Desmoid disease in patients with familial adenomatous polyposis

PURPOSE: The aim of this retrospective study was to review the clinical fea tures, and surgical and medical management of patients with familial adenom atous polyposis-associated desmoid tumors. METHODS: From 1980 to 1997, 97 o f 780 patients with familial adenomatous polyposis developed desmoid diseas e. Clinical and demographic data; operative notes; and histologic, radiolog ic, and follow-up reports were retrieved from patients' medical records. Ri sk factors for desmoid disease, such as prior surgery, age at desmoid tumor diagnosis, pregnancy, and family history were sought. The outcome after no ncytotoxic and cytotoxic therapy was evaluated with respect to improvement of symptoms. RESULTS: There were 38 males with a mean age of 32.1 years and 59 females with a mean age of 29.1 years. A family history of desmoid tumo rs was found in 41 patients (42 percent), and a history of pregnancy was do cumented in 33 females (56 percent). The most common clinical presentation was small-bowel obstruction (58 percent). One-half of the desmoids were loc ated in the mesentery, and 32 percent were located in the mesentery and the abdominal wall. Desmoids developed after colectomy in 77 cases (80 percent ), after a mean time of 4.6 years. Partial resection of desmoid tumor was p erformed in 46 patients (47 percent), resection of extra-abdominal. desmoid tumors was performed in 17 cases (17 percent), and biopsy only was perform ed in 34 patients (35 percent). Postoperative morbidity was 23 percent afte r desmoid tumor resection. Eight patients (8 percent) died of their intra-a bdominal desmoid. Mean follow-up time was 5.3 years. Sulindac, tamoxifen, o r toremifene therapy was able to alleviate symp toms in only 4 of 31 patien ts. Symptomatic improvement was noted after chemotherapy in six of ten pati ents with extremely complex desmoids. CONCLUSION: Desmoid disease was found in 12.4 percent of our patients with familial adenomatous polyposis. In vi ew of the high rate of morbidity, indication for surgery should be limited mainly to acute or chronic small-bowel obstruction, because resection trigg ers a high recurrence rate. Noncytotoxic therapy was not effective for prog ressive desmoid tumors, whereas chemotherapy was effective in aggressive ca ses of intra-abdominal desmoid tumors.