Chemotherapy for desmoid tumors in association with familial adenomatous polyposis

PURPOSE: This study was designed to assess the effect of chemotherapy on complex desmoid tumors associated with familial adenomatous polyposis. METHODS: Five patients (3 males, 2 females, age range, 29-45 years) had symptomatic, unresectable intraabdominal desmoid tumors in association with familial adenomatous polyposis that were unresponsive to conventional medical therapy. Each patient was treated with a cytotoxic chemotherapeutic regimen consisting of doxorubicin and dacarbazine followed by carboplatin and dacarbazine. Response to treatment was assessed by measurement of tumor size using computerized tomography. Follow-up has been for a mean of 22 (range, 10-30) months. RESULTS: One patient has had a complete response, and three patients have had a partial response, with a reduction in tumor volume of at least 50 percent. One patient had a minimum response to treatment and developed a rapid increase in tumor size on cessation of therapy. Complications of treatment included febrile neutropenia, severe epistaxis, and subclavian vein thrombosis. CONCLUSIONS: The cytotoxic chemotherapeutic regimen described is effective in the treatment of selected unresectable desmoid tumors associated with familial adenomatous polyposis and should be considered in symptomatic patients who do not respond to conventional medical therapy.