Important
It is possible that the main title of the report Desmoid Tumoris not the name you expected.
Synonyms
- Musculoaponeurotic fibromatosis
- Aggressive fibromatosis
- Deep fibromatosis
- Nonmetastasizing fibrosarcoma
- Grade I fibrosarcoma
General Discussion
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.
Resources
American Cancer Society, Inc.
1599 Clifton Road NE
Atlanta, GA 30329
USA
Tel: 4043203333
Tel: 8002272345
Internet: http://www.cancer.org
Desmoid Tumor Research Foundation
P.O. Box 273
Suffern, NY 10901
Tel: (914)262-6595
Fax: (845)369-8302
Email: info@dtrf.org
Internet: http://www.dtrf.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders, Inc. (r) (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
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org
Last Updated: 3/9/2008
Copyright 2008National Organization for Rare Disorders, Inc.
