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Aggressive Fibromatosis (Desmoid Tumor)

Summary

Aggressive fibromatosis (AF)(desmoid tumor) is a rare tumor characterized by a monoclonal proliferation of fibroblasts in muscles, tendons, and ligaments. It is histologically benign, but may be locally aggressive, invasive, and destructive. The tumor does not metastasize but recurs frequently following treatment. The clinical presentation, anatomic location, and biologic behavior of this tumor are variable and an individualized treatment approach is required.

Complete Information on this Tumor
Incidence and Demographics: 
Aggressive fibromatosis may be associated with familial adenomatous polyposis (FAP), an autosomal dominant disease which causes intestinal polyposis and colorectal cancer. Gardner's syndrome consists of the non-intestinal manifestations of the disease, such as osteomas, skin cysts, retinal disorders, and AF, that are commmon in families with FAP
Differential Diagnosis: 
Optimal management of AF depends on tumor location and extent. Wide surgical excision remains the treatment of choice except when surgery is mutilating and is associated with considerable function or loss. Postoperative radiotherapy results in a significant reduction of the local recurrence rate, only in the case of involved surgical margins. Radiotherapy for gross disease is considerably effective, but is associated with relatively high rate of complications. Data indicate the likelihood of local recurrence after surgery is high, particularly if margins are positive. Attempts to complete eradication of the disease may be worse than the disease itself.
Suggested Reading and Reference: 
Aggressive fibromatosis. Mendenhall WM, Ziotecki RA, Morris CG, Hochwalt SN, Scarborough MT. Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, Florida, USA. Desmoid tumors: need for an individualized approach. de Bree E, Keus R, Melissas J, Tsiftsis D, van Coevorden F. Department of Surgical Oncology, Medical School of Crete University Hospital.