Desmoid tumor

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Aggressive fibromatosis

Overview

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).

Classification

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.

Pathophysiology

Microscopic Pathology

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

Treatment

Medical Therapy

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

External links

• Desmoid Tumor Research Foundation