National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Desmoid Tumor is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
- Musculoaponeurotic fibromatosis
- Aggressive fibromatosis
- Deep fibromatosis
- Nonmetastasizing fibrosarcoma
- Grade I fibrosarcoma
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 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.
Desmoid tumor is also called as 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.
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). It can adhere to and intertwine with surrounding structures and organs. The myofibroblast is the cell considered to be responsible for the development of Desmoid tumor and usually very difficult to remove because they intertwine extensively with the surrounding tissues. These tumors look like dense scar tissue and just like scar tissue, they adhere tenaciously to surrounding structures and organs.
Surgery has been the traditional main mode of therapy for Desmoid tumors but up to 70% of these tumors recur after surgery.
American Cancer Society, Inc.
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Desmoid Tumor Research Foundation
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Suffern, NY 10901
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This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html