The management of desmoids in patients with familial adenomatous polyposis (FAP).
Desmoids are rare in the general population but occurs in between 10 to 20% of patients with familial adenomatous polyposis (FAP). This risk is about 852 times the risk for the population at large. Desmoids are benign neoplasms that are capable of infiltrating locally with a high risk of recurrence (25-65%) even after extirpating surgery. Desmoids in FAP may occur extra-abdominally, or within the abdominal wall or most commonly intra-abdominally within the mesentery or retroperitoneal. Desmoids are a major problem in patients with FAP. Mortality from desmoids is high in such patients and ranges from 18 to 31%, compared to peri-ampullary carcinomas at about 22% and cancer in the retained rectum at only about 8%. Simple drug treatment with tamoxifen or NSAIDS like sulindac should be used as first line treatment as it carries a response in 30-50% of patients. Surgery should be reserved for extra-abdominal tumours alone and only when needed. Surgery for intra-abdominal desmoids should really only be attempted for intestinal obstruction or ureteric obstruction. Dacarbazine-Doxorubicin chemotherapy may have dramatic response in some cases. Genetic transfer may unlock this disease in future and may give patients with FAP and severe desmoids hope for the future.
Seow-Choen
F
F
Seow-Choen Colorectal Centre Pte Ltd, Singapore.
D011125:Adenomatous Polyposis Coli / Q000150:complications*; D018222:Desmoid Tumors / Q000150:complications / Q000235:genetics / Q000628:therapy*; D006801:Humans