DESMOIDS IN FAMILIAL ADENOMATOUS POLYPOSIS

Clinical desmoid disease affects approximately 10 per cent of patients with familial adenomatous polyposis (FAP); the subclinical rate is un known. Desmoids ar probably neoplastic rather than regenerative in ori gin 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 cellula rity, which in part determines progression, and this may help manageme nt. Surgical treatment of advanced desmoids is hazardous, but medical treatments have limited success. Chemotherapy with doxorubicin and dac arbazine is currently under evaluation.