What are the other Names for this Condition? (Also known as/Synonyms)

• Atypical Myxoinflammatory Fibroblastic Tumor
• Inflammatory Myxohyaline Tumor of the Distal Extremities with Virocyte or Reed-Sternberg-Like Cells
• Inflammatory Myxoid Tumor of the Soft Parts with Bizarre Giant Cells

What is Myxoinflammatory Fibroblastic Sarcoma? (Definition/Background Information)

• Myxoinflammatory Fibroblastic Sarcoma (MIFS) is a rare, malignant, soft tissue tumor that is found to be locally aggressive
• In a majority of cases, Myxoinflammatory Fibroblastic Sarcoma is found in middle-aged adults. MIFS is known to grow very slowly over several years that it is often mistakenly considered as a benign tumor
• Generally, the tumor involves the hands and feet (mostly the fingers). The joints, muscles, and bones may also be affected resulting in associated signs and symptoms
• The treatment of choice for Myxoinflammatory Fibroblastic Sarcoma is a complete surgical excision. However, tumor recurrence is commonly noted
• The prognosis of Myxoinflammatory Fibroblastic Sarcoma depends upon several factors including the size and stage of the tumor. Small-sized tumors that are detected early and can be completely removed have a good prognosis

Who gets Myxoinflammatory Fibroblastic Sarcoma? (Age and Sex Distribution)

• Myxoinflammatory Fibroblastic Sarcoma commonly affects middle-aged adults (40-50 year range; average age 48 years). However, adults of a wide age range may be affected
• Infants, young and old children are usually not affected
• Both males and females are affected at the same incidence rate
• There is no known ethnic or racial preference

What are the Risk Factors for Myxoinflammatory Fibroblastic Sarcoma? (Predisposing Factors)

Currently, the specific risk factors for Myxoinflammatory Fibroblastic Sarcoma are yet to be identified.

• In rare cases, a history of trauma has been implicated
• Immunocompromised state due to renal transplantation may be a risk factor, according to some studies

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Myxoinflammatory Fibroblastic Sarcoma? (Etiology)

• The exact cause and mechanism of formation of Myxoinflammatory Fibroblastic Sarcoma is unknown
• Some reports indicate that they may occur due to genetic defects, as certain chromosomal aberrations have been noted

What are the Signs and Symptoms of Myxoinflammatory Fibroblastic Sarcoma?

The signs and symptoms of Myxoinflammatory Fibroblastic Sarcoma include:

• Presence of a poorly-defined tumor that may be multinodular in appearance
• MIFS is a tumor that may grow quickly (sometimes) to very slowly. It may grow within 6 weeks or even take up to 20 years, due to which it may be mistaken for a benign lesion
• The tumor is normally less than 5 cm in size, though it can grow much larger. The size can vary from 1-20 cm
• The malignancy is mainly observed in the hands and feet (including fingers and toes); 80% are seen in the hands (fingers) or upper extremities. Involvement of the upper parts of the arms/legs are less common than involvement of the lower parts
• The skin overlying the tumor can ulcerate and bleed
• Most involve the fluid-filled sheaths around the tendons (tenosynovial involvement) and the muscles and joints; rarely, the tumors may even involve the bone
• Other locations of the tumor includes the trunk, back, and neck regions

How is Myxoinflammatory Fibroblastic Sarcoma Diagnosed?

A diagnosis of Myxoinflammatory Fibroblastic Sarcoma may involve the following tests and exams:

• Complete physical examination and evaluation of medical history
• X-ray of the affected hand or leg or other body part
• MRI or CT scan of the affected region to aid in obtaining a clear image of the tumor, prior to surgery
• Tissue biopsy of tumor specimen: A tumor biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Myxoinflammatory Fibroblastic Sarcoma?

The complications of Myxoinflammatory Fibroblastic Sarcoma (MIFS) may include:

• Secondary infections due to skin ulceration
• MIFS can be locally-aggressive. If the bone and joint is affected, it can lead to associated signs and symptoms including bone pain and restricted joint movement
• 1 in 30 cases are known to metastasize, mostly to the regional lymph nodes. Metastasis to other distant sites is very rare, but the sites may include the lungs, liver, and inguinal lymph nodes
• The tumor recurrence rate is 20-70% which occurs mostly due to a partial removal of the tumor
• Multiple recurrences have been noted in some cases. In case of multiple recurrences, amputation of the limb part may be necessary (observed in about one-third of the cases)
• Damage of vital nerves, blood vessels, and surrounding structures, during surgery
• Side effects from chemotherapy (toxicity) and/or radiation therapy

How is Myxoinflammatory Fibroblastic Sarcoma Treated?

The treatment of Myxoinflammatory Fibroblastic Sarcoma (MIFS) is undertaken as:

• Wide surgical excision of MIFS with removal of the entire lesion is generally adopted. However, often a complete excision is difficult due to infiltrative growth and involvement of surrounding tissue structures
• In rare cases, amputation of the portion of the affected limb may be necessary, if the bone is severely involved
• Embolization (clotting the vessels in the tumor) may be used to provide temporary relief from the symptoms and reduce blood loss during a surgical procedure
• Radiation therapy and/or chemotherapy may be used to destroy the remaining tumor cells, based on the assessment by the healthcare provider
• Post-operative care is important until the surgical wound heals
• Follow-up care with regular screening and check-ups are important; a long-term follow-up is recommended

How can Myxoinflammatory Fibroblastic Sarcoma be Prevented?

• Current medical research have not established a method of preventing Myxoinflammatory Fibroblastic Sarcoma
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory for those who have been diagnosed with and treated for the tumor, due to its metastasizing potential and possibility of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Myxoinflammatory Fibroblastic Sarcoma? (Outcomes/Resolutions)

• Myxoinflammatory Fibroblastic Sarcoma (MIFS) is a malignant tumor that can display local aggression. The prognosis depends on a combination of factors including:
  • Age of the individual
  • Tumor stage at detection
  • Tumor size and location
  • Individual’s response to therapy
• Tumors that are small in size and present at easily accessible locations, showing no bone involvement, have a good prognosis on their surgical excision and removal
• Late detection of tumors may present with severe bone involvement and even metastasis (in rare cases), which can adversely affect the prognosis
• MIFS may form and develop slowly over decades, thereby mimicking a benign condition. This can lead to a delay in diagnosis

Additional and Relevant Useful Information for Myxoinflammatory Fibroblastic Sarcoma:

Please visit our Cancer & Benign Tumor Health Center for more physician-approved health information:

http://www.dovemed.com/diseases-conditions/cancer/