to confirm the diagnosis and rule out any other anomaly: […] Tissue biopsy. […] X-rays. […] MRI. […] Computed tomography. […] Positron emission tomography.

• #16 Undifferentiated Pleomorphic Sarcoma (UPS): Causes, Symptoms

  https://www.prepladder.com/neet-pg-study-material/pathology/undifferentiated-pleomorphic-sarcoma-ups-causes-symptoms-risk-factors-diagnosis-and-treatment

  The diagnosis of undifferentiated pleomorphic sarcoma usually occurs following a physical examination and symptom assessment. This malignancy is often detected after all other kinds of cancer have been identified and excluded. […] Tests and procedures could include: […] Physical assessment: Your doctor will want to know when your symptoms started and if they have changed over time. He or she will examine the affected area to obtain a better idea of the size and depth of the growth, its relationship to surrounding tissues, and the presence of any swelling or nerve injury. […] Imaging tests: To get more information about your disease and to take photographs of the affected area, your doctor can suggest imaging testing. Imaging examinations encompass positron emission tomography (PET) scans, CT, MRI, and X-rays.

• #17 Undifferentiated pleomorphic sarcoma in the anterior mediastinum: a case report and literature review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11518729/

  Primary undifferentiated high-grade pleomorphic sarcoma (UPS) in the mediastinum is exceptionally rare. […] Undifferentiated pleomorphic sarcoma (UPS) is a diagnosis of exclusion that requires the elimination of various other tumor types prior to confirmation. […] Radiology plays a crucial role in initial diagnosis, long-term follow-up, and the evaluation of many treatment-related complications. […] However, it lacks clear diagnostic criteria and is easily confused with other mediastinal tumors. […] To date, imaging characteristics of UPS at different sites have been documented to enhance diagnostic efficiency, but less so for mediastinal cases; we report a case of anterior mediastinal UPS with complete imaging data. […] The mass exhibited involvement of the pericardium with uncertain demarcations, leading to the displacement of major cardiac vessels and the presence of left pleural effusion.

• #18 Undifferentiated pleomorphic sarcoma | Radiology Case | Radiopaedia.org

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

  Swelling right upper medial thigh for 3 months. Gradually increasing in size, and associated with intermittent pain. No history of trauma, fever, or weight loss. […] A well-defined oval shape mildly heterogeneous hypoechoic mass lesion is seen between the muscle layers of the right upper medial thigh. Mild internal vascularity is seen in it on color Doppler ultrasound examination. […] Differential diagnosis includes peripheral nerve sheath tumor (like neurofibroma), or soft tissue tumor (myxoma, or soft tissue sarcoma). […] A partially circumscribed soft tissue swelling/bulge is seen along the medial aspect of the right upper thigh. No soft tissue calcifications are seen. Morphology of the visualized bones is intact. […] Findings: There is a large well-defined, oval-shape soft tissue lesion in the right upper medial thigh within the adductor longus muscle. The lesion is isointense to the muscles on T1 and hyperintense on T2 weighted images. The lesion shows marked enhancement after the contrast administration. No significant cystic/necrotic changes, fat component, or calcifications are seen in it. No extra-muscular extension is seen. There is no invasion of myofascial planes, subcutaneous soft tissues, neurovascular bundle, or underlying femur. No skip lesion or significant lymphadenopathy is seen. Differential diagnosis includes neurofibroma, myxoma, or soft tissue sarcoma.

• #19 Diagnosis of soft tissue sarcoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/diagnosis

  A complete blood count (CBC) measures the number and quality of white blood cells, red blood cells and platelets. A CBC is done to check your general health, often before a biopsy. […] Blood clotting (coagulation) tests check if your blood is clotting properly. Prothrombin time (PT), partial thromboplastin time (PTT) and international normalized ratio (INR) are common blood clotting tests. They are usually checked before a biopsy is done. […] An MRI is commonly used to examine a lump that doctors think may be a soft tissue sarcoma. It is often the first imaging test done for lumps in the arms or legs. An MRI can check the size of a tumour, what tissues and areas are nearby and if it has spread to other parts of the body. […] A CT scan is commonly used to examine a lump in a specific area, including checking its size and finding out what tissues and areas are nearby. It is often used to check the abdomen, back of the abdomen (retroperitoneum) and chest. If soft tissue sarcoma is diagnosed, a CT scan can be used to check if the cancer has spread to other parts of the body.

• #20 What Is Undifferentiated Pleomorphic Sarcoma?

  https://www.icliniq.com/articles/cancer/undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma (UPS) is a high-grade aggressive soft-tissue sarcoma formerly recognized as malignant fibrous histiocytoma (MFH). […] Diagnosing undifferentiated pleomorphic sarcoma includes histology and immunohistochemistry confirming the diagnosis, examination, and workup, including imaging of the primary tumor, lymph nodes, and distant metastases. […] The TNM Staging Criteria- Will differ based on where the tumor is located. […] Magnetic Resonance Imaging (MRI)- With contrast is recommended to detect tumor size, invasion of visceral tissues, and neurovascular involvement. […] Non-contrast Computed Tomography (CT)- When internal viscera or retroperitoneal involvement is suspected, non-contrast computed tomography (CT) of the chest, pelvis, and abdomen should be considered.

• #21 Clinical presentation, diagnostic evaluation, and staging of soft tissue sarcoma – UpToDate

  https://www.uptodate.com/contents/clinical-presentation-histopathology-diagnostic-evaluation-and-staging-of-soft-tissue-sarcoma

  Clinical presentation, diagnostic evaluation, and staging of soft tissue sarcoma […] This topic review will cover the clinical presentation, diagnostic evaluation, and staging of soft tissue sarcoma. […] Diagnostic Images […] Undifferentiated pleomorphic sarcoma on MRI […] Undifferentiated pleomorphic sarcoma on CT scan […] Undifferentiated pleomorphic sarcoma on PET scan.

• #22 What Is Undifferentiated Pleomorphic Sarcoma?

  https://www.icliniq.com/articles/cancer/undifferentiated-pleomorphic-sarcoma

  Sentinel Lymph Node Biopsy (SLNB)- It is recommended to diagnose undifferentiated pleomorphic sarcomas (UPS). […] Positron Emission Tomography (PET)- F-fluorodeoxyglucose positron emission tomography (PET) scan is a valuable prediction tool in patients with soft tissue sarcoma (STS) and bone sarcomas.

• #23 Clinical presentation, diagnostic evaluation, and staging of soft tissue sarcoma – UpToDate

  https://www.uptodate.com/contents/clinical-presentation-histopathology-diagnostic-evaluation-and-staging-of-soft-tissue-sarcoma

  Clinical presentation, diagnostic evaluation, and staging of soft tissue sarcoma […] This topic review will cover the clinical presentation, diagnostic evaluation, and staging of soft tissue sarcoma. […] Diagnostic Images […] Undifferentiated pleomorphic sarcoma on MRI […] Undifferentiated pleomorphic sarcoma on CT scan […] Undifferentiated pleomorphic sarcoma on PET scan.

• #24 Undifferentiated pleomorphic sarcoma | ABC Medical Center

  https://centromedicoabc.com/en/padecimientos/undifferentiated-pleomorphic-sarcoma/

  The specialist, in addition to analyzing your clinical history and symptoms, will perform a physical examination in which they will pay special attention to the affected area to try to detect the size and depth of the tumor, as well as identify if it is connected to nearby tissues and if there is inflammation or damage to the nerves. […] Also, the following tests will be requested to confirm the diagnosis and rule out any other anomaly: […] Tissue biopsy. […] X-rays. […] MRI. […] Computed tomography. […] Positron emission tomography.

• #25 Undifferentiated pleomorphic sarcoma in the anterior mediastinum: a case report and literature review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11518729/

  Primary undifferentiated high-grade pleomorphic sarcoma (UPS) in the mediastinum is exceptionally rare. […] Undifferentiated pleomorphic sarcoma (UPS) is a diagnosis of exclusion that requires the elimination of various other tumor types prior to confirmation. […] Radiology plays a crucial role in initial diagnosis, long-term follow-up, and the evaluation of many treatment-related complications. […] However, it lacks clear diagnostic criteria and is easily confused with other mediastinal tumors. […] To date, imaging characteristics of UPS at different sites have been documented to enhance diagnostic efficiency, but less so for mediastinal cases; we report a case of anterior mediastinal UPS with complete imaging data. […] The mass exhibited involvement of the pericardium with uncertain demarcations, leading to the displacement of major cardiac vessels and the presence of left pleural effusion.

• #26 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

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

  The undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a high-grade soft-tissue sarcoma (STS). […] At present, UPS is diagnosed by excluding other well-classified STSs. […] Histopathology examination is essential for the diagnosis of UPS. […] The definitive diagnosis of UPS is confirmed by excluding other malignancies with a panel of immunohistochemical markers. […] Once the diagnosis has been confirmed with histopathology and immunohistochemistry, evaluation and workup include imaging of the primary tumor, lymph nodes, and distant metastases. […] 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. […] The most common differential diagnoses of UPS are other types of STSs, which must be distinguished by clinical history, physical examination, and immunohistochemistry markers. […] Trunk and extremities UPS is staged according to the TNM and histologic grade (G). […] Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. […] Follow-up visits are important to detect local recurrences or metastasis.

• #27 Undifferentiated Pleomorphic Sarcoma | Sarcoma UK

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

  Undifferentiated pleomorphic sarcoma, also known as UPS, is one of the most common types of sarcoma. […] A specialist doctor will diagnose UPS through a series of tests. These may include: […] UPS is a diagnosis of exclusion. This means that the tumour shows no identifiable characteristics for the doctors to be able to diagnose it as a specific sarcoma type. A doctor will look to see if the biopsy looks like a specific sarcoma subtype and if it doesn’t, then it will be diagnosed as UPS.

• #28 Undifferentiated Pleomorph – Types of Sarcoma Cancer

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

  Imaging tests: In order to get a better look at the tumor, doctors may use CT scans, x-rays and MRIs. They make it easy to see the size of the tumor, its shape and whether or not its spread. […] Biopsy: Whether with a special needle or through surgery, tissue from a tumor can be removed and put under a microscope for closer examination. This is often enough for a doctor to decide if a patient has a cancerous tumor. […] Blood tests: One preliminary test doctors will often do is checking a patients blood count. Theyll examine white and red blood cell numbers as well as platelet counts. Doctors can also test for enzymes and/or hormones indicative of cancer.

• #29 Accuracy of core needle biopsy for histologic diagnosis of soft tissue sarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-022-05752-4

  The biopsy technique of choice in soft tissue sarcoma (STS) diagnosis is controversial. […] We examined the diagnostic accuracy of percutaneous core needle biopsy (CNB) and compared it to open incisional biopsy. […] Furthermore, we investigated the role of molecular pathology in sarcoma diagnostics. […] CNB leads to faster diagnosis while reaching the same histological accuracy and is less burdensome for patients. […] This study aims to report our institutional experience and investigate the diagnostic accuracy, sensitivity, specificity, safety, and time to diagnosis of CNB in comparison to open incisional biopsy. […] Analysis of all patients regarding the accuracy of biopsy techniques revealed a 96% accuracy for diagnosing malignancy via CNB and 100% for incisional biopsy. […] The correct entity was diagnosed in nearly all cases, and the correct grading was diagnosed in 90% of incisional biopsies and 94% of CNBs.

• #30 Accuracy of core needle biopsy for histologic diagnosis of soft tissue sarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-022-05752-4

  This retrospective analysis provides evidence that core needle biopsy is as accurate as incisional biopsy in diagnosing soft tissue sarcoma. […] CNB achieved histological accuracy for subtype diagnosis in 87% of cases compared to 89% from incisional biopsy. […] In addition, CNB led to faster diagnosis of malignancy while being less burdensome for patients and clinical resources. […] In light of our data and the pertinent literature, we conclude that CNB is reliable and safe as a method of the first choice for soft tissue sarcoma biopsy.

• #31 Diagnosis of soft tissue sarcoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/diagnosis

  During a biopsy, the doctor removes tissues or cells from the body so they can be tested in a lab. A report from the pathologist will show whether or not cancer cells are found in the sample. If cancer is diagnosed, the report will also show the type of cancer and grade of the tumour. […] A bone scan uses bone-seeking radioactive materials called radiopharmaceuticals and a computer to create a picture of the bones. It is used to check if the soft tissue sarcoma has spread to the bone.

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

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

  Diagnosis for undifferentiated pleomorphic sarcoma usually starts with a review of your symptoms and a physical examination. This cancer is often diagnosed after other types of cancer have been ruled out. […] Tests and procedures may include: […] To make a definitive diagnosis, your doctor collects a sample of the tumor tissue and sends it to a lab for testing. […] In the lab, doctors trained in analyzing body tissues (pathologists) examine the sample to determine the types of cells involved and whether the cells are likely to be aggressive. This information helps rule out other types of cancer and guides your treatment. […] Determining the type of biopsy needed and the specifics of how it should be performed requires careful planning by the medical team. […] Undifferentiated pleomorphic sarcoma is rare and often requires complex care. It’s best treated by someone who has significant experience with it, which often means an academic or multispecialized cancer center. […] What kinds of tests do I need to confirm the diagnosis? Do these tests require any special preparation? […] What stage is the sarcoma? […] What treatments are available for undifferentiated pleomorphic sarcoma, and which do you recommend?

• #33 Undifferentiated Pleomorphic Sarcoma (UPS): Causes, Symptoms

  https://www.prepladder.com/neet-pg-study-material/pathology/undifferentiated-pleomorphic-sarcoma-ups-causes-symptoms-risk-factors-diagnosis-and-treatment

  Biopsy: To make a definitive diagnosis, your doctor removes a sample of the tumour tissue and sends it to a lab for examination. Depending on your particular situation, a tissue sample may be obtained during an operation or using a needle inserted through your skin. […] Medical experts who analyse body tissues, known as pathologists, look at the sample in the lab to determine the various cell types that are present and gauge its potential for aggression. This information helps rule out other types of cancer and guides your therapy. […] The type of biopsy that is needed and the best way to perform it are decisions that the medical team must make with great consideration. Doctors have to do the biopsy in a way that won’t interfere with planned cancer excision procedures. […] This is why you should ask your doctor for a referral to a team of specialists who treat soft tissue sarcomas before the biopsy.

• #34 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  It can be exceedingly difficult to distinguish a UPS from a sarcomatoid carcinoma. […] Depending on definitional criteria, UPS still accounts for a significant proportion of sarcomas occurring in late adult life. […] UPS is characteristically a tumor of late adult life, with most cases occurring in persons between the ages of 50 and 70 years. […] The classic form of UPS has a highly variable morphologic pattern and shows frequent transitions from storiform to pleomorphic areas, although the emphasis in most tumors is on haphazardly arranged pleomorphic zones. […] The role of immunohistochemistry in the diagnosis of UPS has traditionally been an ancillary one, primarily serving as a means to exclude other pleomorphic tumors. […] More recently, a number of studies using comparative genomic hybridization have evaluated UPS and compared the findings to those of other pleomorphic sarcomas. […] The vast majority of UPS are high-grade lesions having a local recurrence rate ranging from 19-31%, a metastatic rate of 31-35%, and a 5-year survival of 65-70%. […] In summary, pleomorphic malignant neoplasms are commonly encountered by surgical pathologists.

• #35

  https://journals.lww.com/jpat/fulltext/2023/27001/undifferentiated_pleomorphic_sarcoma_of_the_floor.8.aspx

  On microscopy, UPS shows an intratumoral and intertumoral diversity. […] Once diagnosed, multimodality therapeutic approach combining surgery, external beam radiation therapy chemotherapy drugs like doxorubicin tailored according to the patient is the only option left. […] The prognosis of UPS is dismal. […] The vast majority of UPS are high-grade lesions having a local recurrence rate ranging from 19-31%, a metastatic rate of 31-35%, and a 5-year survival of 65-70%. […] In summary, UPS is rare in oral cavity and it can be easily misdiagnosed as poorly differentiated SCC or sarcomatoid carcinoma.

• #36 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  It can be exceedingly difficult to distinguish a UPS from a sarcomatoid carcinoma. […] Depending on definitional criteria, UPS still accounts for a significant proportion of sarcomas occurring in late adult life. […] UPS is characteristically a tumor of late adult life, with most cases occurring in persons between the ages of 50 and 70 years. […] The classic form of UPS has a highly variable morphologic pattern and shows frequent transitions from storiform to pleomorphic areas, although the emphasis in most tumors is on haphazardly arranged pleomorphic zones. […] The role of immunohistochemistry in the diagnosis of UPS has traditionally been an ancillary one, primarily serving as a means to exclude other pleomorphic tumors. […] More recently, a number of studies using comparative genomic hybridization have evaluated UPS and compared the findings to those of other pleomorphic sarcomas. […] The vast majority of UPS are high-grade lesions having a local recurrence rate ranging from 19-31%, a metastatic rate of 31-35%, and a 5-year survival of 65-70%. […] In summary, pleomorphic malignant neoplasms are commonly encountered by surgical pathologists.

• #37 Undifferentiated pleomorphic sarcoma of the spleen: a case report and literature review | Surgical Case Reports | Full Text

  https://surgicalcasereports.springeropen.com/articles/10.1186/s40792-023-01734-4

  Given the rarity of the diagnosis, the disease is rarely suspected on radiological or clinical grounds, with the most cases suspecting lymphoma at the first instance. As presented here, most cases only reached the final diagnosis after thorough histopathological work up. […] The immunohistochemical profile of UPS is non-specific and is principally geared at excluding other diagnoses. The tumour is persistently negative for cytokeratins and markers of melanocytic differentiation. Non-specific expression of lysozyme, CD68, fascin, CD10 and cyclin D1 may be observed. However, expression of histiocytic markers is only focal which helps to differentiate this tumour from histiocytic sarcoma. […] Due to the paucity of data and rare nature of the disease, there are no established staging criteria for splenic UPS. This has implications on stratifying patients and guiding management.

• #38 Undifferentiated pleomorphic sarcoma in the maxillary sinus: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-024-05019-8

  Owing to the different degrees of cellular pleomorphism, it is sometimes relatively difficult to diagnose UPS only through morphology as it may be confused with other lesions; therefore, immunohistochemical staining is necessary for the final diagnosis of the lesion. […] In the present case, considering the possibility of pleomorphic sarcomata, immunohistochemical markers, cytokeratin, vimentin, CD68 antigen, S100 protein, and desmin were requested. […] Finally, these findings were consistent with the diagnosis of an undifferentiated pleomorphic sarcoma (or malignant fibrous histiocytoma), of storiform/pleomorphic giant-cell type, by excluding other possible diagnoses in an exclusive manner. […] In the present report, osteoid deposits were observed only in limited areas on the margin of the lesion immediately under the epithelium, but were not present in other parts of the lesion in incisional and excisional biopsy; therefore, the possibility of an extraskeletal osteosarcoma was rejected and the diagnosis of a UPS with reactive steroid production was confirmed.

• #39 Undiff. Pleomorphic Sarcoma : Differential Diagnosis

  https://www.webpathology.com/images/soft-tissue/fibrohistiocytic/undifferentiated-pleomorphic-sarcoma/43982

  Differential Diagnosis of Undifferentiated Pleomorphic Sarcoma (UPS): Pleomorphic Rhabdomyosarcoma: Microscopically, pleomorphic rhabdomyosarcoma (RMS) is characterized by haphazardly arranged large highly pleomorphic cells with one or more irregular hyperchromatic nuclei and abnormal mitoses. […] The cells have deeply eosinophilic cytoplasm and show some cell-to-cell molding. […] There may be large irregular or tadpole-shaped rhabdomyoblasts which resemble the pleomorphic cells of UPS. […] Cells with cross-striations are less frequent than in embryonal RMS with pleomorphic or anaplastic features. […] Uncommon features include rhabdoid cells, phagocytosis by tumor cells, intracytoplasmic glycogen, and a lymphohistiocytic infiltrate. […] Myogenin and MyoD1 – which are nuclear regulatory proteins involved in skeletal muscle differentiation – are positive (nuclear staining).

• #40 Histologic and Genetic Advances in Refining the Diagnosis of “Undifferentiated Pleomorphic Sarcoma”

  https://www.mdpi.com/2072-6694/5/1/218

  Undifferentiated pleomorphic sarcoma (UPS) is an inclusive term used for sarcomas that defy formal sub-classification. The frequency with which this diagnosis is assigned has decreased in the last twenty years. This is because when implemented, careful histologic assessment, immunohistochemistry, and ultra-structural evaluation can often determine lineage of differentiation. […] Improvements in histologic practices and institution of gene expression clustering methods can frequently establish the true diagnostic assignment. […] The careful approach to cases of MFH applied by Fletcher (tumour morphology, immune-histochemistry and ultra-structural analysis) has led to attrition in the percentage of cases diagnosed as MFH, a diagnosis of exclusion. […] Gene expression profiling to eliminate contamination with other sarcoma subtypes due to histologic pleomorphism is required.

• #41 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  The only criterion for recognizing extraskeletal osteosarcoma is the production of osteoid or bone by cytologically malignant cells. […] As mentioned, several studies have suggested that pleomorphic sarcomas with myogenic differentiated are clinically more aggressive than those without myogenic differentiation. […] However, there are a number of practical issues that make the assessment of myogenic differentiation in a pleomorphic sarcoma rather difficult. […] The process of tumor progression or dedifferentiation involves the transformation of a low-grade sarcoma to a higher-grade sarcoma, which usually (but not always) resembles a UPS. […] The utility of MDM2 and CDK4 analysis in distinguishing dedifferentiated liposarcoma from other types of pleomorphic sarcoma is controversial, but the preponderance of evidence suggests that this analysis is useful.

• #42 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  The only criterion for recognizing extraskeletal osteosarcoma is the production of osteoid or bone by cytologically malignant cells. […] As mentioned, several studies have suggested that pleomorphic sarcomas with myogenic differentiated are clinically more aggressive than those without myogenic differentiation. […] However, there are a number of practical issues that make the assessment of myogenic differentiation in a pleomorphic sarcoma rather difficult. […] The process of tumor progression or dedifferentiation involves the transformation of a low-grade sarcoma to a higher-grade sarcoma, which usually (but not always) resembles a UPS. […] The utility of MDM2 and CDK4 analysis in distinguishing dedifferentiated liposarcoma from other types of pleomorphic sarcoma is controversial, but the preponderance of evidence suggests that this analysis is useful.

• #43 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

  Current knowledge on UPSs suggests an aggressive clinical course, high incidence of recurrence and metastasis compared with other histologic STS subtypes. […] In the present case, we identified a LMNA-NTRK1 fusion gene comprising exons 1117 of the NKRT1 gene and exons 12 of LMNA gene in the patients tumor. […] The tumor regression and clinical response observed in the present case establishes that this LMNA-NTRK1 fusion may be a molecular driver of carcinogenesis in this patient and provides clinical validation of a molecular target in oncology. […] 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.

• #44 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

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

  The undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a high-grade soft-tissue sarcoma (STS). […] At present, UPS is diagnosed by excluding other well-classified STSs. […] Histopathology examination is essential for the diagnosis of UPS. […] The definitive diagnosis of UPS is confirmed by excluding other malignancies with a panel of immunohistochemical markers. […] Once the diagnosis has been confirmed with histopathology and immunohistochemistry, evaluation and workup include imaging of the primary tumor, lymph nodes, and distant metastases. […] 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. […] The most common differential diagnoses of UPS are other types of STSs, which must be distinguished by clinical history, physical examination, and immunohistochemistry markers. […] Trunk and extremities UPS is staged according to the TNM and histologic grade (G). […] Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. […] Follow-up visits are important to detect local recurrences or metastasis.

• #45 What Is Undifferentiated Pleomorphic Sarcoma?

  https://www.icliniq.com/articles/cancer/undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma (UPS) is a high-grade aggressive soft-tissue sarcoma formerly recognized as malignant fibrous histiocytoma (MFH). […] Diagnosing undifferentiated pleomorphic sarcoma includes histology and immunohistochemistry confirming the diagnosis, examination, and workup, including imaging of the primary tumor, lymph nodes, and distant metastases. […] The TNM Staging Criteria- Will differ based on where the tumor is located. […] Magnetic Resonance Imaging (MRI)- With contrast is recommended to detect tumor size, invasion of visceral tissues, and neurovascular involvement. […] Non-contrast Computed Tomography (CT)- When internal viscera or retroperitoneal involvement is suspected, non-contrast computed tomography (CT) of the chest, pelvis, and abdomen should be considered.

• #46 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. […] The presentation is usually with a painless, enlarging palpable mass. […] 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.

• #47 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

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

  The undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a high-grade soft-tissue sarcoma (STS). […] At present, UPS is diagnosed by excluding other well-classified STSs. […] Histopathology examination is essential for the diagnosis of UPS. […] The definitive diagnosis of UPS is confirmed by excluding other malignancies with a panel of immunohistochemical markers. […] Once the diagnosis has been confirmed with histopathology and immunohistochemistry, evaluation and workup include imaging of the primary tumor, lymph nodes, and distant metastases. […] 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. […] The most common differential diagnoses of UPS are other types of STSs, which must be distinguished by clinical history, physical examination, and immunohistochemistry markers. […] Trunk and extremities UPS is staged according to the TNM and histologic grade (G). […] Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. […] Follow-up visits are important to detect local recurrences or metastasis.

• #48 What Is Undifferentiated Pleomorphic Sarcoma?

  https://www.icliniq.com/articles/cancer/undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma (UPS) is a high-grade aggressive soft-tissue sarcoma formerly recognized as malignant fibrous histiocytoma (MFH). […] Diagnosing undifferentiated pleomorphic sarcoma includes histology and immunohistochemistry confirming the diagnosis, examination, and workup, including imaging of the primary tumor, lymph nodes, and distant metastases. […] The TNM Staging Criteria- Will differ based on where the tumor is located. […] Magnetic Resonance Imaging (MRI)- With contrast is recommended to detect tumor size, invasion of visceral tissues, and neurovascular involvement. […] Non-contrast Computed Tomography (CT)- When internal viscera or retroperitoneal involvement is suspected, non-contrast computed tomography (CT) of the chest, pelvis, and abdomen should be considered.

• #49 What Is Undifferentiated Pleomorphic Sarcoma?

  https://www.icliniq.com/articles/cancer/undifferentiated-pleomorphic-sarcoma

  Undifferentiated pleomorphic sarcoma (UPS) is a high-grade aggressive soft-tissue sarcoma formerly recognized as malignant fibrous histiocytoma (MFH). […] Diagnosing undifferentiated pleomorphic sarcoma includes histology and immunohistochemistry confirming the diagnosis, examination, and workup, including imaging of the primary tumor, lymph nodes, and distant metastases. […] The TNM Staging Criteria- Will differ based on where the tumor is located. […] Magnetic Resonance Imaging (MRI)- With contrast is recommended to detect tumor size, invasion of visceral tissues, and neurovascular involvement. […] Non-contrast Computed Tomography (CT)- When internal viscera or retroperitoneal involvement is suspected, non-contrast computed tomography (CT) of the chest, pelvis, and abdomen should be considered.

• #50 What Is Undifferentiated Pleomorphic Sarcoma?

  https://www.icliniq.com/articles/cancer/undifferentiated-pleomorphic-sarcoma

  Sentinel Lymph Node Biopsy (SLNB)- It is recommended to diagnose undifferentiated pleomorphic sarcomas (UPS). […] Positron Emission Tomography (PET)- F-fluorodeoxyglucose positron emission tomography (PET) scan is a valuable prediction tool in patients with soft tissue sarcoma (STS) and bone sarcomas.

• #51 What Is Undifferentiated Pleomorphic Sarcoma?

  https://www.icliniq.com/articles/cancer/undifferentiated-pleomorphic-sarcoma

  Sentinel Lymph Node Biopsy (SLNB)- It is recommended to diagnose undifferentiated pleomorphic sarcomas (UPS). […] Positron Emission Tomography (PET)- F-fluorodeoxyglucose positron emission tomography (PET) scan is a valuable prediction tool in patients with soft tissue sarcoma (STS) and bone sarcomas.

• #52 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

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

  The undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a high-grade soft-tissue sarcoma (STS). […] At present, UPS is diagnosed by excluding other well-classified STSs. […] Histopathology examination is essential for the diagnosis of UPS. […] The definitive diagnosis of UPS is confirmed by excluding other malignancies with a panel of immunohistochemical markers. […] Once the diagnosis has been confirmed with histopathology and immunohistochemistry, evaluation and workup include imaging of the primary tumor, lymph nodes, and distant metastases. […] 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. […] The most common differential diagnoses of UPS are other types of STSs, which must be distinguished by clinical history, physical examination, and immunohistochemistry markers. […] Trunk and extremities UPS is staged according to the TNM and histologic grade (G). […] Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. […] Follow-up visits are important to detect local recurrences or metastasis.

• #53 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  The term malignant fibrous histiocytoma (MFH) has been supplanted by undifferentiated pleomorphic sarcoma (UPS). […] This review will discuss historical aspects of MFH/UPS as well as provide an approach to the pleomorphic malignant neoplasm with a discussion of useful ancillary techniques in the evaluation of such cases. […] The extent to which such lesions can be subclassified as sarcomas of alternative type is, in large part, dependent on definitional criteria and the number of ancillary studies a pathologist is willing to bring to bear on the evaluation of a pleomorphic sarcoma. […] At this point, we use the term UPS in our diagnostic reports, but also state that this is synonymous with so-called MFH in parentheses so as to avert any misunderstanding with clinicians who continue to be familiar with that term.

• #54 Undiff. Pleomorphic Sarcoma : Differential Diagnosis

  https://www.webpathology.com/images/soft-tissue/fibrohistiocytic/undifferentiated-pleomorphic-sarcoma/43982

  Differential Diagnosis of Undifferentiated Pleomorphic Sarcoma (UPS): Pleomorphic Rhabdomyosarcoma: Microscopically, pleomorphic rhabdomyosarcoma (RMS) is characterized by haphazardly arranged large highly pleomorphic cells with one or more irregular hyperchromatic nuclei and abnormal mitoses. […] The cells have deeply eosinophilic cytoplasm and show some cell-to-cell molding. […] There may be large irregular or tadpole-shaped rhabdomyoblasts which resemble the pleomorphic cells of UPS. […] Cells with cross-striations are less frequent than in embryonal RMS with pleomorphic or anaplastic features. […] Uncommon features include rhabdoid cells, phagocytosis by tumor cells, intracytoplasmic glycogen, and a lymphohistiocytic infiltrate. […] Myogenin and MyoD1 – which are nuclear regulatory proteins involved in skeletal muscle differentiation – are positive (nuclear staining).

• #55 An approach to pleomorphic sarcomas: can we subclassify, and does it matter? | Modern Pathology

  https://www.nature.com/articles/modpathol2013174

  Whenever I encounter a soft tissue neoplasm with a MFH-like pattern, I consider several broad possibilities before concluding that the lesion is in fact an UPS. […] The rationale behind attempting to more precisely classify a pleomorphic sarcoma is discussed below. […] Although a specific type of pleomorphic sarcoma may be suggested by histologic features, immunohistochemical stains are often required to confirm the diagnosis. […] The only criterion for rendering a diagnosis of pleomorphic liposarcoma is the recognition of multivacuolated pleomorphic lipoblasts. […] A definitive diagnosis of pleomorphic malignant peripheral nerve sheath tumor can be difficult unless the pleomorphic sarcoma clearly arises from a benign nerve sheath tumor or arises from a peripheral nerve in a patient with type 1 neurofibromatosis.

• #56 Soft Tissue Sarcoma Update, Issue 1, 2017 (Video Program) | Research To Practice

  https://www.researchtopractice.com/index.php?q=STSU117/Video/2

  DR POLLACK: Yes. So theres so many different sarcomas that sometimes it can help to classify them in your mind. There are the very mutated genetically complex sarcomas, and those include undifferentiated pleomorphic sarcomas. […] DR POLLACK: Its actually quite common. Its very important to have an experienced bone and soft tissue pathologist review the case, because almost a third of the time, pathologists that are not used to dealing with sarcoma will misclassify the sarcoma subtype, whereas theres a very high concordance rate in experienced bone and soft tissue pathologists in classifying these sarcoma cases. […] DR POLLACK: No. There are definitely markers that will classify sarcomas into different subtypes. For example, in part it has to do with morphology. But, for example, there are certain immunohistochemical stains that will classify different sarcomas as different types. […] So there are, in some cases, very specific tests that you can use to confirm the diagnosis.

• #57

  http://waocp.com/journal/index.php/apjcc/article/view/274

  Reaching the correct histo-pathological diagnosis of soft tissue sarcomas (STS) is a great challenge and is cornerstone for treatment planning. […] Histopathologic diagnosis in more than half of patients referred from outside centers was discordant with respect to the diagnosis of our centre with major implications on 37% of cases. […] The main purpose of this study is to discuss the importance of highly specialized skills in pathology to ensure accurate diagnosis of sarcoma in India. Our data shows alarming discrepancy of 60.8% in the histopathological diagnosis outside tertiary care center. […] The frequency of major discrepancy was significantly higher in non-extremity sarcoma and this could be because pathologist and clinicians dont suspect sarcoma in visceral sites. […] We believe in time to come this discrepancy can be lessened by reviewing histopathology in tertiary care center as a part of treatment paradigm in soft tissue sarcoma.

• #58

  https://winshipcancer.emory.edu/cancer-types-and-treatments/sarcoma/diagnosis.php

  At Winship Cancer Institute of Emory University, our sarcoma specialists bring extraordinary expertise in sarcoma diagnosis so that an individualized and personalized treatment plan can be developed. […] An accurate sarcoma diagnosis is critical to achieving the best outcomes. And because there are more than 150 different types of sarcoma each one biologically unique the correct diagnosis can be challenging for pathologists who lack expertise in the field of sarcoma. […] As an NCI-designated Comprehensive Cancer Center, Winship Cancer Institute of Emory University not only sees a high volume of sarcoma patients every year, but also uses the latest diagnostic methods to help get them on their way to the appropriate treatment plan. […] Being able to appropriately identify and name the specific sarcoma that a patient has is the crucial first step in the management of sarcoma and allows Winships team to come up with an individualized treatment plan.

• #59

  https://winshipcancer.emory.edu/cancer-types-and-treatments/sarcoma/diagnosis.php

  The first step in making a sarcoma diagnosis is a thorough physical examination, followed by imaging. […] Your Winship care team may use one or more tests and procedures to determine the stage of your cancer: […] A critical component in accurately diagnosing a sarcoma is having an expert sarcoma pathologist review the biopsy tissue. […] At Winship, our purpose is to give you an accurate sarcoma diagnosis so you can get the most effective treatment.

• #60 Undifferentiated pleomorphic sarcoma in the anterior mediastinum: a case report and literature review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11518729/

  Primary undifferentiated high-grade pleomorphic sarcoma (UPS) in the mediastinum is exceptionally rare. […] Undifferentiated pleomorphic sarcoma (UPS) is a diagnosis of exclusion that requires the elimination of various other tumor types prior to confirmation. […] Radiology plays a crucial role in initial diagnosis, long-term follow-up, and the evaluation of many treatment-related complications. […] However, it lacks clear diagnostic criteria and is easily confused with other mediastinal tumors. […] To date, imaging characteristics of UPS at different sites have been documented to enhance diagnostic efficiency, but less so for mediastinal cases; we report a case of anterior mediastinal UPS with complete imaging data. […] The mass exhibited involvement of the pericardium with uncertain demarcations, leading to the displacement of major cardiac vessels and the presence of left pleural effusion.

• #61

  https://journals.lww.com/jpat/fulltext/2023/27001/undifferentiated_pleomorphic_sarcoma_of_the_floor.8.aspx

  Undifferentiated high-grade pleomorphic sarcoma is currently a diagnosis of exclusion and represents 5% of soft tissue sarcomas in adults. […] It is a soft tissue sarcoma composed of undifferentiated mesenchymal cells. […] The tumor occurs most frequently in the lower extremity especially thighs. […] Its location in the head and neck is rare. […] The most frequent location in head and neck is the neck and parotid gland, followed by the scalp, face, anterior skull base, and orbit. Their presence in oral cavity is extremely rare. […] Clinically, the most common symptom is the presence of a mass of progressive and rapid growth that may be associated with nonspecific symptoms such as pain and paresthesia of the compromised site. […] Imaging studies like the computed tomography and magnetic resonance imaging usually reveal a heterogeneously enhancing, lobulated soft tissue mass with adjacent soft tissue, and bone extension.

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

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

  Undifferentiated pleomorphic sarcoma (UPS) accounts for approximately 15% of all soft-tissue sarcoma (STS) cases and have a 5-year survival prognosis of around 60%. […] The primary objectives of this systematic review are: (I) identifying patient characteristics with lesion; (II) compiling patient outcomes following surgery; (III) identifying best therapy modalities; (IV) characterizing reported lesion histology; (V) assessing current surgical recommendations for resection; (VI) classifying lesions and their association with radiation. […] Of 433 studies, 24 case reports and 22 records were selected to inform on UPS in the chest wall. […] Treatment of UPS involving the chest is extremely complex. […] This study also notes no difference in recurrence or metastasis between patient who were treated and those who were not treated with other therapies.

• #63 Undifferentiated pleomorphic sarcoma in the maxillary sinus: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-024-05019-8

  Undifferentiated pleomorphic sarcoma, previously called malignant fibrous histiocytoma, is a type of malignant mesenchymal tumor (sarcoma) of soft tissue and sometimes bone. Microscopic diagnosis of this malignancy in the maxillary sinus can be very challenging, because there is a range of features that may overlap with other benign and malignant tumors. […] In this paper, a case of undifferentiated pleomorphic sarcoma in the maxillary sinus is reported, with an emphasis on the management of its problems and diagnostic errors. […] This study reviews the challenges and histopathological diagnostic errors of this uncommon tumor in this rare location. This lesion may be similar to other malignant tumors, and its correct diagnosis requires a detailed and complete examination. […] Given the fullness of the maxillary sinus at the time of the patients visit and on the basis of the evidence of imaging and the short interval that had lapsed since the formation of the intraoral mass as the patient stated, there was a strong possibility that the lesion originated from the maxillary sinus, which was also confirmed by the surgical findings.

• #64 Undifferentiated pleomorphic sarcoma in the anterior mediastinum: a case report and literature review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11518729/

  Primary undifferentiated high-grade pleomorphic sarcoma (UPS) in the mediastinum is exceptionally rare. […] Undifferentiated pleomorphic sarcoma (UPS) is a diagnosis of exclusion that requires the elimination of various other tumor types prior to confirmation. […] Radiology plays a crucial role in initial diagnosis, long-term follow-up, and the evaluation of many treatment-related complications. […] However, it lacks clear diagnostic criteria and is easily confused with other mediastinal tumors. […] To date, imaging characteristics of UPS at different sites have been documented to enhance diagnostic efficiency, but less so for mediastinal cases; we report a case of anterior mediastinal UPS with complete imaging data. […] The mass exhibited involvement of the pericardium with uncertain demarcations, leading to the displacement of major cardiac vessels and the presence of left pleural effusion.

• #65 Undifferentiated pleomorphic sarcoma in the maxillary sinus: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-024-05019-8

  Undifferentiated pleomorphic sarcoma, previously called malignant fibrous histiocytoma, is a type of malignant mesenchymal tumor (sarcoma) of soft tissue and sometimes bone. Microscopic diagnosis of this malignancy in the maxillary sinus can be very challenging, because there is a range of features that may overlap with other benign and malignant tumors. […] In this paper, a case of undifferentiated pleomorphic sarcoma in the maxillary sinus is reported, with an emphasis on the management of its problems and diagnostic errors. […] This study reviews the challenges and histopathological diagnostic errors of this uncommon tumor in this rare location. This lesion may be similar to other malignant tumors, and its correct diagnosis requires a detailed and complete examination. […] Given the fullness of the maxillary sinus at the time of the patients visit and on the basis of the evidence of imaging and the short interval that had lapsed since the formation of the intraoral mass as the patient stated, there was a strong possibility that the lesion originated from the maxillary sinus, which was also confirmed by the surgical findings.

• #66 Pleomorphic Sarcoma of the Liver; To Cut or Not to Cut? | ACS

  https://www.facs.org/for-medical-professionals/news-publications/journals/case-reviews/issues/v1n3/dishong-pleomorphic/

  Undifferentiated pleomorphic sarcoma (UPS) of the liver, previously referred to as primary malignant fibrous histocytoma (MFH), is a rare entity. […] Proper diagnosis is crucial in determining the correct treatment method. Thus, when presented with a pleomorphic sarcoma of the liver, immunohistochemical staining should be performed to confirm the diagnosis. […] Prognosis of a patient with UPS varies with the size as well as the grade of the tumor. […] Low grade, small, UPS of the liver should absolutely be resected with curative intent. […] Prognosis of UPS is often poor regardless of intervention. Prognosis is often based upon size and staging of the primary UPS. […] In conclusion, for lesions larger than 10 cm, we would encourage a brief trial of nutritional adjuncts limited to no more than two weeks and if there are no objective signs of nutritional improvement, the decision to operate or not to operate should be finalized. […] Palliative surgery and care are important considerations for UPS.

• #67 Undifferentiated pleomorphic sarcoma of the spleen: a case report and literature review | Surgical Case Reports | Full Text

  https://surgicalcasereports.springeropen.com/articles/10.1186/s40792-023-01734-4

  Undifferentiated pleomorphic sarcoma is an uncommon sarcoma and its presence in the spleen is even rarer, with only a handful of cases reported in English literature. It is typically only diagnosed following histological analysis. Its rarity also means that there is little consensus over ideal management. […] Splenic undifferentiated pleomorphic sarcoma is a rare tumour which may pose a significant diagnostic challenge on both clinical and histopathological grounds. Following diagnosis and treatment, its aggressive nature often results in a poor prognosis. Current literature fails to delineate any superior management strategy to increase survival. […] It remains a diagnostic challenge, being characterized by the absence of genetic characteristics and specific lines of differentiation on histological examination.

• #68

  https://journals.lww.com/joms/fulltext/2024/44020/metastatic_undifferentiated_pleomorphic_sarcoma_of.6.aspx

  Pleomorphic undifferentiated sarcoma (PUS) is an extremely aggressive soft-tissue sarcoma that occurs most commonly in the trunk and the extremities but is extremely rare in the kidney. This entity has no distinctive clinical or radiological signs for diagnosis. It has high local recurrence and distant metastasis rates along with poor prognosis. […] Since there is no specific clinical representation or any pathognomonic radiological features, preoperative diagnosis of PUS becomes rarely possible. Thereafter, the definitive and confirmatory diagnosis completely rests on postoperative histopathology. […] Moreover, since there are no specific clinical features or pathognomonic radiological signs to arrive at a provisional diagnosis of PUS, confirmatory diagnosis rests completely on histopathological examination.

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

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

  There are more reported cases of undifferentiated pleomorphic sarcoma (UPS) in the chest for women than men. […] Recurrence/metastasis for these patients was not different for patients treated with radiotherapy and/or chemotherapy. […] Prognosis is worse in patients with deeper and larger tumors of the chest wall. […] Future case reports need to provide information on margin distance to limit complications without compromising patient outcomes. […] Current literature suggests that radiation-associated UPS occurs in 5.2% of UPS cases, but these case reports demonstrate an occurrence of 5/22 for secondary malignancies (22.7%). […] This study illustrates that are more case reports of women with UPS of the chest wall despite men tending to develop UPS more often in general. […] Additionally, RT and chemotherapy are often recommended in the treatment UPS, but this study found no difference in the recurrence or metastasis of UPS in the chest wall between patients receiving therapy and those who did not.

• #70 Undifferentiated pleomorphic sarcoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/undifferentiated-pleomorphic-sarcoma/symptoms-causes/syc-20389554

  Undifferentiated pleomorphic sarcoma (UPS) is a rare type of cancer that begins mostly in the soft tissues of the body. […] Treatment for UPS depends on the location of the cancer, but often involves surgery, radiation and drug treatments. […] Make an appointment with a doctor if you develop any persistent signs or symptoms that worry you. […] It’s not clear what causes undifferentiated pleomorphic sarcoma. […] Doctors know this cancer begins when a cell develops changes in its DNA. […] Factors that may increase the risk of undifferentiated pleomorphic sarcoma include older age and previous radiation therapy. […] Most people who develop undifferentiated pleomorphic sarcoma have no known risk factors, and many people who have risk factors never develop the cancer.

• #71 Pleomorphic Sarcoma of the Liver; To Cut or Not to Cut? | ACS

  https://www.facs.org/for-medical-professionals/news-publications/journals/case-reviews/issues/v1n3/dishong-pleomorphic/

  Undifferentiated pleomorphic sarcoma (UPS) of the liver, previously referred to as primary malignant fibrous histocytoma (MFH), is a rare entity. […] Proper diagnosis is crucial in determining the correct treatment method. Thus, when presented with a pleomorphic sarcoma of the liver, immunohistochemical staining should be performed to confirm the diagnosis. […] Prognosis of a patient with UPS varies with the size as well as the grade of the tumor. […] Low grade, small, UPS of the liver should absolutely be resected with curative intent. […] Prognosis of UPS is often poor regardless of intervention. Prognosis is often based upon size and staging of the primary UPS. […] In conclusion, for lesions larger than 10 cm, we would encourage a brief trial of nutritional adjuncts limited to no more than two weeks and if there are no objective signs of nutritional improvement, the decision to operate or not to operate should be finalized. […] Palliative surgery and care are important considerations for UPS.

• #72 Undifferentiated pleomorphic sarcoma of the extremity and trunk: a retrospective cohort study of 166 cases in a large institution – Zhang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/62307/html

  An imperative need for better management strategies to improve the survival in patients with undifferentiated pleomorphic sarcoma (UPS). […] The existing study reveals that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are the prognostic markers of poor survival rate. […] R0 resection margin was an only independent favorable prognostic factor, which was correlated with LRFS, DMFS, and OS. […] The UPS in trunk, tumor size 5 cm and R1/R2 resection margin are prognostic markers of poor over survival. R1/R2 resection margin significantly correlated with high LR rate and women are more susceptible to LR. The UPS in trunk and R1/R2 resection margin are significantly correlated with DM and old patients (60 years) are more susceptible to DM.

• #73 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. […] Your healthcare provider will start by reviewing your health and biological family history. Theyll also ask questions about your symptoms, like when they started and whether theyve changed over time. Theyll also do: […] A physical exam. Theyll check the size of the lump and feel its location and how hard it is. […] Imaging tests. These typically include X-rays, an ultrasound and/or an MRI. […] Biopsy. Your provider will take a small sample of tissue and send it to a pathologist for testing. This involves looking at the sample under a microscope. […] Its 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.

• #74 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. […] The presentation is usually with a painless, enlarging palpable mass. […] 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.

• #75 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

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

  The undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a high-grade soft-tissue sarcoma (STS). […] At present, UPS is diagnosed by excluding other well-classified STSs. […] Histopathology examination is essential for the diagnosis of UPS. […] The definitive diagnosis of UPS is confirmed by excluding other malignancies with a panel of immunohistochemical markers. […] Once the diagnosis has been confirmed with histopathology and immunohistochemistry, evaluation and workup include imaging of the primary tumor, lymph nodes, and distant metastases. […] 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. […] The most common differential diagnoses of UPS are other types of STSs, which must be distinguished by clinical history, physical examination, and immunohistochemistry markers. […] Trunk and extremities UPS is staged according to the TNM and histologic grade (G). […] Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. […] Follow-up visits are important to detect local recurrences or metastasis.

• #76 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. […] The presentation is usually with a painless, enlarging palpable mass. […] 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.

• #77 Undifferentiated pleomorphic sarcoma – Wikipedia

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

  The most often used treatment for localized (i.e. no metastases) UPS tumors is complete surgical removal with the object of leaving no tumor cells behind as evidenced by microscopic examinations. Adjuvant therapy combining radiotherapy or/and chemotherapy with surgical resection is employed to reduce the risk of developing recurrent and metastatic disease in cases with high-risk disease (e.g. large tumors, tumors deemed highly aggressive based on their pathology and/or local invasiveness, inoperable tumors, and resections that did not remove all tumor cells).

• #78 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

  Current knowledge on UPSs suggests an aggressive clinical course, high incidence of recurrence and metastasis compared with other histologic STS subtypes. […] In the present case, we identified a LMNA-NTRK1 fusion gene comprising exons 1117 of the NKRT1 gene and exons 12 of LMNA gene in the patients tumor. […] The tumor regression and clinical response observed in the present case establishes that this LMNA-NTRK1 fusion may be a molecular driver of carcinogenesis in this patient and provides clinical validation of a molecular target in oncology. […] 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.

• #79 Undifferentiated pleomorphic sarcoma of the extremity and trunk: a retrospective cohort study of 166 cases in a large institution – Zhang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/62307/html

  An imperative need for better management strategies to improve the survival in patients with undifferentiated pleomorphic sarcoma (UPS). […] The existing study reveals that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are the prognostic markers of poor survival rate. […] R0 resection margin was an only independent favorable prognostic factor, which was correlated with LRFS, DMFS, and OS. […] The UPS in trunk, tumor size 5 cm and R1/R2 resection margin are prognostic markers of poor over survival. R1/R2 resection margin significantly correlated with high LR rate and women are more susceptible to LR. The UPS in trunk and R1/R2 resection margin are significantly correlated with DM and old patients (60 years) are more susceptible to DM.

• #80 Undifferentiated pleomorphic sarcoma in the maxillary sinus: a case report | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-024-05019-8

  Basically, there are three types of treatment for malignant fibrous histiocytoma: surgery, radiotherapy, and chemotherapy. […] The prognosis of this tumor is not good and its recurrence is common; there are usually metastases to the lungs, liver, and bones. […] Factors associated with a favorable prognosis in patients with malignant fibrous histiocytoma include: age less than 60 years, tumor size less than 5 cm, superficial lesion location, low-grade malignancy, myxoid histological subtype, and absence of metastatic disease. […] The overall survival rate after a 5-year follow-up of patients ranges from 30% to 74%. […] In terms of histopathology, this lesion may be similar to other malignant tumors, and its correct diagnosis requires a detailed and complete examination of the morphology and the use of immunohistochemical markers to exclude other pleomorphic sarcomata. Timely diagnosis and prompt referral have better results in the lifetime of patients.

• #81 Undifferentiated pleomorphic sarcoma of the extremity and trunk: a retrospective cohort study of 166 cases in a large institution – Zhang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/62307/html

  An imperative need for better management strategies to improve the survival in patients with undifferentiated pleomorphic sarcoma (UPS). […] The existing study reveals that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are the prognostic markers of poor survival rate. […] R0 resection margin was an only independent favorable prognostic factor, which was correlated with LRFS, DMFS, and OS. […] The UPS in trunk, tumor size 5 cm and R1/R2 resection margin are prognostic markers of poor over survival. R1/R2 resection margin significantly correlated with high LR rate and women are more susceptible to LR. The UPS in trunk and R1/R2 resection margin are significantly correlated with DM and old patients (60 years) are more susceptible to DM.

• #82 Undifferentiated pleomorphic sarcoma of the extremity and trunk: a retrospective cohort study of 166 cases in a large institution – Zhang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/62307/html

  An imperative need for better management strategies to improve the survival in patients with undifferentiated pleomorphic sarcoma (UPS). […] The existing study reveals that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are the prognostic markers of poor survival rate. […] R0 resection margin was an only independent favorable prognostic factor, which was correlated with LRFS, DMFS, and OS. […] The UPS in trunk, tumor size 5 cm and R1/R2 resection margin are prognostic markers of poor over survival. R1/R2 resection margin significantly correlated with high LR rate and women are more susceptible to LR. The UPS in trunk and R1/R2 resection margin are significantly correlated with DM and old patients (60 years) are more susceptible to DM.

• #83 Undifferentiated pleomorphic sarcoma of the extremity and trunk: a retrospective cohort study of 166 cases in a large institution – Zhang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/62307/html

  An imperative need for better management strategies to improve the survival in patients with undifferentiated pleomorphic sarcoma (UPS). […] The existing study reveals that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are the prognostic markers of poor survival rate. […] R0 resection margin was an only independent favorable prognostic factor, which was correlated with LRFS, DMFS, and OS. […] The UPS in trunk, tumor size 5 cm and R1/R2 resection margin are prognostic markers of poor over survival. R1/R2 resection margin significantly correlated with high LR rate and women are more susceptible to LR. The UPS in trunk and R1/R2 resection margin are significantly correlated with DM and old patients (60 years) are more susceptible to DM.

• #84 Undifferentiated pleomorphic sarcoma of the extremity and trunk: a retrospective cohort study of 166 cases in a large institution – Zhang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/62307/html

  An imperative need for better management strategies to improve the survival in patients with undifferentiated pleomorphic sarcoma (UPS). […] The existing study reveals that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are the prognostic markers of poor survival rate. […] R0 resection margin was an only independent favorable prognostic factor, which was correlated with LRFS, DMFS, and OS. […] The UPS in trunk, tumor size 5 cm and R1/R2 resection margin are prognostic markers of poor over survival. R1/R2 resection margin significantly correlated with high LR rate and women are more susceptible to LR. The UPS in trunk and R1/R2 resection margin are significantly correlated with DM and old patients (60 years) are more susceptible to DM.

• #85 Undifferentiated pleomorphic sarcoma of the extremity and trunk: a retrospective cohort study of 166 cases in a large institution – Zhang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/62307/html

  An imperative need for better management strategies to improve the survival in patients with undifferentiated pleomorphic sarcoma (UPS). […] The existing study reveals that the UPS in trunk, tumor size 5 cm and R1/R2 resection margin are the prognostic markers of poor survival rate. […] R0 resection margin was an only independent favorable prognostic factor, which was correlated with LRFS, DMFS, and OS. […] The UPS in trunk, tumor size 5 cm and R1/R2 resection margin are prognostic markers of poor over survival. R1/R2 resection margin significantly correlated with high LR rate and women are more susceptible to LR. The UPS in trunk and R1/R2 resection margin are significantly correlated with DM and old patients (60 years) are more susceptible to DM.

• #86 Undifferentiated Pleomorphic Sarcoma – StatPearls – NCBI Bookshelf

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

  The undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a high-grade soft-tissue sarcoma (STS). […] At present, UPS is diagnosed by excluding other well-classified STSs. […] Histopathology examination is essential for the diagnosis of UPS. […] The definitive diagnosis of UPS is confirmed by excluding other malignancies with a panel of immunohistochemical markers. […] Once the diagnosis has been confirmed with histopathology and immunohistochemistry, evaluation and workup include imaging of the primary tumor, lymph nodes, and distant metastases. […] 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. […] The most common differential diagnoses of UPS are other types of STSs, which must be distinguished by clinical history, physical examination, and immunohistochemistry markers. […] Trunk and extremities UPS is staged according to the TNM and histologic grade (G). […] Early disease recognition and an adequate treatment strategy are the most important interventions to improve overall prognosis. […] Follow-up visits are important to detect local recurrences or metastasis.

• #87

  https://journals.lww.com/jpat/fulltext/2023/27001/undifferentiated_pleomorphic_sarcoma_of_the_floor.8.aspx

  On microscopy, UPS shows an intratumoral and intertumoral diversity. […] Once diagnosed, multimodality therapeutic approach combining surgery, external beam radiation therapy chemotherapy drugs like doxorubicin tailored according to the patient is the only option left. […] The prognosis of UPS is dismal. […] The vast majority of UPS are high-grade lesions having a local recurrence rate ranging from 19-31%, a metastatic rate of 31-35%, and a 5-year survival of 65-70%. […] In summary, UPS is rare in oral cavity and it can be easily misdiagnosed as poorly differentiated SCC or sarcomatoid carcinoma.

• #88 Sarcoma relapse survivor: MD Anderson gave me a second chance to savor new motherhood | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/sarcoma-relapse-survivor–md-anderson-gave-me-a-second-chance-to-savor-new-motherhood.h00-159545268.html

  My husband, Wes, and I moved to The Woodlands a suburb of Houston about 30 miles north of downtown in September 2019. I am a survivor of a rare type of cancer called undifferentiated pleomorphic sarcoma, and I need to be monitored closely in case of a recurrence. […] I was supposed to get them every three months. […] Sarcoma medical oncologist Dr. Anthony Conley told me the cancer had returned, and in the same place as before. […] The thought of recurrence is what keeps us cancer survivors up at night. […] Dr. Katz performed the surgery on Aug. 16, 2021. […] I was also cancer-free again and wouldn’t need any additional treatment. […] I am thankful to Dr. Katz and MD Anderson for making me cancer-free again.

• #89 Sarcoma relapse survivor: MD Anderson gave me a second chance to savor new motherhood | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/sarcoma-relapse-survivor–md-anderson-gave-me-a-second-chance-to-savor-new-motherhood.h00-159545268.html

  My husband, Wes, and I moved to The Woodlands a suburb of Houston about 30 miles north of downtown in September 2019. I am a survivor of a rare type of cancer called undifferentiated pleomorphic sarcoma, and I need to be monitored closely in case of a recurrence. […] I was supposed to get them every three months. […] Sarcoma medical oncologist Dr. Anthony Conley told me the cancer had returned, and in the same place as before. […] The thought of recurrence is what keeps us cancer survivors up at night. […] Dr. Katz performed the surgery on Aug. 16, 2021. […] I was also cancer-free again and wouldn’t need any additional treatment. […] I am thankful to Dr. Katz and MD Anderson for making me cancer-free again.

• #90 Histologic and Genetic Advances in Refining the Diagnosis of “Undifferentiated Pleomorphic Sarcoma”

  https://www.mdpi.com/2072-6694/5/1/218

  Improved sub-categorisation of undifferentiated pleomorphic sarcoma offers further potential therapeutic opportunities. […] The percentage of cases of sarcoma diagnosed as undifferentiated pleomorphic sarcoma within an institutions pathology service over time is also an internal audit of the quality and extent of tissue sampling. […] New advances offer the promise of a more effective way to find the lineage of cellular differentiation of undifferentiated pleomorphic sarcomas.

• #91 Histologic and Genetic Advances in Refining the Diagnosis of “Undifferentiated Pleomorphic Sarcoma”

  https://www.mdpi.com/2072-6694/5/1/218

  Improved sub-categorisation of undifferentiated pleomorphic sarcoma offers further potential therapeutic opportunities. […] The percentage of cases of sarcoma diagnosed as undifferentiated pleomorphic sarcoma within an institutions pathology service over time is also an internal audit of the quality and extent of tissue sampling. […] New advances offer the promise of a more effective way to find the lineage of cellular differentiation of undifferentiated pleomorphic sarcomas.

• #92 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

  Current knowledge on UPSs suggests an aggressive clinical course, high incidence of recurrence and metastasis compared with other histologic STS subtypes. […] In the present case, we identified a LMNA-NTRK1 fusion gene comprising exons 1117 of the NKRT1 gene and exons 12 of LMNA gene in the patients tumor. […] The tumor regression and clinical response observed in the present case establishes that this LMNA-NTRK1 fusion may be a molecular driver of carcinogenesis in this patient and provides clinical validation of a molecular target in oncology. […] 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.

• #93 Undifferentiated pleomorphic sarcoma (UPS) Clinical Trial Finding | RareCan

  https://rarecan.com/about/rare-cancer-list/sarcoma/undifferentiated-pleomorphic-sarcoma-ups/

  For a much more detailed guide, please have a look at our undifferentiated pleomorphic sarcoma information pack. […] A diagnosis of UPS is sometimes called a “diagnosis of exclusion” where it is used to label any soft tissue tumour that doesn’t fit any other description. […] RareCan is working to accelerate research into UPS by making it easier for researchers to connect with people who have it. You can help us do this by becoming a RareCan member and sharing information with us about your UPS cancer diagnosis. We will then get in touch with you about any research opportunities that might be suitable for you.

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