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Desmoid Tumor

Abstract

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NORD is very grateful to Charisse D. Litchman, MD, Co-founder, The Desmoid Tumor Research Foundation, for assistance in the preparation of this report.

Synonyms of Desmoid Tumor

  • aggressive fibromatosis
  • deep fibromatosis
  • grade I fibrosarcoma
  • musculoaponeurotic fibromatosis
  • nonmetastasizing fibrosarcoma

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Summary
Desmoid tumor commonly develops in the fibrous (connective) tissue of the body that forms tendons and ligaments, usually in the arms, legs or midsection, and also in the head and neck. These tissues of the body connect, support, and surround other body parts and organs. The myofibroblast is the cell considered to be responsible for the development of desmoid tumor. Regardless of its scientific classification, a desmoid tumor can be invasive to surrounding tissues and difficult to control. Desmoid tumors can develop virtually at any body site. Superficial desmoids tend to be less aggressive than deep desmoids (abdominal, extra abdominal, mesenteric). These tumors look like dense scar tissue and just like scar tissue, they adhere tenaciously to surrounding structures and organs, and, thus they are commonly difficult to remove. Surgery has been the traditional main mode of therapy for desmoid tumors but up to 20-50% of these tumors recur after surgery.

Introduction
The Greek word "desmos" which means tendon or band like has been the origin of the term desmoid and was coined by Muller in 1838. Desmoid tumor is called an aggressive fibromatosis as it has similarities with a malignant (cancerous) tumor called fibrosarcoma. However, it is considered benign because it does not metastasize (spread) to other parts of the body.

Organizations related to Desmoid Tumor

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