DESMOID TUMORS IN PATIENTS WITH FAMILIAL ADENOMATOUS POLYPOSIS

Background. Sporadic desmoid tumors occur mainly in the abdominal wall and in extraabdominal sites. Desmoid tumors in patients with familial adenomatous polyposis (FAP) usually occur in the abdominal wall and i n the bowel mesentery. Surgical resection of desmoids in patients with FAP has been controversial. Methods. A retrospective review of patien ts with FAP and desmoid tumors treated from 1950 to 1991 was performed . Patients were evaluated for gender, age, site of desmoid tumors, tre atment, recurrence, and survival. Results. Twenty-one of 24 patients u nderwent 60 surgical procedures related to the desmoid tumors. Seven o f nine patients who underwent potentially curative surgery had recurre nces; three were reresected. Major morbidity after palliative or curat ive surgery was 47%. Five patients were alive with no evidence of dise ase at a median of 198 months, 10 patients were alive with disease at a median of 102 months, and 5 patients died with disease at a median o f 31 months after diagnosis. Conclusions. Desmoid tumors are common in patients with FAP. Unresectability and recurrence are more common tha n cure. Palliative and curative resections have a high morbidity. Surg ery should be reserved for those patients with symptomatic mesenteric desmoids.