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Genetic and Rare Diseases Information Center (GARD)

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Epithelioid sarcoma

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Your Question

What is epithelioid sarcoma?  Why is it difficult to diagnose?  What is the average length of time between the beginning of symptoms and diagnosis?  How rare is it?  

Our Answer

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What is epithelioid sarcoma?

Epithelioid sarcoma is a rare cancer that most often occurs in the soft tissue of the fingers, hands and forearms of young adults.[1]  It may also be found in the legs, trunk, head or neck regions.  It is rare in young children and adults, and it occurs more frequently in men.[2][3]  Epithelioid sarcoma begins as a painless, firm growth or bump that may be accompanied by an open wound (ulceration) in the skin covering the growth.[3]  It is considered an aggressive cancer because it has a high chance of regrowing after treatment (a recurrence), or spreading to surrounding tissues or more distant parts of the body (a metastasis).[3]  Epithelioid sarcoma is first treated with surgery to remove all the cancer cells (wide local excision).[2][3]  Amputation of part of the affected limb may be needed in severe cases.[1]  Radiation therapy or chemotherapy may also be used to destroy any cancer cells not removed during surgery.[2]
Last updated: 9/16/2011

Why is epithelioid sarcoma difficult to diagnosis? What is the average length of time between the beginning of symptoms and diagnosis?

Epithelioid sarcoma is difficult to diagnosis because it looks, both physically and under the microscope, similiar to many other benign and malignant conditions.[3]  Due to its ambiguous appearance, there may be a delay in establishing an exact diagnosis.[3]  Two studies which examined the length of time between the beginning of symptoms and diagnosis found that it took 29 months and 18 months, respectively, to establish a diagnosis of epithelioid sarcoma.[4][5]
Last updated: 9/16/2011

How rare is epithelioid sarcoma?

Epithelioid sarcoma is quite rare, counting for less than 1% of all soft tissue sarcomas.[3]  In the United States, only 229 individuals were diagnosed with this condition from 2004 to 2008.[6]
Last updated: 9/16/2011