Management of desmoid tumors

Abstract

Desmoid tumors are rare, being less than 0.03 per cent of all neoplasms. Because of scarcity of data and relatively small numbers of patients, optimal treatment remains controversial. In this report, our experience with 36 patients evaluated and treated from 1960 to 1987 is analyzed. The most common primary site was the wall of the chest (ten); eight tumors originated in the abdominal wall. Nine patients had a history of previous trauma, and eight of these were women. Thirty-two patients had wide local excision and two had amputations. Clear margins were obtained in only 22 patients despite an attempt at wide resection in all instances. With a mean follow-up period of 41 months and a median of 24 months, only one of 22 patients with negative histologic margins had recurrence of tumor. Among the 11 patients with positive margins, four received postoperative radiation therapy and two remain disease-free; of the seven remaining patients with positive margins, three had recurrences. One patient with unresectable disease was treated with tamoxifen with regression of tumor and remains alive 15 months later. These data suggest that the best treatment of desmoid tumors remains resection with a clear margin of normal tissue surrounding the tumor. Adjuvant radiotherapy did not appear to decrease the rate of local recurrence.