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

• SEF (Sclerosing Epithelioid Fibrosarcoma)

What is Sclerosing Epithelioid Fibrosarcoma? (Definition/Background Information)

• Sclerosing Epithelioid Fibrosarcoma (SEF) is a rare and malignant tumor of the soft tissues. It is typically seen in middle-aged and older adults and has a high potential for metastasis
• The tumor occurs deep in the body tissues and can invade muscles and bones. The common sites of the tumor includes the legs, trunk, and arms
• There are no specifically identified risk factors or cause for formation of Sclerosing Epithelioid Fibrosarcoma, but certain genetic aberrations have been observed in tumor studies
• The treatment of Sclerosing Epithelioid Fibrosarcoma may include a combination of surgery, chemotherapy and radiation therapy, based on the assessment by the healthcare provider
• However, despite appropriate therapy, the prognosis of Sclerosing Epithelioid Fibrosarcoma is poor. The prognosis is dependent upon a set of factors that includes the size, location, and stage of the tumor

Who gets Sclerosing Epithelioid Fibrosarcoma? (Age and Sex Distribution)

• Most cases of Sclerosing Epithelioid Fibrosarcoma are seen in middle-aged and older adults (average age of 47 years)
• It can be sometimes seen in older children and young adults. A wide age range of 14-87 years is observed
• Both males and females are affected
• All racial and ethnic groups may be affected

What are the Risk Factors for Sclerosing Epithelioid Fibrosarcoma? (Predisposing Factors)

• Currently, there are no specific risk factors associated with Sclerosing Epithelioid Fibrosarcoma

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 Sclerosing Epithelioid Fibrosarcoma? (Etiology)

The exact cause of Sclerosing Epithelioid Fibrosarcoma formation is not known.

• The present and available information on genetic research related to the tumor is insufficient to propose a cause for SEF
• The tumor analysis shows some genetic abnormality similar to Low-Grade Fibromyxoid Sarcoma (LGFS). Hence, it is suggested that SEF may be related to or a variant of LGFS

What are the Signs and Symptoms of Sclerosing Epithelioid Fibrosarcoma?

The signs and symptoms of Sclerosing Epithelioid Fibrosarcoma may include:

• Tumor mass may present variable growth; about 35% of the cases present pain and noticeable enlargement of tumor. It is noted that these two factors are prime reasons for seeking a healthcare advise/consult
• SEF is a well-defined mass that can be multinodular. It may involve the deep muscles and show bone attachment; in a few cases, the bone may also be eroded
• Tumor size is typically less than 10 cm; some grow to 20 cm or over
• Most frequent site is the lower limb girdle (40% cases), then chest and back (20%), followed by the arms, shoulders, and head and neck region
• Rarely, the abdomen (retroperitoneum) and pelvis, body organs, and bones are involved

How is Sclerosing Epithelioid Fibrosarcoma Diagnosed?

A diagnosis of Sclerosing Epithelioid Fibrosarcoma may involve the following tests and exams:

• A complete physical examination and evaluation of medical history
• X-ray studies of the affected region; areas of calcification may be seen
• Imaging studies, such as CT and MRI scans, of the affected region which can show the extent of the tumor and help in surgery
• Tissue biopsy of the tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination. A 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 diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis

Note: Sclerosing Epithelioid Fibrosarcoma can be difficult to diagnose, since it may show the presence of other tumor patterns (resembling other tumor types).

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 Sclerosing Epithelioid Fibrosarcoma?

The possible complications from Sclerosing Epithelioid Fibrosarcoma include:

• Emotional stress due to a concern of cancer
• Surgically excised tumors are known to return; the recurrence rate can be over 50%. Sometimes, multiple recurrences may be noted
• Low-grade tumors on recurrence may become high-grade fibrosarcomas
• The rate of metastasis is between 40% to 60%; the lungs and pleura are the main sites, followed by the bones, brain, heart, liver, scalp, chest wall and breast
• Damage to vital nerves, blood vessels, and surrounding structures during surgery to remove the tumor
• Side effects from chemotherapy and radiation therapy

How is Sclerosing Epithelioid Fibrosarcoma Treated?

Treatment measures for Sclerosing Epithelioid Fibrosarcoma may include the following:

• Surgical excision of SEF with removal of the entire tumor is generally adopted
• Embolization is 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 tumor cells, based on the assessment by the healthcare provider
• Post-operative care is important; a minimum activity level is to be ensured until the surgical wound heals
• Follow-up care with regular screening and check-ups are important

How can Sclerosing Epithelioid Fibrosarcoma be Prevented?

• Current medical research has not established a way of preventing Sclerosing Epithelioid Fibrosarcoma
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory due to its high metastasizing potential and chances of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Sclerosing Epithelioid Fibrosarcoma? (Outcomes/Resolutions)

• Sclerosing Epithelioid Fibrosarcoma is a rare malignancy, with metastasizing potential. The prognosis of the tumor depends upon many factors including the size of the tumor, stage of the tumor, tumor location, age and health status of the individual
• The prognosis of small-sized tumors with complete excision and removal is generally better than large-sized tumors that are detected later
• Studies indicate that the key prognostic indices are tumor size, location (tumors at head and neck and arms have worse prognosis), and metastasis
• Some reports indicate that males with the tumor have worse prognosis than females, while others inform that there are no gender-based differences in prognostic determinants

In general, the prognosis of Sclerosing Epithelioid Fibrosarcoma is poor, with survival rate of 65% over a period of 4 years.

Additional and Relevant Useful Information for Sclerosing Epithelioid Fibrosarcoma:

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

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