Desmoids in familial adenomatous polyposis
Article first published online: 7 DEC 2005
Copyright © 1996 British Journal of Surgery Society Ltd.
British Journal of Surgery
Volume 83, Issue 11, pages 1494–1504, November 1996
How to Cite
Clark, S. K. and Phillips, R. K. S. (1996), Desmoids in familial adenomatous polyposis. Br J Surg, 83: 1494–1504. doi: 10.1002/bjs.1800831105
- Issue published online: 7 DEC 2005
- Article first published online: 7 DEC 2005
- Manuscript Accepted: 12 JUN 1996
Clinical desmoid disease affects approximately 10 per cent of patients with familial adenomatous polyposis (FAP); the subclinical rate is unknown. Desmoids are probably neoplastic rather than regenerative in origin and may arise in association with germline or somatic mutations at or beyond codon 1444 of the APC gene. Intra-abdominal desmoids behave unpredictably but are an important cause of death in those with FAP. Signal intensity on magnetic resonance imaging reflects tumour cellularity, which in part determines progression, and this may help management. Surgical treatment of advanced desmoids is hazardous, but medical treatments have limited success. Chemotherapy with doxorubicin and dacarbazine is currently under evaluation.