Surgery for large intra-abdominal desmoid tumors

Abstract

PURPOSE: Desmoids are rare, locally aggressive but nonmetastasizing fibrous masses that occur sporadically and in association with familial adenomatous polyposis. Therapeutic options are limited, and there is reluctance to operate on mesenteric desmoids because of the risk of complications or recurrence. Consequently, there is an increasing reliance on alternative therapeutic modalities. Nonsurgical options are of variable efficacy, however, and surgery is still required for the complications of desmoids. Each of the last four patients with desmoids presenting to this unit has required life-saving surgery as a result of failure of nonsurgical treatments or the development of complications. METHODS: We present case reports of four consecutive patients with large mesenteric desmoid tumors requiring surgical management. RESULTS: All four patients had massive mesenteric desmoids. Three cases associated with familial adenomatous polyposis had developed their desmoids after colectomy while the sporadic desmoid had continued to grow rapidly after diagnosis at laparotomy. Sulindac and toremifene were unsuccessful in all cases and one patient with familial adenomatous polyposis suffered dramatic erosion of her desmoid through the abdominal wall during antisarcoma chemotherapy. Two others required emergency laparotomy for complications, and the sporadic case underwent elective resection for symptomatic relief. Three had complete excision of their desmoid, and all remained well with no recurrence at a median follow-up of 12 (range, 7–14) months. CONCLUSION: Despite the risks, there remains a role for surgery in the management of large mesenteric desmoids.