Cytoreductive strategy for multiple intra-abdominal and abdominal wall desmoid tumors in familial adenomatous polyposis: Report of three cases

Desmoid tumors (DTs) are benign myofibroblastic neoplasms originating from the fascia or muscle aponeurosis, which occur in one-third of patients with familial adenomatous polyposis (FAP). Most FAP-associated DTs occur in the intra-abdominal or abdominal wall region, thus, their infiltrative or expansive growth causes life-threatening organ damage, such as intestinal obstruction, urethral obstruction, and mesenteric infiltration with the involvement of mesenteric vessels. Treatments including surgical resection, cytotoxic chemotherapy, nonsteroidal anti-inflammatory drugs and anti-estrogen therapy have all been tried with variable success. Here, we report on three patients with FAP who developed multiple intra-abdominal and abdominal wall DTs after total proctocolectomy and ileal pouch-anal anastomosis. Two cases underwent surgical resection of uncontrolled abdominal wall DTs after successful control of intra-abdominal DTs by systemic chemotherapy. The remaining case underwent repeated surgical resections of multiple intra-abdominal and abdominal wall DTs, and consequently had recurrent intra-abdominal DTs, with involvement of the small bowel and ureter. Surgical intervention as tumor volume reduction (cytoreduction) may be useful for cases with medical treatment-refractory or symptomatic FAP-associated abdominal DTs.