Materiały źródłowe

• #1 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. The disease occurs mainly in people between the ages of 50 and 70 and is slightly more common in men than in women. […] 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.

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

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

• #3 What Is Myxofibrosarcoma? – Klarity Health Library

  https://my.klarity.health/what-is-myxofibrosarcoma/

  Given its aggressive behaviour, timely diagnosis, treatment, and ongoing monitoring are vital in effectively managing myxofibrosarcoma. […] Treatment for myxofibrosarcoma involves several considerations, including tumour grade, stage, size, and depth. […] In many cases, surgical removal of the tumour along with a margin of surrounding tissue is the primary treatment. […] The treatment plan will be tailored to your situation, aiming to effectively address the cancer while minimising potential side effects. Consulting with a healthcare provider experienced in treating myxofibrosarcoma is crucial for making informed decisions about your treatment journey. […] Myxofibrosarcoma displays a higher tendency to recur post-treatment compared to other variants of soft tissue sarcomas. It’s estimated that up to 50% of individuals with myxofibrosarcoma experience a recurrence within five years of treatment. […] Regular consultations with your healthcare provider heighten the possibility of timely detection and intervention if myxofibrosarcoma reoccurs.

• #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. […] 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. […] Myxofibrosarcoma is more likely to come back after treatment than other types of soft tissue sarcomas. Up to 1 in 2 people have myxofibrosarcoma return within five years of treatment. […] 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 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #6 Myxofibrosarcoma // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/myxofibrosarcoma

  Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you. If your healthcare professional thinks that you might have myxofibrosarcoma, you may be referred to a specialist. Specialists who care for people with myxofibrosarcoma include: Cancer doctors, called medical oncologists. […] Appointments can be short and being prepared can help. Here’s some information that may help you get ready. […] Take a family member or friend along, if possible, to help you remember the information you’re given.

• #7 Myxofibrosarcoma | Norton Children’s

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

  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. […] Norton Children’s Hospital’s cancer care program is one of the oldest oncology programs in the U.S. that has been continuously accredited by the American College of Surgeons’ Commission on Cancer. Backed by nearly 60 years of pediatric expertise, we have a proven cancer care team of more than 200 leading cancer specialists, including oncologists, surgeons, nurses, social workers, chaplains, behaviorists, therapists and pharmacists. This skilled, multidisciplinary team is entirely focused on the needs of your child and family.

• #8 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #9 What Is Myxofibrosarcoma? – Klarity Health Library

  https://my.klarity.health/what-is-myxofibrosarcoma/

  Given its aggressive behaviour, timely diagnosis, treatment, and ongoing monitoring are vital in effectively managing myxofibrosarcoma. […] Treatment for myxofibrosarcoma involves several considerations, including tumour grade, stage, size, and depth. […] In many cases, surgical removal of the tumour along with a margin of surrounding tissue is the primary treatment. […] The treatment plan will be tailored to your situation, aiming to effectively address the cancer while minimising potential side effects. Consulting with a healthcare provider experienced in treating myxofibrosarcoma is crucial for making informed decisions about your treatment journey. […] Myxofibrosarcoma displays a higher tendency to recur post-treatment compared to other variants of soft tissue sarcomas. It’s estimated that up to 50% of individuals with myxofibrosarcoma experience a recurrence within five years of treatment. […] Regular consultations with your healthcare provider heighten the possibility of timely detection and intervention if myxofibrosarcoma reoccurs.

• #10 Myxofibrosarcoma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/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. […] 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 it’s hard to get all the myxofibrosarcoma because this cancer tends to spread out as it grows. It also may grow close to important structures such as blood vessels and nerves that make surgery hard to perform. It can grow deep into the tissue. Sometimes surgeons can’t see all the cancer to make sure it’s removed.

• #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. […] 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. […] 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.

• #12 Myxofibrosarcoma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/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. […] 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 it’s hard to get all the myxofibrosarcoma because this cancer tends to spread out as it grows. It also may grow close to important structures such as blood vessels and nerves that make surgery hard to perform. It can grow deep into the tissue. Sometimes surgeons can’t see all the cancer to make sure it’s removed.

• #13 Myxofibrosarcoma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/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. […] 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 it’s hard to get all the myxofibrosarcoma because this cancer tends to spread out as it grows. It also may grow close to important structures such as blood vessels and nerves that make surgery hard to perform. It can grow deep into the tissue. Sometimes surgeons can’t see all the cancer to make sure it’s removed.

• #14 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 margin resection is challenging because of microscopic infiltration. […] 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. A surgical margin of 2 mm was not sufficient to significantly influence the incidence of recurrence. Consequently, a wider surgical margin may be necessary to reduce the risk of myxofibrosarcoma recurrence. […] 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). […] The recurrence-free survival rate of patients with clear margin resection (60.2%) was higher compared with patients with microscopic infiltration (31.1%) at 5 years after the initial surgical procedure. The effect of radiotherapy is still controversial.

• #15 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 margin resection is challenging because of microscopic infiltration. […] 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. A surgical margin of 2 mm was not sufficient to significantly influence the incidence of recurrence. Consequently, a wider surgical margin may be necessary to reduce the risk of myxofibrosarcoma recurrence. […] 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). […] The recurrence-free survival rate of patients with clear margin resection (60.2%) was higher compared with patients with microscopic infiltration (31.1%) at 5 years after the initial surgical procedure. The effect of radiotherapy is still controversial.

• #16 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

  A positive resection margin was associated with adverse prognosis in local recurrence, while this element had no impact on survival. A surgical margin of 2 mm did not significantly influence the incidence of recurrence. This suggests that a wider surgical margin may be necessary to reduce the risk of tumor recurrence.

• #17 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 margin resection is challenging because of microscopic infiltration. […] 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. A surgical margin of 2 mm was not sufficient to significantly influence the incidence of recurrence. Consequently, a wider surgical margin may be necessary to reduce the risk of myxofibrosarcoma recurrence. […] 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). […] The recurrence-free survival rate of patients with clear margin resection (60.2%) was higher compared with patients with microscopic infiltration (31.1%) at 5 years after the initial surgical procedure. The effect of radiotherapy is still controversial.

• #18 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. […] For patients with a limb tumor that cannot be resected (less than 5% of all cases), limb amputation is the treatment of choice. […] 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.

• #19 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. […] For patients with a limb tumor that cannot be resected (less than 5% of all cases), limb amputation is the treatment of choice. […] 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.

• #20 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. The disease occurs mainly in people between the ages of 50 and 70 and is slightly more common in men than in women. […] 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.

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

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

• #22 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. […] For patients with a limb tumor that cannot be resected (less than 5% of all cases), limb amputation is the treatment of choice. […] 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.

• #23 Myxofibrosarcoma // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/myxofibrosarcoma

  In the past, surgeons often had to remove a limb to get rid of all the cancer. Today that’s often not needed because of newer treatments. 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.

• #24 Myxofibrosarcoma // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/myxofibrosarcoma

  In the past, surgeons often had to remove a limb to get rid of all the cancer. Today that’s often not needed because of newer treatments. 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.

• #25 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. […] 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. […] Myxofibrosarcoma is more likely to come back after treatment than other types of soft tissue sarcomas. Up to 1 in 2 people have myxofibrosarcoma return within five years of treatment. […] 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.

• #26 Myxofibrosarcoma | Beacon Health System

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

  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 Mayo Clinic Health Library – Myxofibrosarcoma | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20357210

  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.

• #28 Myxofibrosarcoma | Beacon Health System

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

  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.

• #29 Mayo Clinic Health Library – Myxofibrosarcoma | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20357210

  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.

• #30 Myxofibrosarcoma | Beacon Health System

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

  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.

• #31 Myxofibrosarcoma of the scalp with difficult preoperative diagnosis: A case report and review of the literature

  https://www.wjgnet.com/2307-8960/full/v8/i11/2350.htm

  Therefore, the grade should be an important reference basis for clinical treatment. […] Given the highly malignant MFS in our case, combined with the anatomic location, size, and other factors, surgery was performed and adjuvant radiotherapy was delivered to avoid local and distant recurrences. […] Although there is no gold standard of treatment, a complete tumor resection with sufficient resection margins, assisted by adjuvant radiotherapy, may be effective. […] The case reported herein of MFS occurred in an extremely rare location on the scalp and had atypical MRI findings, which serves as a reminder to radiologists of the possibility of this diagnosis to assist in clinical treatment. Although there is no gold standard of treatment, a complete tumor resection with clear resection margins, assisted by adjuvant radiotherapy, may be effective.

• #32 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 margin resection is challenging because of microscopic infiltration. […] 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. A surgical margin of 2 mm was not sufficient to significantly influence the incidence of recurrence. Consequently, a wider surgical margin may be necessary to reduce the risk of myxofibrosarcoma recurrence. […] 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). […] The recurrence-free survival rate of patients with clear margin resection (60.2%) was higher compared with patients with microscopic infiltration (31.1%) at 5 years after the initial surgical procedure. The effect of radiotherapy is still controversial.

• #33

  https://www.orthobullets.com/evidence/31662702

  Myxofibrosarcoma (MFS) is one of the more common types of soft-tissue sarcoma (STS) in patients over 60 years of age. Local recurrence (LR) rates have been reported to be higher compared to other STS types. […] The rate of LR for patients with myxofibrosarcoma was high. The impact of RT on local tumor control was unclear. The surgical margin was important for both local and distant tumor control. Large tumor size was a risk factor for distant metastasis.

• #34 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #35 Epidemiology and survival factors of appendicular myxofibrosarcoma: a SEER-retrospective study | Dahl | Reports of Practical Oncology and Radiotherapy

  https://journals.viamedica.pl/rpor/article/view/97733

  The sensitivity of MFS to radiotherapy is unclear, given that high-risk tumors will typically receive (neo)adjuvant therapy in addition to resection. MFS clinically tends to have lower rates of distant metastasis and higher rates of local recurrence than other types of sarcomas. […] This analysis of the SEER database contributes to the breadth and scope of prognostic data available for clinical decision making in patients diagnosed with MFS. MFS are rare tumors and have historically only been investigated in small groups and studies performed at single institutions. The results of this large-scale analysis provided accurate and generalizable data about 5-year and overall survival rates for MFS.

• #36 Myxofibrosarcoma | Norton Children’s

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

  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. […] Norton Children’s Hospital’s cancer care program is one of the oldest oncology programs in the U.S. that has been continuously accredited by the American College of Surgeons’ Commission on Cancer. Backed by nearly 60 years of pediatric expertise, we have a proven cancer care team of more than 200 leading cancer specialists, including oncologists, surgeons, nurses, social workers, chaplains, behaviorists, therapists and pharmacists. This skilled, multidisciplinary team is entirely focused on the needs of your child and family.

• #37 Myxofibrosarcoma of the scalp with difficult preoperative diagnosis: A case report and review of the literature

  https://www.wjgnet.com/2307-8960/full/v8/i11/2350.htm

  Therefore, the grade should be an important reference basis for clinical treatment. […] Given the highly malignant MFS in our case, combined with the anatomic location, size, and other factors, surgery was performed and adjuvant radiotherapy was delivered to avoid local and distant recurrences. […] Although there is no gold standard of treatment, a complete tumor resection with sufficient resection margins, assisted by adjuvant radiotherapy, may be effective. […] The case reported herein of MFS occurred in an extremely rare location on the scalp and had atypical MRI findings, which serves as a reminder to radiologists of the possibility of this diagnosis to assist in clinical treatment. Although there is no gold standard of treatment, a complete tumor resection with clear resection margins, assisted by adjuvant radiotherapy, may be effective.

• #38 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. […] 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. […] Myxofibrosarcoma is more likely to come back after treatment than other types of soft tissue sarcomas. Up to 1 in 2 people have myxofibrosarcoma return within five years of treatment. […] 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.

• #39 Mayo Clinic Health Library – Myxofibrosarcoma | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20357210

  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.

• #40 Myxofibrosarcoma | Beacon Health System

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

  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.

• #41 Mayo Clinic Health Library – Myxofibrosarcoma | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20357210

  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.

• #42 Myxofibrosarcoma | Beacon Health System

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

  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.

• #43 Mayo Clinic Health Library – Myxofibrosarcoma | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20357210

  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.

• #44 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. […] For patients with a limb tumor that cannot be resected (less than 5% of all cases), limb amputation is the treatment of choice. […] 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.

• #45 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. […] For patients with a limb tumor that cannot be resected (less than 5% of all cases), limb amputation is the treatment of choice. […] 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.

• #46 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #47 Epidemiology and survival factors of appendicular myxofibrosarcoma: a SEER-retrospective study | Dahl | Reports of Practical Oncology and Radiotherapy

  https://journals.viamedica.pl/rpor/article/view/97733

  Myxofibrosarcoma (MFS) is a rare malignant soft tissue sarcoma (STS) subset of the heterogenous group of fibrohistiocytic tumors with an estimated prevalence of 0.1/100,000/years. It is an aggressive soft tissue malignancy that presents with a high recurrence and metastatic rate. Current clinical management for MFS is dependent on characteristics of the malignancy, including depth, size, grade, and episodes of previous recurrence. MFS always requires surgical resection as part of the treatment plan and radiation therapy is generally supplementary to surgical treatment. […] Surgical resection is the cornerstone in the treatment of STS and this is described as well in patients with MFS. Our study confirmed that surgical excision greatly increases survival. Previous reports have shown mixed benefits of using chemotherapy and/or radiotherapy for MFS. Our study showed no clear evidence that survival improves with either of these treatment options, as we observed that chemotherapy in stage III and IV patients increased risk of death and combined chemo- and radiotherapy increased risk of death for those in stages I and II.

• #48 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #49 Epidemiology and survival factors of appendicular myxofibrosarcoma: a SEER-retrospective study | Dahl | Reports of Practical Oncology and Radiotherapy

  https://journals.viamedica.pl/rpor/article/view/97733

  Myxofibrosarcoma (MFS) is a rare malignant soft tissue sarcoma (STS) subset of the heterogenous group of fibrohistiocytic tumors with an estimated prevalence of 0.1/100,000/years. It is an aggressive soft tissue malignancy that presents with a high recurrence and metastatic rate. Current clinical management for MFS is dependent on characteristics of the malignancy, including depth, size, grade, and episodes of previous recurrence. MFS always requires surgical resection as part of the treatment plan and radiation therapy is generally supplementary to surgical treatment. […] Surgical resection is the cornerstone in the treatment of STS and this is described as well in patients with MFS. Our study confirmed that surgical excision greatly increases survival. Previous reports have shown mixed benefits of using chemotherapy and/or radiotherapy for MFS. Our study showed no clear evidence that survival improves with either of these treatment options, as we observed that chemotherapy in stage III and IV patients increased risk of death and combined chemo- and radiotherapy increased risk of death for those in stages I and II.

• #50 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #51 Myxofibrosarcoma – DoveMed

  https://www.dovemed.com/diseases-conditions/myxofibrosarcoma?trendmd-shared=0

  The treatment of Myxofibrosarcoma is undertaken as follows: […] 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.

• #52 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. […] 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. […] Myxofibrosarcoma is more likely to come back after treatment than other types of soft tissue sarcomas. Up to 1 in 2 people have myxofibrosarcoma return within five years of treatment. […] 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.

• #53 Myxofibrosarcoma – DoveMed

  https://www.dovemed.com/diseases-conditions/myxofibrosarcoma?trendmd-shared=0

  The treatment of Myxofibrosarcoma is undertaken as follows: […] 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.

• #54 Myxofibrosarcoma – DoveMed

  https://www.dovemed.com/diseases-conditions/myxofibrosarcoma?trendmd-shared=0

  The treatment of Myxofibrosarcoma is undertaken as follows: […] 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.

• #55 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. […] 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. […] Myxofibrosarcoma is more likely to come back after treatment than other types of soft tissue sarcomas. Up to 1 in 2 people have myxofibrosarcoma return within five years of treatment. […] 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.

• #56 Myxofibrosarcoma – Overview of Information and Clinical Research

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

  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. […] 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. […] Living with MFS involves regular follow-up care to monitor for recurrence. Regular imaging and consultations with healthcare providers are essential to detect and treat any recurrence early. […] 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. […] 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.

• #57 Myxofibrosarcoma – Overview of Information and Clinical Research

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

  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. […] 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. […] Living with MFS involves regular follow-up care to monitor for recurrence. Regular imaging and consultations with healthcare providers are essential to detect and treat any recurrence early. […] 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. […] 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.

• #58 Myxofibrosarcoma – Overview of Information and Clinical Research

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

  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. […] 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. […] Living with MFS involves regular follow-up care to monitor for recurrence. Regular imaging and consultations with healthcare providers are essential to detect and treat any recurrence early. […] 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. […] 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.

• #59 Myxofibrosarcoma of the scalp with difficult preoperative diagnosis: A case report and review of the literature

  https://www.wjgnet.com/2307-8960/full/v8/i11/2350.htm

  Myxofibrosarcoma (MFS) is a malignant fibroblastic tumor that tends to occur in the lower and upper extremities. The reported incidence of head and neck MFSs is extremely rare. […] The treatment plan is closely associated with the anatomic location and histologic grade, and more importantly, aggressive surgery and adjuvant radiotherapy may be helpful. […] Given the special anatomic location and the high malignant potential of this rare tumor, combined surgical and adjuvant radiotherapy should be considered to avoid local recurrence and distant metastasis. The significance of regular follow-up is strongly recommended to improve the long-term survival rate. […] The treatment for MFS is a matter of international discussion. In our case, aggressive surgery and adjuvant radiotherapy was effective.

• #60 What Is Myxofibrosarcoma? – Klarity Health Library

  https://my.klarity.health/what-is-myxofibrosarcoma/

  Given its aggressive behaviour, timely diagnosis, treatment, and ongoing monitoring are vital in effectively managing myxofibrosarcoma. […] Treatment for myxofibrosarcoma involves several considerations, including tumour grade, stage, size, and depth. […] In many cases, surgical removal of the tumour along with a margin of surrounding tissue is the primary treatment. […] The treatment plan will be tailored to your situation, aiming to effectively address the cancer while minimising potential side effects. Consulting with a healthcare provider experienced in treating myxofibrosarcoma is crucial for making informed decisions about your treatment journey. […] Myxofibrosarcoma displays a higher tendency to recur post-treatment compared to other variants of soft tissue sarcomas. It’s estimated that up to 50% of individuals with myxofibrosarcoma experience a recurrence within five years of treatment. […] Regular consultations with your healthcare provider heighten the possibility of timely detection and intervention if myxofibrosarcoma reoccurs.

• #61 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. […] 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. […] Myxofibrosarcoma is more likely to come back after treatment than other types of soft tissue sarcomas. Up to 1 in 2 people have myxofibrosarcoma return within five years of treatment. […] 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.

• #62 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 margin resection is challenging because of microscopic infiltration. […] 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. A surgical margin of 2 mm was not sufficient to significantly influence the incidence of recurrence. Consequently, a wider surgical margin may be necessary to reduce the risk of myxofibrosarcoma recurrence. […] 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). […] The recurrence-free survival rate of patients with clear margin resection (60.2%) was higher compared with patients with microscopic infiltration (31.1%) at 5 years after the initial surgical procedure. The effect of radiotherapy is still controversial.

• #63

  https://www.orthobullets.com/evidence/31662702

  Myxofibrosarcoma (MFS) is one of the more common types of soft-tissue sarcoma (STS) in patients over 60 years of age. Local recurrence (LR) rates have been reported to be higher compared to other STS types. […] The rate of LR for patients with myxofibrosarcoma was high. The impact of RT on local tumor control was unclear. The surgical margin was important for both local and distant tumor control. Large tumor size was a risk factor for distant metastasis.

• #64 Myxofibrosarcoma – Overview of Information and Clinical Research

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

  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. […] 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. […] Living with MFS involves regular follow-up care to monitor for recurrence. Regular imaging and consultations with healthcare providers are essential to detect and treat any recurrence early. […] 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. […] 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.

• #65 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. […] In addition, CD44 may be used to assess the metastatic risk of patients with MFS. […] A positive surgical margin is an important cause of recurrence and metastasis in patients with MFS of the trunk and extremities. 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.

• #66 Myxofibrosarcoma — Cancer Survivors Network

  https://csn.cancer.org/discussion/191765/myxofibrosarcoma

  My husband was diagnosed with myxofibrosarcoma on 4/2010. At that time he had the tumor removed surgically and did well for a year. Then the cancer recurred. It is now in his groin area and he is undergoing chemo at Fox Chase Cancer Hospital in Philadelphia. They are talking amputating the leg and hip area if the chemo does not work. I have a lot of faith in the doctors at Fox Chase and whatever the outcome I know it will be a good one. […] My sarcoma was on my upper thigh and last year I had two operations and 34 radiation treatments. Because myxofibrosarcoma is so rare, it’s hard to find a lot of information on it. Occasionally there are clinical trials that are available. I would consider this option before amputation. […] I have been diagnosed with Myxofibrosarcoma after two wrong diagnosis. I have had the tumor removed thinking it was a lipoma. I have not started treatment yet. I wanted to know if anyone has had chemo with this type of cancer?

• #67 Myxofibrosarcoma — Cancer Survivors Network

  https://csn.cancer.org/discussion/191765/myxofibrosarcoma

  My husband had 25 days of radiation therapy, followed by a radical resection of the tumor site with plastic surgery in August. So far, CT scans of his chest are clear and there are no other signs of metastasis. […] They first did 6 weeks of radiation then surgery. It came back in 3/2017 and they had to amputate his left leg right below the knee. Now its come back again but in his lungs (8/2018). They said he had 20+ to many to count tumors, they are all over in his lungs so they can’t remove them so the Drs. suggested we should try chemo but the Dr.s told us it is incurable and they are just trying to slow it down to give him a little more time. […] I had a surgery for a myxofibrosarcoma grade 3 (fnlcc) 8×5 in my hip last month. I refused chimio/radio for the moment because I am convinced I am definitely healed. Let’s see what time says…

• #68 Myxofibrosarcoma — Cancer Survivors Network

  https://csn.cancer.org/discussion/191765/myxofibrosarcoma

  My husband was diagnosed with myxofibrosarcoma on 4/2010. At that time he had the tumor removed surgically and did well for a year. Then the cancer recurred. It is now in his groin area and he is undergoing chemo at Fox Chase Cancer Hospital in Philadelphia. They are talking amputating the leg and hip area if the chemo does not work. I have a lot of faith in the doctors at Fox Chase and whatever the outcome I know it will be a good one. […] My sarcoma was on my upper thigh and last year I had two operations and 34 radiation treatments. Because myxofibrosarcoma is so rare, it’s hard to find a lot of information on it. Occasionally there are clinical trials that are available. I would consider this option before amputation. […] I have been diagnosed with Myxofibrosarcoma after two wrong diagnosis. I have had the tumor removed thinking it was a lipoma. I have not started treatment yet. I wanted to know if anyone has had chemo with this type of cancer?

• #69 Myxofibrosarcoma — Cancer Survivors Network

  https://csn.cancer.org/discussion/191765/myxofibrosarcoma

  My husband had 25 days of radiation therapy, followed by a radical resection of the tumor site with plastic surgery in August. So far, CT scans of his chest are clear and there are no other signs of metastasis. […] They first did 6 weeks of radiation then surgery. It came back in 3/2017 and they had to amputate his left leg right below the knee. Now its come back again but in his lungs (8/2018). They said he had 20+ to many to count tumors, they are all over in his lungs so they can’t remove them so the Drs. suggested we should try chemo but the Dr.s told us it is incurable and they are just trying to slow it down to give him a little more time. […] I had a surgery for a myxofibrosarcoma grade 3 (fnlcc) 8×5 in my hip last month. I refused chimio/radio for the moment because I am convinced I am definitely healed. Let’s see what time says…

• #70 Myxofibrosarcoma — Cancer Survivors Network

  https://csn.cancer.org/discussion/191765/myxofibrosarcoma

  My husband had 25 days of radiation therapy, followed by a radical resection of the tumor site with plastic surgery in August. So far, CT scans of his chest are clear and there are no other signs of metastasis. […] They first did 6 weeks of radiation then surgery. It came back in 3/2017 and they had to amputate his left leg right below the knee. Now its come back again but in his lungs (8/2018). They said he had 20+ to many to count tumors, they are all over in his lungs so they can’t remove them so the Drs. suggested we should try chemo but the Dr.s told us it is incurable and they are just trying to slow it down to give him a little more time. […] I had a surgery for a myxofibrosarcoma grade 3 (fnlcc) 8×5 in my hip last month. I refused chimio/radio for the moment because I am convinced I am definitely healed. Let’s see what time says…

• #71 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #72 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #73 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. […] For patients with a limb tumor that cannot be resected (less than 5% of all cases), limb amputation is the treatment of choice. […] 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.

• #74 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #75 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #76 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. […] For patients with a limb tumor that cannot be resected (less than 5% of all cases), limb amputation is the treatment of choice. […] 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.

• #77 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. Patients affected by MFS should be managed in specialized multidisciplinary teams. Treatment options include surgery, radiotherapy (RT), and chemotherapy (CT). In the localized disease the gold standard is represented by radical surgery with the goal of achieving a complete resection of the lesion which means obtaining free margins from tumor infiltration. RT and CT could be considered as (neo)-adjuvant treatments and their impact is still debated. 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. However, despite treatments, local recurrence rates are between 50% and 60%. The most sensitive and accurate imaging tool to assess margins in STS is represented by MRI, with the most important feature on baseline MRI being an infiltrative growth pattern. The beneficial effect of RT on local tumor control for MFS is still unclear. Indeed, several retrospective studies reported that RT in combination with surgery is associated with a lower risk of LR in retroperitoneal STSs, while other trials did not observe significant benefit for adjuvant RT. Even though administration of chemotherapy represents the standard clinical care in metastatic MFS, it is mainly used as palliative and outcome remains very poor. Indeed, no large randomized clinical trials clarifying the beneficial effect of CT in this kind of tumor have been conducted. However, data available from very few cohort studies conducted over a decade ago suggested that overall survival is not significantly increased after chemotherapy nor it is effective against distant metastases. Currently, targeted therapies for sarcomas aim at inhibiting cell cycle progression, sustained proliferative signaling, DNA repair, epigenetics, tumor microenvironment and angiogenesis. 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.

• #78 Myxofibrosarcoma — Cancer Survivors Network

  https://csn.cancer.org/discussion/191765/myxofibrosarcoma

  My husband was diagnosed with myxofibrosarcoma on 4/2010. At that time he had the tumor removed surgically and did well for a year. Then the cancer recurred. It is now in his groin area and he is undergoing chemo at Fox Chase Cancer Hospital in Philadelphia. They are talking amputating the leg and hip area if the chemo does not work. I have a lot of faith in the doctors at Fox Chase and whatever the outcome I know it will be a good one. […] My sarcoma was on my upper thigh and last year I had two operations and 34 radiation treatments. Because myxofibrosarcoma is so rare, it’s hard to find a lot of information on it. Occasionally there are clinical trials that are available. I would consider this option before amputation. […] I have been diagnosed with Myxofibrosarcoma after two wrong diagnosis. I have had the tumor removed thinking it was a lipoma. I have not started treatment yet. I wanted to know if anyone has had chemo with this type of cancer?

• #79 Myxofibrosarcoma Clinical Trial Finding Service | RareCan

  https://rarecan.com/about/rare-cancer-list/myxofibrosarcoma/

  For a much more detailed guide, please have a look at our myxofibrosarcoma information pack. […] MFS can return aggressively after surgery, so surgery often takes a wide margin of tissue and is combined with radiotherapy. […] RareCan is working to accelerate research into myxofibrosarcoma 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 myxofibrosarcoma diagnosis. We will then get in touch with you about any research opportunities that might be suitable for you. […] If you are looking for further support with myxofibrosarcoma we recommend: […] Sarcoma UK is a charity for those with bone and soft tissue sarcoma diagnoses. They provide information on all types of sarcomas and have a patient support line to help assist with any queries and worries.

• #80 Myxofibrosarcoma Clinical Trial Finding Service | RareCan

  https://rarecan.com/about/rare-cancer-list/myxofibrosarcoma/

  For a much more detailed guide, please have a look at our myxofibrosarcoma information pack. […] MFS can return aggressively after surgery, so surgery often takes a wide margin of tissue and is combined with radiotherapy. […] RareCan is working to accelerate research into myxofibrosarcoma 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 myxofibrosarcoma diagnosis. We will then get in touch with you about any research opportunities that might be suitable for you. […] If you are looking for further support with myxofibrosarcoma we recommend: […] Sarcoma UK is a charity for those with bone and soft tissue sarcoma diagnoses. They provide information on all types of sarcomas and have a patient support line to help assist with any queries and worries.

• #81 Myxofibrosarcoma Clinical Trial Finding Service | RareCan

  https://rarecan.com/about/rare-cancer-list/myxofibrosarcoma/

  For a much more detailed guide, please have a look at our myxofibrosarcoma information pack. […] MFS can return aggressively after surgery, so surgery often takes a wide margin of tissue and is combined with radiotherapy. […] RareCan is working to accelerate research into myxofibrosarcoma 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 myxofibrosarcoma diagnosis. We will then get in touch with you about any research opportunities that might be suitable for you. […] If you are looking for further support with myxofibrosarcoma we recommend: […] Sarcoma UK is a charity for those with bone and soft tissue sarcoma diagnoses. They provide information on all types of sarcomas and have a patient support line to help assist with any queries and worries.

• #82 Myxofibrosarcoma // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/myxofibrosarcoma

  Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you. If your healthcare professional thinks that you might have myxofibrosarcoma, you may be referred to a specialist. Specialists who care for people with myxofibrosarcoma include: Cancer doctors, called medical oncologists. […] Appointments can be short and being prepared can help. Here’s some information that may help you get ready. […] Take a family member or friend along, if possible, to help you remember the information you’re given.

• #83 Myxofibrosarcoma Clinical Trial Finding Service | RareCan

  https://rarecan.com/about/rare-cancer-list/myxofibrosarcoma/

  For a much more detailed guide, please have a look at our myxofibrosarcoma information pack. […] MFS can return aggressively after surgery, so surgery often takes a wide margin of tissue and is combined with radiotherapy. […] RareCan is working to accelerate research into myxofibrosarcoma 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 myxofibrosarcoma diagnosis. We will then get in touch with you about any research opportunities that might be suitable for you. […] If you are looking for further support with myxofibrosarcoma we recommend: […] Sarcoma UK is a charity for those with bone and soft tissue sarcoma diagnoses. They provide information on all types of sarcomas and have a patient support line to help assist with any queries and worries.

• #84 Myxofibrosarcoma Clinical Trial Finding Service | RareCan

  https://rarecan.com/about/rare-cancer-list/myxofibrosarcoma/

  For a much more detailed guide, please have a look at our myxofibrosarcoma information pack. […] MFS can return aggressively after surgery, so surgery often takes a wide margin of tissue and is combined with radiotherapy. […] RareCan is working to accelerate research into myxofibrosarcoma 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 myxofibrosarcoma diagnosis. We will then get in touch with you about any research opportunities that might be suitable for you. […] If you are looking for further support with myxofibrosarcoma we recommend: […] Sarcoma UK is a charity for those with bone and soft tissue sarcoma diagnoses. They provide information on all types of sarcomas and have a patient support line to help assist with any queries and worries.

• #85 Myxofibrosarcoma // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/myxofibrosarcoma

  Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you. If your healthcare professional thinks that you might have myxofibrosarcoma, you may be referred to a specialist. Specialists who care for people with myxofibrosarcoma include: Cancer doctors, called medical oncologists. […] Appointments can be short and being prepared can help. Here’s some information that may help you get ready. […] Take a family member or friend along, if possible, to help you remember the information you’re given.

• #86 Myxofibrosarcoma // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/myxofibrosarcoma

  Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you. If your healthcare professional thinks that you might have myxofibrosarcoma, you may be referred to a specialist. Specialists who care for people with myxofibrosarcoma include: Cancer doctors, called medical oncologists. […] Appointments can be short and being prepared can help. Here’s some information that may help you get ready. […] Take a family member or friend along, if possible, to help you remember the information you’re given.

• #87 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. […] Despite these medical interventions, Joel remains physically strong, continuing his workouts and daily routine without pain. […] He firmly believes in leveraging crises to bring awareness, comfort, and inspiration to those in need, rather than letting them go to waste.

• #88 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. […] Despite these medical interventions, Joel remains physically strong, continuing his workouts and daily routine without pain. […] He firmly believes in leveraging crises to bring awareness, comfort, and inspiration to those in need, rather than letting them go to waste.

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