Combination chemotherapy for desmoid tumors

BACKGROUND: Desmoid tumor (aggressive fibromatosis) is an aggressive fibroblastic proliferation of well circumscribed, locally invasive, differentiated fibrous tissue. For patients with desmoid tumors that are not amenable to surgery or radiation therapy, the use of hormonal agents and nonsteroidal antiinflammatory drugs (NSAIDs) have been attempted, with some success. The use of chemotherapy also has been reported to have activity. METHODS: Seven patients (5 males and 2 females) with a median age of 40 years (range, 17-66 years) who received cytotoxic chemotherapy (combinations of cyclophosphamide and doxorubicin; mitomycin, doxorubicin, and cisplatin; and ifosfamide and etoposide) for desmoid tumor were reviewed retrospectively. Five patients were found to have recurrent tumors. Four patients had familial adenomatous polyposis. Four patients had failed tamoxifen and six had failed NSAIDs prior to receiving cytotoxic chemotherapy. In six patients the desmoid tumor was intraabdominal and one tumor had occurred on the buttock. RESULTS: Patients received a median number of six cycles of chemotherapy (range, two to eight cycles). Objective disease regression occurred in 3 patients. There was an apparent clinical benefit in six patients with the duration of benefit ranging from 3 months to 15 years. The chemotherapy was well tolerated and no treatment-related mortality was reported. CONCLUSIONS: The results of the current study indicate that the use of combination chemotherapy for desmoid tumors may provide long-term clinical benefits.