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Clinical Problem Solving: Pathology
May 1, 2008

Pathology Quiz Case: Diagnosis

Author Affiliations

Copyright 2008 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2008

Arch Otolaryngol Head Neck Surg. 2008;134(5):560-561. doi:10.1001/archotol.134.5.560

Fibromatosis was first described in 1832.1It is a rare, cytologically bland, monoclonal fibroblastic proliferation that arises within the musculoaponeurotic structures throughout the body.1-4The lesion is frequently referred to as a desmoid tumorbecause of its tendonlike appearance.4,5Several other terms have been used to describe this disorder, including aggressive fibromatosis, desmoplastic fibroma, desmoma, well-differentiated nonmetastasizing fibrosarcoma, and grade I fibrosarcoma.5There may be a genetic component in the pathogenesis of fibromatosis, as demonstrated by a cumulative lifetime risk of 20.6% in patients with familial adenomatous polyposis.1Other factors, such as trauma and endocrine, also seem to be associated with the development of fibromatosis, but the exact cause is still unknown.1,4,5