Desmoid tumors: a novel approach for local control

Abstract

Background and objectives: 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.

Methods: 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.

Results: 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).

Conclusions: 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.