Desmoid Tumors Complicated by Familial Adenomatous Polyposis in Siblings

Abstract

It is often difficult to clinically manage patients with desmoid tumors complicated by familial adenomatous polyposis (FAP). Case 1: A 20-year-old female underwent total colectomy for FAP. The diagnosis of mesenteric desmoid tumor associated with FAP had been made when she underwent surgical treatment for suspicious ovarian tumor at age 19. Administration of sulindac, prednisolone, and tamoxifen resulted in no effect on the intraabdominal and abdominal wall desmoids, and they rapidly grew after the second surgery. She died of ileus due to the progressive desmoid tumors 27 months following the second surgery. Case 2 : A 23-year-old female, who was the elder sister of Case 1, underwent total colectomy for FAP. The desmoid tumor, which developed in the operative scar, was resected 10 months after the primary operation. Administration of sulindac was started after the second operation, but the desmoid recurred 26 months postoperatively. A careful follow-up has been made over the past four months because of its slow growth. Careful consideration is needed when scheduling prophylactic colectomy for patients with FAP whose family members have been affected by desmoid tumors.