Materiały źródłowe

• #1 Myxofibrosarcoma landscape: diagnostic pitfalls, clinical management and future perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9244941/

  Myxofibrosarcoma (MFS) is a common entity of adult soft tissue sarcomas (STS) characterized by a predilection of the extremities and a high local recurrence rate. […] The mainstay of treatment for localized disease is represented by surgical resection, with (neo)-adjuvant radio- and chemotherapy. […] In the metastatic setting, chemotherapy represents the backbone of treatments, however its role is still controversial and the outcome is very poor. […] The cornerstone of treatment for localized disease is surgical resection, possibly in combination with radiotherapy, while in metastatic MFS the standard systemic treatment is based on anthracycline chemotherapy, targeted therapy such as TKIs and CDKIs or immunotherapy. […] Since local recurrence frequently occurs but distant metastasis is rare, the current standard of care for localized disease is radical resection confirmed by histological clear surgical margins combined with neoadjuvant or adjuvant radiotherapy.

• #2 Biology and Management of High-Grade Myxofibrosarcoma: State of the Art and Future Perspectives

  https://www.mdpi.com/2075-4418/13/19/3022

  Myxofibrosarcoma (MFS) is one of the most common adult soft tissue sarcomas, typically arising in the extremities. […] The standard treatment for localized MFS is surgical resection. […] Wide resection is the standard treatment for local disease. […] Radiation therapy (RT) can be used as neoadjuvant/adjuvant treatment strategies to improve local tumor control. […] Like other STS subtypes, anthracycline, with or without ifosfamide, is the first-line treatment for advanced MFS. […] Gemcitabine can be used as a monotherapy or in combination with docetaxel or dacarbazine in pretreated STS patients. […] Trabectedin can be administered effectively and safely to patients with advanced STS at second- or later-line setting. […] Eribulin is currently licensed for use in patients with unresectable or metastatic liposarcoma who received a prior anthracycline-based regimen.

• #3 Prognostic Factors and Outcomes for Patients With Myxofibrosarcoma: A 13-Year Retrospective Evaluation | Anticancer Research

  https://ar.iiarjournals.org/content/39/6/2985

  Myxofibrosarcoma (MFS) represents approximately 20% of all soft-tissue sarcomas, especially in elderly patients in their sixth to eighth decades of life. The treatment for soft-tissue sarcomas varies from primary surgical resection to neoadjuvant or adjuvant radiotherapy or cytotoxic chemotherapy. […] The gold-standard treatment for MFS is surgical resection with histologically negative margins, which has superior results and good clinical outcomes compared to exclusive radiotherapy/chemotherapy. […] Negative margins (R0) were detected in 67.6% of patients after surgical resection, and local recurrence occurred in 23.5% of all patients after a mean disease-free period of 19.4 months. […] Radiotherapy was performed in a neoadjuvant or an adjuvant setting in 50% of patients (eight neoadjuvant, nine adjuvant).

• #4 Myxofibrosarcoma: Prognosis, Treatment & Staging

  https://my.clevelandclinic.org/health/diseases/22563-myxofibrosarcoma

  Myxofibrosarcoma treatment depends on several factors, including: […] Your healthcare provider surgically removes the tumor and a small amount of surrounding tissue. You may have radiation therapy before or after surgery to shrink the tumor or lower the risk of it coming back. Healthcare providers are less likely to use chemotherapy for myxofibrosarcoma than for other types of cancer. […] If you have a high-grade myxofibrosarcoma, you may have surgery along with other treatments such as radiation therapy. […] A tumor that extends to your deeper tissues, such as between your muscles, may require surgery and radiation therapy. […] No. Removing your arm or leg (amputation) used to be a common treatment for myxofibrosarcoma. Today, healthcare providers do everything they can to avoid amputation. […] After myxofibrosarcoma treatment, you’ll have regular follow-up imaging with your healthcare provider. If the tumor does return, regularly seeing your healthcare provider increases the chances of finding and treating myxofibrosarcoma early.

• #5 Treatment Options For Myxofibrosarcoma – Klarity Health Library

  https://my.klarity.health/treatment-options-for-myxofibrosarcoma/

  Myxofibrosarcoma is one type of soft tissue sarcoma or soft tissue cancer that tends to spread widely through the surrounding tissues, such as tissues supporting bones, muscles, and organs. […] Current treatments for myxofibrosarcoma are involved in incorporating various types of therapy including surgery, radiation, and chemotherapy. However, it tends to explore personalised models to help guide treatment decisions and predict individual outcomes based on unique characteristics. […] Recent studies are increasingly focusing on targeted therapy and immunotherapy as many clinical trials provided promising effective outcomes for myxofibrosarcoma. […] In patients whose tumours localise or do not spread to other places, the first treatment recommended is to perform surgery which can be combined with radiotherapy. The objective of surgery is to remove the tumour cell as much as possible, although there is still a chance of myxofibrosarcoma coming back.

• #6

  https://content.irisoncology.com/library/myxofibrosarcoma

  Myxofibrosarcoma treatment often involves surgery to remove the cancer. Other treatments include radiation therapy and chemotherapy. […] Myxofibrosarcoma can come back after surgery. This might be more likely if the cancer grows large or if lab tests show the cells are growing quickly. Your healthcare team may create a treatment plan with the goal of getting rid of all the cancer cells. To reach this goal, treatment might involve radiation therapy, chemotherapy or sometimes both treatments before or after surgery. These additional treatments may make it more likely that the cancer won’t come back. […] Which treatment is best for you will depend on your cancer, such as the size and location of the myxofibrosarcoma. […] The goal of surgery is to cut out as much of the cancer as possible. If all the myxofibrosarcoma is removed, surgery might be the only treatment needed.

• #7

  https://www.sydneysarcomaunit.com.au/myxofibrosarcoma

  Surgical Treatment for Myxofibrosarcoma The most common treatment for myxofibrosarcoma is surgical removal of the tumour. This can be a challenging process depending on where the tumour is located. The aim of surgery is to remove the tumour with a margin of normal surrounding tissue if possible. This improves the chance of cure and reduces the risk of the tumour re-occurring. Myxofibrosarcomas are known to be highly infiltrative and a wide margin of normal tissue needs to be removed to ensure all the tumour is removed. […] Sometimes, radiation therapy or chemotherapy is given before surgery to help shrink the tumour and improve the results (compared with surgery alone). […] Radiation Therapy The use of radiotherapy for these tumours is common. Radiotherapy (either before or after surgery) is useful in the management of myxofibrosarcomas. It has been shown to reduce the chance of the tumour re-occurring locally after surgical resection. […] Chemotherapy Chemotherapy is reserved for select cases, studies have shown benefit from chemotherapy following surgical resection for larger myxofibrosarcomas.

• #8 Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11254341/

  Undifferentiated pleomorphic sarcoma (UPS) and myxofibrosarcoma (MFS) are the most common histologic subtypes of soft tissue sarcoma (STS) found in the trunk and extremities, together representing approximately 25% of STS diagnosed in these locations. […] Surgery forms the backbone of therapeutic algorithms for localized disease, although adjuvant radiation plays a role in preventing local recurrence in many patients and neoadjuvant chemotherapy can be considered in a subset of high-risk cases. […] The infiltrative nature of MFS requires modification of surgery with 2 cm margins resected en bloc with the tumor where feasible. […] Adjuvant radiation mitigates risks of local recurrence in UPS/MFS, and there is growing evidence of survival benefit associated with the use of perioperative chemotherapy in patients with high-risk of metastatic disease including those with larger UPS/MFS. […] Treatments for advanced disease include anthracycline-based therapies in the first-line treatment and gemcitabine-docetaxel combination; recent research demonstrates the efficacy of immunotherapy in a proportion of patients.

• #9 Myxofibrosarcoma | Oncohema Key

  https://oncohemakey.com/myxofibrosarcoma/

  Myxofibrosarcoma is a unique subtype of soft tissue sarcoma with a locally infiltrative behavior. High-quality MRI imaging is critical for preoperative planning. Wide surgical resection with a 2 cm soft tissue margin is the mainstay of treatment and can require complex vascular and plastic surgery reconstruction. […] Local recurrence is common, and a subset of patients with higher-grade lesions will develop distant metastases. Radiation may be beneficial in reducing local recurrence. […] Patients presenting with a diagnosis of myxofibrosarcoma should undergo high-quality T1- and T2-weighted MRI with pre- and postgadolinium imaging to accurately define the extent of the disease. […] Wide surgical resection with a 2 cm soft tissue margin encompassing the entire area of increased signal on T2-weighted MRI is required, which can require complex vascular and plastic surgery reconstruction.

• #10 Myxofibrosarcoma – Wikipedia

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

  MFS tumors are often treated by surgical resection. However, these tumors have high recurrence rates at the sites of their resections. […] The recommended treatment for individuals presenting with localized MFS tumors is radical surgical resection. The resection should include a 2 cm margin of soft tissue surrounding the tumor, with planned resection of the entire area as defined by MRI-detected increased signals on T2-weighted images. This is done to ensure that all tumor tissue is removed in order to avoid the high risks of local recurrences and worsening prognoses. […] Consequently, adjuvant radiotherapy has been used to help reduce these recurrences and metastatic transformations. For patients with a limb tumor that cannot be resected (less than 5% of all cases), limb amputation is the treatment of choice.

• #11 Myxofibrosarcoma – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/myxofibrosarcoma/

  The primary treatment for myxofibrosarcoma (MFS) is surgical removal of the tumor. This involves excising the tumor along with a margin of healthy tissue to ensure complete removal of cancerous cells. […] Surgeons aim for a complete microscopic resection, often referred to as R0 resection, which is associated with decreased rates of local recurrence. […] Radiation therapy is frequently used in conjunction with surgery to manage MFS. It can be administered before surgery to shrink the tumor, making it easier to remove, or after surgery to eliminate any remaining cancer cells. […] While chemotherapy is not the primary treatment for MFS, it is considered in certain scenarios, such as when the cancer is aggressive or has metastasized. […] Newer treatment options, such as immunotherapy and targeted therapies, are being explored for MFS. […] Due to the high recurrence rate of MFS, regular follow-up care is essential. Patients undergo routine imaging to monitor for any signs of recurrence, which allows for early intervention if the cancer returns.

• #11 Myxofibrosarcoma – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/myxofibrosarcoma/

  Myxofibrosarcoma (MFS) is a rare and challenging type of soft tissue sarcoma known for its high local recurrence rates and infiltrative growth pattern. The management of MFS involves a multidisciplinary approach, primarily focusing on surgical removal of the tumor, often complemented by radiation therapy to reduce recurrence risks. […] While chemotherapy is not the primary treatment, it is considered in aggressive or metastatic cases. Emerging treatments like immunotherapy and targeted therapies are being explored, offering hope for improved outcomes. Regular follow-up care is crucial due to the high recurrence rate, and various factors such as tumor size, patient age, and surgical margins significantly influence patient outcomes. Clinical trials continue to explore new treatment combinations to enhance survival rates and quality of life for MFS patients.

• #12 Clear surgical margins as a prognostic indicator for disease recurrence, with no impact on survival rates in patients with myxofibrosarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-024-63035-6

  Myxofibrosarcoma presents an infiltrating growth pattern that results in a high tendency for local recurrence. […] Clear margins had a positive impact on recurrence-free survival, but did not significantly affect overall patient survival, suggesting that other factors may play a more significant role in determining patient outcomes. […] Consequently, a wider surgical margin may be necessary to reduce the risk of myxofibrosarcoma recurrence. […] Radiotherapy (dosage range: 5065 Gy) was recommended for cases at high risk of local recurrence, specifically those with G3 tumors and a size exceeding 5 cm. […] The type of resection performed, in terms of residual tumor classification, had a significant impact on tumor recurrence (HR 2.4 [95% CI 1.05.6]; p=0.048). […] A surgical margin of 2 mm was insufficient to influence overall survival (HR 0.7 [95% CI 0.22.3]; p=0.631) or local recurrence (HR 1.1 [95% CI 0.33.7]; p=0.888). […] A positive resection margin was associated with adverse prognosis in local recurrence, while this element had no impact on survival. […] This suggests that a wider surgical margin may be necessary to reduce the risk of tumor recurrence.

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

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

  Surgical resection is the mainstay of therapy for soft tissue sarcomas. […] In some small low-grade tumors of the extremities or trunk, surgery alone can be performed without the use of radiation. […] Patient selection factors may vary among surgeons. In general, surgery alone is considered in patients with low-grade tumors of the extremity or superficial trunk that are 5 cm or smaller in diameter (T1) and have microscopically negative surgical margins. […] When feasible, wide-margin function-sparing surgical excision is the cornerstone of effective treatment for extremity tumors. […] This may be facilitated by soft tissue reconstructive surgery, which generally permits wider margins than those obtained when the surgical plan involves direct closure of the excision site. […] Cutting into the tumor mass or shelling out the gross tumor is associated with an elevated risk of local recurrence.

• #14 Pathology Outlines – Myxofibrosarcoma

  https://www.pathologyoutlines.com/topic/softtissuemyxofibrosarcoma.html

  Complete surgical excision with negative margins: 2 cm margin of surrounding normal tissue / fascial plane / periosteum (Surg Oncol Clin N Am 2016;25:775) […] Adjuvant radiotherapy for local control (Sarcoma 2019;2019:8517371) […] Staged resection and reconstruction for local control (Eur J Surg Oncol 2021;47:2627) […] Role of chemotherapy and targeted therapy is under investigation (Clin Sarcoma Res 2017;7:16, Curr Oncol 2021;28:813, Medicine (Baltimore) 2021;100:e25262, Mol Clin Oncol 2020;13:54)

• #15 Myxofibrosarcoma – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/myxofibrosarcoma/

  Treatment depends on the characteristics of the tumor, including: […] MFS always requires surgery to remove the cancer and a margin of healthy tissue around it. The extent of surgery varies according to tumor size and other characteristics. Radiation therapy usually supplements surgical treatment. […] In the past, as many as half the MFS patients were initially treated with amputation of the affected limb. Today, surgical, medical and radiation oncologists make limb preservation a priority whenever possible. In many cases, plastic surgeons also are involved in the initial surgery, laying the groundwork for reconstructive procedures to restore limb function.

• #16 Myxofibrosarcoma | Sarcoma UK

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

  The main treatment for myxofibrosarcoma is surgery. If you have surgery, your surgeon will remove the tumour. Theyll also aim to take out an area of normal tissue too. Sometimes people call this taking a margin. They do this to make sure they remove all of the cancer. […] If your tumour is in your arms or legs, your surgeon will perform limb-sparing surgery. They do this to make sure the limb can continue to work well. […] If the cancer has spread, your surgeon may need to perform a partial or full amputation to stop the cancer although this is very rare. […] Radiotherapy is a treatment that uses radiation to kill cancer cells. […] You might have it before or after surgery. When used before surgery, it aims to make the tumour smaller so that its easier to remove. When used after surgery, it aims to kill off any cancer cells left after your operation.

• #17 Myxofibrosarcoma Tumor Removal Patient Testimonial

  https://www.orlandoortho.com/myxofibrosarcoma-tumor-removal-patient-testimonial/

  Fearing the worst, and in a determined effort to save her life, Marla turned to Craig P. Jones, M.D., a board-certified orthopaedic surgeon specializing in orthopaedic oncology at the Orlando Orthopaedic Center, to surgically remove the fast-growing tumor. […] Surgery is always required to remove the tumor and some normal tissue that surrounds it. Radiation therapy is typically administered after surgery. Some smaller, low-grade, and surface MFS tumors may only need surgery if the cancer is not located close to a joint or other critical bodily structures. […] Given the aggressive nature of Marlas tumor, Dr. Jones decided to perform chemotherapy before surgery and radiation treatment following the procedure. […] Marla underwent two cycles of chemotherapy. A repeat MRI showed that the mass had gotten smaller which made the surgery less invasive. After surgery, Marla underwent radiation therapy to reduce the likelihood of local recurrence. […] Traditionally, almost half of MFS patients underwent amputation of the diseased limb to completely remove the tumor and prevent a recurrence of cancer. However, with the advances in modern surgical techniques, orthopaedic oncologists make it a priority to preserve a patients limb whenever possible.

• #18 A Second Opinion Changed Joel’s Grade 2 Myxofibrosarcoma Treatment Plan – The Patient Story | For Cancer Patients & Caregivers

  https://thepatientstory.com/patient-stories/sarcoma/soft-tissue/myxofibrosarcoma/joel-s/

  Joel’s grade 2 myxofibrosarcoma treatment plan consists of radiation therapy, which he undergoes daily for a few minutes. […] After radiation, surgeons will remove the tumor and perform a skin graft and muscle repositioning to help the area heal properly. […] Treatments: Radiation therapy, Surgery: tumor removal & reconstruction of affected area.

• #19

  https://content.irisoncology.com/library/myxofibrosarcoma

  Sometimes radiation therapy and chemotherapy are used before surgery to shrink the cancer. This makes it easier to remove and might reduce the risk that the cancer will come back. […] Radiation therapy uses powerful energy beams to kill cancer cells. The energy beams can come from X-rays, protons and other sources. […] Radiation might be done before surgery to help shrink the myxofibrosarcoma. It also can be done after surgery to kill any cancer cells that might be left behind. […] Chemotherapy treats cancer with strong medicines. It might help control a myxofibrosarcoma that’s growing quickly. It might be an option if myxofibrosarcoma spreads to other parts of the body. […] Clinical trials are studies of new treatments. These studies provide a chance to try the latest treatments. The risk of side effects might not be known. Ask your healthcare team if you might be able to be in a clinical trial.

• #20 Management Of Recurrent Myxofibrosarcoma – Klarity Health Library

  https://my.klarity.health/management-of-recurrent-myxofibrosarcoma/

  Neoadjuvant therapy is when treatment (radiotherapy or chemotherapy) is first given to shrink the tumour so that surgery can then be performed to remove the reduced tumour. A larger tumour is also more likely to lead to recurrence because there is a higher chance that some tumour cells are left behind following resection surgery. […] If the cancer has metastasised (spread) to other parts of the body, chemotherapy tends to be the answer. […] Usually, the first line of chemotherapeutic drugs given are anthracycline (also known as doxorubicin) and ifosfamide, either separately or together, and sometimes along with dacarbazine. As a second line of treatment, the drugs often prescribed include gemcitabine, either with or without docetaxel. […] Another treatment option for myxofibrosarcoma is radiation therapy.

• #21 Primary thyroid gland myxofibrosarcoma: a case report and review of the literature | Surgical Case Reports | Full Text

  https://surgicalcasereports.springeropen.com/articles/10.1186/s40792-022-01496-5

  Myxofibrosarcoma is a common soft tissue sarcoma of the extremities, which occurs very rarely in the thyroid gland. […] Myxofibrosarcoma of the thyroid gland is extremely rare. The diagnosis is based on histopathological features. Radical surgery achieving tumor-free resection margins remains the only chance for cure. However, the role of radiotherapy and/or chemotherapy is still under debate. Due to their high tendency for locoregional recurrence, a close follow-up after surgery is mandatory. […] Independent of the localization, complete resection of MFS with tumor-free resection margins remains the gold standard for optimal local tumor control. […] For large high-grade intramuscular MFS, adjuvant chemo- or radiotherapy may be indicated. […] The existing case series, retrospective studies, and case reports regarding the use of radiotherapy in the treatment of MFS, mostly in an adjuvant setting, show conflicting results.

• #22 Myxofibrosarcoma landscape: diagnostic pitfalls, clinical management and future perspectives

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9244941/

  The beneficial effect of RT on local tumor control for MFS is still unclear. […] Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. […] Targeted therapies may represent a powerful tool to improve the current therapeutic outcome of standard CT. […] High-intensity focused ultrasound (HIFU) is a noninvasive heating technique that allows ablation of the central tumor zone inducing thermal coagulation necrosis, thus resulting in precise and localized ablation. […] From an immunogenomic point of view, sarcomas carrying complex karyotypes such as MFS are known to display a heavily immune infiltrated tumor microenvironment (TME), making these tumors likely to be responsive to immunotherapy. […] Despite the fact that many efforts have been put into sarcoma research in recent years, the advances made in unraveling the pathogenesis and development of MFS have not led to a substantial improvement of the clinical outcome.

• #23 Myxofibrosarcoma: Causes, Symptoms, and Treatment Options

  https://sehathub.com/myxofibrosarcoma

  Surgery remains the primary treatment approach, with wide excision being crucial to reduce the high recurrence rates, which range from 10% to 61%. […] The most common treatment for MFS is surgery. However, radiation and chemotherapy are also suitable options for treating and managing the condition. […] Surgery is the standard treatment for localized MFS, aiming for wide excision with negative margins (no cancer cells at the edges). […] The beneficial effect of RT on local tumors for treating MFS and clearing it out is still controversial. […] Chemotherapy is not standard for localized MFS but may be considered for high-grade or metastatic cases. […] Targeted therapies can target specific growth pathways involved in cancer development and inhibit the development of cancer cells. The healthcare provider uses immunotherapy to treat MFS.

• #24 Primary thyroid gland myxofibrosarcoma: a case report and review of the literature | Surgical Case Reports | Full Text

  https://surgicalcasereports.springeropen.com/articles/10.1186/s40792-022-01496-5

  The role of chemotherapy in the treatment of MFS remains unclear. […] Radical and wide surgical resection is still the cornerstone in the treatment of MFS and a close clinical follow-up combined with a CT-scan or MRI is mandatory. In addition, adjuvant radiotherapy may play a role in preventing locoregional recurrence. However, the existing data regarding radio- and chemotherapy are from retrospective studies and case series of low evidence. Accordingly, this elucidates the urgent need of multicentric and randomized controlled clinical trials that specifically focus on this aggressive tumor entity.

• #25 Myxofibrosarcoma | Sarcoma UK

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

  Chemotherapy is a treatment that uses medicine to kill cancer cells. […] Its sometimes used if you have a high risk of the cancer coming back or your tumour has spread to other parts of the body (also known as metastasis). […] Its not clear how well chemotherapy works for people with myxofibrosarcoma. Some types of chemotherapy have shown to work well while others have not. You may be more likely to have surgery (with or without radiotherapy) as your main treatment. […] Researchers are learning more about treatments called targeted therapies and immunotherapies. […] Clinical trials have found that some targeted therapies and immunotherapies work well for myxofibrosarcoma. But more research is needed before these treatments can be approved.

• #26 Myxofibrosarcoma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/myxofibrosarcoma/

  The goal of surgery is to cut out as much of the cancer as possible. If all the myxofibrosarcoma is removed, surgery might be the only treatment needed. […] Sometimes radiation therapy and chemotherapy are used before surgery to shrink the cancer. This makes it easier to remove and might reduce the risk that the cancer will come back. […] Radiation therapy uses powerful energy beams to kill cancer cells. The energy beams can come from X-rays, protons and other sources. […] Radiation might be done before surgery to help shrink the myxofibrosarcoma. It also can be done after surgery to kill any cancer cells that might be left behind. […] Chemotherapy treats cancer with strong medicines. It might help control a myxofibrosarcoma that’s growing quickly. It might be an option if myxofibrosarcoma spreads to other parts of the body.

• #27 Myxofibrosarcoma – Wikipedia

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

  Recurrent and metastatic MFS first-line treatment has employed two chemotherapy drugs, anthracycline and Ifosfamide, while second-line treatment has employed two other chemotherapy drugs, gemcitabine and paclitaxel. […] Current drug therapies that are or may soon be tried in treating MFS include angiogenesis inhibitors and immunotherapeutics such as Bevacizumab and Nivolumab.

• #28 Undifferentiated Pleomorphic Sarcoma/Myxofibrosarcoma/Other Fibrosarcomas | Oncohema Key

  https://oncohemakey.com/undifferentiated-pleomorphic-sarcomamyxofibrosarcomaother-fibrosarcomas/

  For localized disease, the treatment of high-grade tumors includes a multimodality approach (perioperative chemotherapy, radiation therapy, and surgery), followed by close surveillance with scans and follow-up visits. […] For metastatic disease, these patients will require systemic therapies as well as radiation therapy and surgery in selected patients. […] Wide excision plus radiotherapy remains the cornerstone of treatment of nonmetastatic tumors, and it is considered the standard of care. […] Neoadjuvant or adjuvant anthracycline-based systemic chemotherapy is a consideration for patients with large (5 cm), high-grade UPS per current consensus guidelines. […] For patients with resectable, localized UPS who are not being treated on a clinical trial, wide surgical excision with adjuvant or neoadjuvant radiotherapy remains the standard of care; anthracycline-based chemotherapy with doxorubicin and ifosfamide should be considered for high-risk patients with good performance status and preserved organ function.

• #29 Gemcitabine-Containing Chemotherapy for the Treatment of Metastatic Myxofibrosarcoma Refractory to Doxorubicin: A Case Series

  https://www.mdpi.com/1718-7729/28/1/78

  Gemcitabine-Containing Chemotherapy for the Treatment of Metastatic Myxofibrosarcoma Refractory to Doxorubicin: A Case Series […] The goal of this study was to evaluate the activity of gemcitabine-containing regimens for the treatment of metastatic myxofibrosarcoma refractory to doxorubicin. […] Gemcitabine-based chemotherapy was associated with encouraging response rates in this cohort, similar to those seen in UPS. […] Our study showed that gemcitabine in second-line setting in metastatic myxofibrosarcoma was associated with encouraging response rates, similar to those observed in UPS, further hinting at the similarities between these two sarcoma types. […] On the basis of the above-mentioned relationship between UPS and MFS and the promising response of MFS to gemcitabine, we agree that both entities could be studied together for novel gemcitabine-based regimens, as already done by some investigators.

• #30 Treatment Options For Myxofibrosarcoma – Klarity Health Library

  https://my.klarity.health/treatment-options-for-myxofibrosarcoma/

  To make tumours smaller, radiotherapy may be administered using radiation. To remove all cancer cells, it may be given after surgery. […] Chemotherapy is one of the standard treatments for various cancers which work by inhibiting or slowing the growth of cancer cells. […] As the current treatments including surgery, chemotherapy, and radiation have a chance of cancer coming back and are associated with high side effects, researchers are trying to explore new treatments to reduce limitations, which are targeted therapy and immunotherapy. […] Immunotherapy encourages your immune system to fight cancer cells. Studies of Immunotherapy on myxofibrosarcoma are emerging as its promising potential. […] After treatment, you still have follow-up appointments in order to check your symptoms and examine signs of cancer coming back. […] Myxofibrosarcoma is one type of soft tissue sarcoma or cancer, however, its causes remain unclear. Therefore, current treatments including surgery, radiation, and chemotherapy are broad and not specific to the causes of cancer, leading to several side effects on healthy cells.

• #31 Biology and Management of High-Grade Myxofibrosarcoma: State of the Art and Future Perspectives

  https://www.mdpi.com/2075-4418/13/19/3022

  Pazopanib is an oral multitargeted tyrosine kinase inhibitor (TKI) with anti-angiogenic and anti-tumorigenic properties and it has been approved in multiple countries as a second- or later-line treatment for patients with advanced non-adipocytic STS. […] The major targets of FDA-approved immunotherapeutic antibodies are programmed cell death protein-1 (PD-1) and its ligand, programmed cell death ligand-1 (PD-L1). […] High-intensity focused ultrasound (HIFU) is a minimally invasive treatment modality that can ablate target tissue or tumors within the body.

• #32 Undifferentiated Pleomorphic Sarcoma/Myxofibrosarcoma/Other Fibrosarcomas | Oncohema Key

  https://oncohemakey.com/undifferentiated-pleomorphic-sarcomamyxofibrosarcomaother-fibrosarcomas/

  In the metastatic setting, doxorubicin as a single agent or in combination with ifosfamide is likely to be the first choice of chemotherapy. […] Other drugs such as trabectedin, gemcitabine, docetaxel, and pazopanib also have shown activity in the advanced setting. […] Emerging data on the use of checkpoint inhibitors opens new potential treatment options for patients with advanced disease. […] The dynamic evolution of the immunotherapy and targeted therapy fields forecasts the development of new clinical trials for these patients.

• #33 Combination nivolumab and bevacizumab for metastatic myxofibrosarcoma: A case report and review of the literature

  https://www.spandidos-publications.com/10.3892/mco.2020.2124

  The main therapeutic strategy for metastatic Myxofibrosarcoma (MFS) is palliative chemotherapy. […] A number of studies have demonstrated that antiangiogenic therapy and immunotherapy could improve the survival rate of patients with metastases. […] The current study reports a case of metastatic myxofibrosarcoma that was treated with combination Nivolumab (monoclonal antibody, PD1 inhibitor) and Bevacizumab (monoclonal antibody, antiVEGF) after progression from the single use of Nivolumab. […] The aim of the current study is to assess the efficacy and safety of Nivolumab and Bevacizumab for metastatic myxofibrosarcoma and to review the literature. […] Nivolumab and Bevacizumab treatment indicate beneficial clinical effects and are indicated to be safe to use in patients with metastatic myxofibrosarcoma.

• #34 A Rare Case Of Recurrent Myxofibrosarcoma And The Role Of Immunotherapy; A New Treatment Strategy | Cureus

  https://www.cureus.com/posters/2147-a-rare-case-of-recurrent-myxofibrosarcoma-and-the-role-of-immunotherapy-a-new-treatment-strategy

  A Rare Case Of Recurrent Myxofibrosarcoma And The Role Of Immunotherapy; A New Treatment Strategy […] Myxofibrosarcoma (MFS) is a rare malignant soft-tissue sarcoma characteristically presenting as a painless slowly growing mass in the extremities of elderly males. It is a malignant tumour with a high risk of local recurrence and a low risk of distant metastasis. It tends to become a progressively higher grade in recurrences. Local occurrences of MFS are treated surgically but metastatic presentations do not have effective treatment modalities. The recent development in genomic studies has provided us with an advanced treatment modality in the form of immunotherapy. Programmed Death Ligand 1 (PDL1) is a therapeutic target found in sarcomas. Pembrolizumab, a monoclonal antibody targeting PDL1, is highly effective against tumours with this marker. […] Treatment options for metastatic sarcomas are very minimal, consisting of toxic chemotherapeutic drugs. Genomic studies provide a targeted approach for the treatment of metastatic MFS using immunotherapy. With an 80% PDL1 positivity rate, treatment with the first cycle of Pembrolizumab showed a significant improvement in pain levels. Immunotherapeutic medications have a milder side effect profile while being more efficacious.

• #35 Pembrolizumab for Refractory Metastatic Myxofibrosarcoma: A Case Report

  https://www.e-crt.org/journal/view.php?number=2827

  Myxofibrosarcoma is a rare tumor, refractory to cytotoxic chemotherapy and radiotherapy. […] Pembrolizumab is an innovative immunotherapy drug consisting of programmed death receptor ligand 1 antibody proven to be useful for numerous types of cancer cells. […] The patient achieved a partial response during palliative chemotherapy with pembrolizumab for 14 cycles. […] This report represents the first evidence of pembrolizumab exhibiting efficacy against refractory metastatic MFS without any toxicity. […] In conclusion, we believe pembrolizumab could be an option for controlling metastatic sarcoma, including MFS without degrading the patients quality of life. Further studies of the efficacy and biomarkers of pembrolizumab for refractory metastatic sarcoma are warranted.

• #36

  https://journals.lww.com/md-journal/fulltext/2021/03260/remarkable_response_to_anti_pd1_immunotherapy_in.101.aspx

  Myxofibrosarcoma (MFS) is a locally aggressive tumor and has the potential to be fatal because of distant metastasis. […] Although the immunotherapy has been applied in sarcoma, there is little information about the efficiency to treat metastatic MFS. […] Thus, he received Camrelizumab (PD-1 inhibitor). […] Six months after the initiating immunotherapy of Camrelizumab, the size of pulmonary lesions showed marked shrinkage, indicating a partial response. […] This case described here demonstrated that immunotherapy of PD-1 inhibitor is a promising treatment option for refractory MFS with PD-L1 positive or tumor mutational burden -high, which could contribute to effective tumor response. […] Margin-negative surgical resection of the mass is the most common treatment for myxofibrosarcoma patients.

• #37

  https://journals.lww.com/md-journal/fulltext/2021/03260/remarkable_response_to_anti_pd1_immunotherapy_in.101.aspx

  Immunotherapy is a novel approach for tumor treatment. […] A multicenter, open-label phase 2 trial included the treatment of soft tissue sarcoma with nivolumab offered no evidence of clinical activity in soft tissue sarcoma containing 2 patients with MFS. […] Hence, a unique patient of metastatic high-grade myxofibrosarcoma with marked response to the administration of PD-1 blockade was presented. […] The patient was administrated with anti-PD1 immunotherapy and a partial response was obtained. […] Therefore, for severely metastatic myxofibrosarcoma with no response to conventional therapeutic options, immunotherapy is a promising selection.

• #38 UCSF Myxofibrosarcoma Clinical Trials for 2025 — San Francisco Bay Area

  https://clinicaltrials.ucsf.edu/myxofibrosarcoma

  The objective of this study is to assess the safety and efficacy of mecbotamab vedotin (BA3011) in solid tumors. […] This randomized phase II/III trial studies how well pazopanib, when combined with chemotherapy and radiation therapy or radiation therapy alone, work in the treatment of patients with newly diagnosed non-rhabdomyosarcoma soft tissue sarcomas that can eventually be removed by surgery. Radiation therapy uses high energy x-rays to kill tumor cells. Drugs used in chemotherapy, such as ifosfamide and doxorubicin, work in different ways to stop the growth of tumor cells, either by killing the cells, by stopping them from dividing, or by stopping them from spreading. Pazopanib may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth. It is not yet known whether these therapies can be safely combined and if they work better when given together in treating patients with non-rhabdomyosarcoma soft tissue sarcomas.

• #39 University of California Health Myxofibrosarcoma Clinical Trials for 2025 — California

  https://clinicaltrials.ucbraid.org/myxofibrosarcoma

  The objective of this study is to assess the safety and efficacy of mecbotamab vedotin (BA3011) in solid tumors. […] This is a multicenter open-label, randomized, non-comparative, parallel cohort pivotal study of treatment with envafolimab (cohort A and C) or envafolimab combined with ipilimumab (cohort B and D) in patients with locally advanced, unresectable or metastatic undifferentiated pleomorphic sarcoma (UPS)/myxofibrosarcoma (MFS) who have progressed on one or two lines of chemotherapy. […] This phase I trial studies the side effects of BO-112 when given together with nivolumab before surgery in treating patients with soft tissue sarcoma that can be removed by surgery (resectable). […] This randomized phase II/III trial studies how well pazopanib, when combined with chemotherapy and radiation therapy or radiation therapy alone, work in the treatment of patients with newly diagnosed non-rhabdomyosarcoma soft tissue sarcomas that can eventually be removed by surgery.

• #40 Myxofibrosarcoma – Symptoms, Treatment & Support – Without a Ribbon

  https://withoutaribbon.org/myxofibrosarcoma-symptoms-treatment-support/

  Surgical resection (surgical removal of the tumor part of the skin along with the removal of some surrounding healthy tissues) of the tumor along with the chemotherapy and radiation is the main treatment option.

• #41 Myxofibrosarcoma | Norton Children’s

  https://nortonchildrens.com/services/cancer/conditions/sarcoma/myxofibrosarcoma/

  The board-certified and fellowship-trained oncologists at Norton Children’s Cancer Institute come together at regular conferences to share viewpoints from various perspectives that help determine the best course of treatment. It’s like getting second, third and fourth opinions all at once. […] By staying at the forefront of research, our physicians are experienced with newly approved therapies for myxofibrosarcoma and can provide access to trials of experimental treatments. […] Treatment includes surgery to remove the tumor and adjacent tissue to reduce the risk of recurrence. Radiation therapy may be used to help shrink the tumor before or after surgery, especially in cases of high-grade (faster growing and spreading cells) myxofibrosarcoma, when the tumor is larger than 2 inches or has grown into deeper tissues. Chemotherapy typically is not used to treat myxofibrosarcoma.

• #42 Management Of Recurrent Myxofibrosarcoma – Klarity Health Library

  https://my.klarity.health/management-of-recurrent-myxofibrosarcoma/

  Myxofibrosarcoma is a malignant cancer that affects soft tissue and can have a high local recurrence rate, meaning that it returns in a specific area frequently. This makes it a tricky disease to treat and can be devastating to live with. However, understanding the nature of recurrent myxofibrosarcoma and management options can empower patients and families with knowledge, providing them with guidance through this difficult period. […] There are a number of treatment options available for myxofibrosarcoma and they are dependent on the stage, grade, size and depth of the cancer. It is important to discuss which one is best for you with your healthcare team. […] If the cancer is localised and has not spread to any other sites on the body yet, then surgical resection coupled with neoadjuvant radiotherapy and chemotherapy is the primary method of treatment.

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

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

  Even for high-grade disease, soft tissue sarcomas of the extremities can usually be effectively treated while preserving the limb with combined-modality treatment consisting of preRT or PORT to reduce local recurrence. […] Local control of high-grade soft tissue sarcomas of the trunk and the head and neck can be achieved with surgery in combination with radiation therapy. […] Effective treatment of retroperitoneal sarcomas requires removal of all gross disease while sparing adjacent viscera not invaded by tumor. […] Local disease control is crucial in patients with retroperitoneal sarcomas. […] In the setting of distant metastasis, surgery may be associated with long-term disease-free survival (DFS) in patients with pulmonary metastasis and optimal underlying disease biology. […] The role of adjuvant chemotherapy remains controversial.

• #44 Myxofibrosarcoma – DoveMed

  https://www.dovemed.com/diseases-conditions/myxofibrosarcoma

  The treatment of choice for Myxofibrosarcoma is a complete surgical excision. Since tumor recurrence and metastasis is commonly noted, radiation therapy and/or chemotherapy may be necessary […] Wide surgical excision of MFS with removal of the entire tumor is generally adopted. However, often a complete excision is difficult due to infiltrative growth and involvement of surrounding tissue structures. […] Radiation therapy and/or chemotherapy may be used to destroy the remaining tumor cells, based on the assessment by the healthcare provider. […] Post-operative care is important; a minimum activity level is to be ensured until the surgical wound heals. […] Follow-up care with regular screening and check-ups are important; a long-term follow-up is recommended.

• #45 FIBROSARCOMA – MYXOFIBROSARCOMA

  https://www.seyitaligumustas.com/en/fibrosarcoma-myxofibrosarcoma

  Chemotherapy is not routinely used in patients with fibrosarcoma. Chemotherapy is used for large and deep-seated tumors, especially in the presence of metastases. […] Patients diagnosed with fibrosarcoma should be followed up for many years at regular intervals for recurrence and metastasis, which means spread to other parts of the body.

• #46 Prognostic Factors and Outcomes for Patients With Myxofibrosarcoma: A 13-Year Retrospective Evaluation | Anticancer Research

  https://ar.iiarjournals.org/content/39/6/2985

  Tumour grade and metastasis were significant prognostic factors of survival (log-rank test p=0.041 and p=0.00007). […] The understanding of the molecular biological patterns that result in the metastasis of these tumours will help develop better treatment plans in the future. […] The survival rate was significantly affected by the tumour grade and presence of metastases. Patients with metastases had significantly worse overall survival than patients without metastases (hazard ratio=2.97, p=0.005) and than patients with G3 MFS (hazard ratio=1.56, p=0.024). […] Negative margins after the first surgery are an important finding that can guide the treatment process, emphasizing the need for primary treatment at a referral hospital.

• #47 Analysis of clinical factors impacting recurrence in myxofibrosarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-024-53606-y

  Myxofibrosarcoma (MFS) is a malignant fibroblastic/myofibroblastic neoplasm with a prominent myxoid area. It has the clinical features of frequent local recurrence (LR) and occasional distant metastasis. […] The probability of LR in patients with MFS was significantly greater in association with unplanned operations, positive margins, low serum CK levels or superficial tumor depth. These data could help identify high-risk patients; thus, more careful follow-up should be performed for higher-risk patients. Diagnosis and treatment at qualified regular medical centers can reduce the local recurrence rate of MFS. […] Surgery represents the primary therapeutic intervention for MFS, and the importance of clear surgical margins has been extensively delineated in the literature. […] In this study, we found that the likelihood of recurrence in patients who underwent planned surgery was significantly lower than that in patients who underwent unplanned surgery.

• #48 Analysis of prognostic factors in 171 patients with myxofibrosarcoma of the trunk and extremities: a cohort study

  https://atm.amegroups.org/article/view/77255/html

  A negative surgical margin can be reduced effectively the rate of recurrence and metastasis in patients with MFS of the trunk and limbs. […] The multivariate analysis showed that the surgical margin [hazard ratio (HR) =3.635, 95% confidence interval (CI): 1.8837.016, P0.001] and tumor size (HR =1.889, 95% CI: 1.0393.435, P=0.037) were risk factors for local recurrence. […] The surgical margin (HR =4.475, 95% CI: 1.91810.438, P=0.001) and presence of CD44 (HR =3.406, 95% CI: 1.4628.405, P=0.005) were risk factors for distant metastasis. […] Extended resection is the first-choice therapy for patients with MFS. […] For those with a positive incision margin, appropriate adjuvant therapy may reduce the mortality rate caused by the recurrence and metastasis of tumors.

• #49 Analysis of clinical factors impacting recurrence in myxofibrosarcoma | Scientific Reports

  https://www.nature.com/articles/s41598-024-53606-y

  This study reinforces the critical role of negative margins in determining LR outcomes. […] Some scholars have advocated for the use of postoperative radiotherapy in patients with insufficient incisal margins, as a means to increase the likelihood of controlling recurrence. However, the practical role of radiotherapy in treating MFS remains unclear. […] Research indicates that standardized adjuvant radiotherapy may ameliorate the recurrence rate in such patients, albeit not to the level achieved with specialized treatment at a sarcoma center from the outset. […] The management of MFSs presents several important challenges. First, MFS is characterized by a high propensity for recurrence, often necessitating limb amputation, which underscores the complexity of its treatment.

• #50 Myxofibrosarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/myxofibrosarcoma?lang=us

  Myxofibrosarcoma is a malignant soft tissue tumor. It is classified as a type of a fibroblastic/myofibroblastic tumor. […] Prognosis will somewhat depend on grade.

• #51 Myxofibrosarcoma

  https://www.mymlc.com/health-information/diseases-and-conditions/m/myxofibrosarcoma/

  Myxofibrosarcoma (MFS) is a type of cancer that typically appears as a slow-growing, painless lump on one of your legs or arms. […] MFS is a soft tissue sarcoma. […] Although MFS tends to recur in the same area where it started, a majority of patients live five or more years after treatment. […] Treatment depends on the characteristics of the tumor, including: […] MFS always requires surgery to remove the cancer and a margin of healthy tissue around it. […] Radiation therapy usually supplements surgical treatment. […] In the past, as many as half the MFS patients were initially treated with amputation of the affected limb. Today, surgical, medical and radiation oncologists make limb preservation a priority whenever possible.

• #52 Primary cardiac myxofibrosarcoma of the left atrium and pericardium: a case report | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-023-02441-9

  Primary cardiac myxofibrosarcoma is rare and commonly occurs in the left atrium. Complete surgical resection is considered important; however, effective treatment options have not been established. […] Considering preoperative laboratory findings, surgical and adjuvant therapy, and the patients wishes are important for the best therapeutic course for an individual. […] Complete surgical resection is considered important to improve the prognosis; however, effective treatment options have not been established. […] The median survival time of primary cardiac myxofibrosarcoma patients treated with tumor resection is only 14 months. […] Although complete surgical resection is important for prolonged survival, it is difficult, and only occurs in one-third of cases. […] Therefore, if the tumor is a high grade myxofibrosarcoma or cannot be completely removed, additional radiotherapy and chemotherapy should be considered.

• #53 Management Of Recurrent Myxofibrosarcoma – Klarity Health Library

  https://my.klarity.health/management-of-recurrent-myxofibrosarcoma/

  Most patients who undergo radiotherapy tend to have tumours that are bigger than 5cm in diameter. The primary function of radiotherapy in this context is likely to be as part of neoadjuvant radiotherapy. It may, however, also be given as adjuvant therapy, which is when radiotherapy is given following surgery, to decrease the chance of recurrence. […] Targeted therapy is a type of treatment where we specifically target proteins that contribute to the growth and spread of the cancer. Currently, the most common targeted therapy used for myxofibrosarcoma involves inhibiting the progression of tumour cells through the cell cycle. […] There are many advances in treatment for myxofibrosarcoma, with current research into immunotherapies and targeted therapies showing promising results. […] From surgery to clinical trials and targeted therapy, it is important to discuss all possible treatment plans with the healthcare team before committing to one.

• #54 Myxofibrosarcoma – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/myxofibrosarcoma/

  The management of myxofibrosarcoma (MFS) requires a comprehensive approach due to its high recurrence rates and complex nature. Surgical treatment remains the cornerstone, with the goal of achieving an R0 resection to minimize local recurrence. […] Radiation therapy is often used alongside surgery to further reduce the risk of recurrence, although its effectiveness is still being studied. […] Chemotherapy is reserved for specific cases where the cancer is particularly aggressive or has spread. […] Emerging treatments, such as immunotherapy and targeted therapies, are being investigated and hold promise for future management strategies. Regular follow-up care is essential to monitor for recurrence, which is a significant concern for MFS patients. Factors such as tumor size, patient age, and surgical margins play critical roles in determining patient outcomes. Clinical trials continue to explore new treatment combinations, aiming to improve survival rates and quality of life for patients with advanced soft tissue sarcomas.

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