Name: Desmoid Tumors

Description: Aggressive Fibromatosis

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A desmoid tumor is the abnormal growth of cells in connective tissues. Connective tissue helps to maintain the structure of the body and include the tissues covering muscle (fascia), cartilage and fat. 

Though these tumors have a tendency to invade surrounding tissues and organs (be aggressive), they rarely spread to more distant parts of the body (metastasize).

Desmoid tumors occur in 2-5 people per million and are most often found in the muscles of the shoulder or abdominal wall, though they can occur in other parts of the body as well.

The first signs that a desmoid tumor is growing include a growing mass or lump; if the mass presses on other parts of the body, it could cause moderate pain, numbness, tingling, or limit the movement of limbs. 

A desmoid tumor is usually diagnosed by magnetic resonance imaging (MRI) followed by a biopsy.  Treatment consists of surgery to remove as much of the tumor as possible; radiation therapy, chemotherapy, or hormone therapy may also be used to reduce the chance that the tumor might regrow following surgery.

The cause of desmoid tumors is not completely clear;  however, approximately 5% of these tumors are associated with an inherited colon cancer syndrome known as familial adenomatous polyposis (FAP).
Genetic and Rare Diseases

Bookmarks The following information, which has been distilled by the casebook author from this and other websites is particularly relevant to this casebook.
Bookmarks - Web
Web Page Notes Concepts
 Extra-Abdominal Desmoid Tumors - OrthoInfo - AAOS American Academy of Orthopedic Surgeons
 About Desmoid Tumors Desmoid Tumor Research Foundation
 Aggressive fibromatosis - Wikipedia, the free encyclopedia Wikipedia
 Desmoid Tumors — Diagnosis and Treatment Options at Mayo Clinic Mayo Clinic

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