Desmoid tumor

From Wikipedia, the free encyclopedia

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.

Current experimental studies are being done with Gleevec for treatment of Desmoid tumors, and show promising success rates.