Desmoid tumors: a novel approach for local control.

Abstract

After resection, desmoid tumors are associated with a recurrence rate that is typically 25-50%. Although this is an unusual problem, we instituted a prospective cohort study with neoadjuvant chemotherapy and radiation, followed by surgical resection, in an effort to improve local control. Between 1985 and 1999, 13 patients with potentially resectable disease were managed with a treatment protocol of preoperative doxorubicin (30 mg continuous infusion daily for 3 days) and radiotherapy (10 x 300 cGy). Resection was performed 4-6 weeks later. All lesions were resected with an intended margin of 1 cm, but clear adventitial margins were accepted in order to preserve critical structures. The median follow-up was 71 months (range, 22-109). Six patients (46%) presented after failure of a previous surgery. Clear microscopic margins were obtained in 11 patients, and 2 patients had positive margins. There were two local recurrences (15% local recurrence). Both recurrences followed resection of large thigh lesions, which appeared at 30 and 49 months of follow-up. In one patient with a chest wall tumor, two new primary desmoid tumors developed outside the treatment area, in the ipsilateral arm and forearm. Eleven patients have been disease free for a median of 71 months (range, 22-109). For potentially resectable lesions, this protocol provides excellent local control, even in those with recurrent disease. Neoadjuvant treatment with doxorubicin and radiotherapy appears to be a better option than surgery alone, or surgery and adjuvant radiotherapy. These results need to be confirmed in larger, prospective randomized trials.