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 polypos is. METHODS: Five patients (3 males, 2 females, age range, 29-45 years ) had symptomatic, unresectable intraabdominal desmoid tumors in assoc iation with familial adenomatous polyposis that were unresponsive to c onventional medical therapy. Each patient was treated with a cytotoxic chemotherapeutic regimen consisting of doxorubicin and dacarbazine fo llowed by carboplatin and dacarbazine. Response to treatment was asses sed by measurement of tumor size using computerized tomography. Follow -up has been for a mean of 22 (range, 10-30) months. RESULTS: One pati ent has had a complete response, and three patients have had a partial response, with a reduction in tumor volume of at least 50 percent. On e patient had a minimum response to treatment and developed a rapid in crease in tumor size on cessation of therapy. Complications of treatme nt included febrile neutropenia, severe epistaxis, and subclavian vein thrombosis. CONCLUSIONS: The cytotoxic chemotherapeutic regimen descr ibed is effective in the treatment of selected unresectable desmoid tu mors associated with familial adenomatous polyposis and should be cons idered in symptomatic patients who do not respond to conventional medi cal therapy.