A desmoid tumor (sometimes referred to as aggressive fibromatosis) is a rare (3 per million population) tumor that may or may not be part of a genetic syndrome such as familial adenomatous polyposis (FAP).

Histologically they resemble low-grade fibrosarcomas, but they are very infiltrative and tend to recur even after complete resection (systemic metastasis is rare).

 

Desmoid tumors may be classified as extra-abdominal, abdominal wall, or intra-abdominal (the last is more common in patients with FAP). It is thought that the lesions may develop in relation to estrogen levels or trauma/operations.

 

Treatment may consist of watching and waiting, complete surgical removal, radiation therapy, antiestrogens and NSAIDs, or chemotherapy.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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