Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Desmoid Tumor

Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_1578-2

Synonyms

Definition

Desmoid (meaning tendon-like) tumors are a heterogeneous group of rare connective tissue neoplasms, which can occur at almost any anatomical location.

Desmoids have been classified as fibromatoses, along with pathologies such as palmar fasciitis, which are due to proliferation of well-differentiated fibroblasts and are locally infiltrative and tend to recur after excision but do not metastasize.

Characteristics

Desmoids are rare, accounting for less than 0.1 % of all tumors, and have an annual incidence of two to four per million. While most occur sporadically, 2 % are associated with familial adenomatous polyposis (FAP), an autosomal dominantly inherited cancer predisposition syndrome due to mutation of the tumor suppressor gene APC (APC gene in familial adenomatous polyposis). Desmoids are over 1,000 times more common in individuals with FAP than in the population in general, occurring in about...

Keywords

Familial Adenomatous Polyposis Desmoid Tumor Ureteric Obstruction Aggressive Fibromatosis Small Bowel Mesentery 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. Clark SK, Phillips RKS (1996) Desmoids in familial adenomatous polyposis. Br J Surg 83:1494–1504CrossRefPubMedGoogle Scholar
  2. Hosalkar HS, Fox EJ, Delaney T et al (2006) Desmoid tumours and current status of management. Orthop Clin North Am 37:53–63CrossRefPubMedGoogle Scholar
  3. Okuno S (2006) The enigma of desmoid tumours. Curr Treat Options Oncol 7:438–443CrossRefPubMedGoogle Scholar
  4. Reitamo JJ, Scheinin TM, Hayry P (1986) The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumour. Am J Surg 151:230–237CrossRefPubMedGoogle Scholar
  5. Sturt NJH, Clark SK (2006) Current ideas in desmoid tumours. Fam Cancer 5:275–285CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Imperial College LondonLondonUK