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Genetic and Rare Diseases Information Center (GARD)

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Desmoid tumor

Other Names for this Disease
  • Aggressive fibromatosis
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Overview


A desmoid tumor is the abnormal growth of cells in connective tissues, which are tissues that help 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.[1]  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.[2]  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.[1]  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).[3]


References

  1. Biermann JS. Desmoid Tumors. Current Treatment Options in Oncology. 2000. http://www.ncbi.nlm.nih.gov/pubmed/12057169. Accessed OCtober 25, 2011.
  2. Hosalkar HS, Fox EJ, Delaney T, Torbert JT, Ogilvie CM, Lackman RD. Desmoid Tumors and Current Status of Management. The Orthopedic Clinics of North America. 2006. http://www.ncbi.nlm.nih.gov/pubmed/16311111. Accessed October 24, 2011.
  3. de Bree E, Keus R, Melissas J, Tsiftsis D, van Coevorden F. Desmoid tumors: need for an individualized approach. Expert Review of Anticancer Therapy. 2009. http://www.ncbi.nlm.nih.gov/pubmed/19374605. Accessed October 24, 2011.
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General Information

  • The Dana-Farber Cancer Institute Web site provides information on desmoid tumor. Click on the link above to view this information page.
  • The American Academy of Orthopaedic Surgeons provide information on extra-abdominal desmoid tumors. Click on the link above to access this page.
  • The Desmoid Tumor Research Foundation Web site offers information on desmoid tumors. Click on the link above to access this page.
  • Genetics Home Reference contains information on Desmoid tumor. Click on the link to go to GHR and review the information.
  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Desmoid tumor. Click on the link to view a sample search on this topic.