Abstract
BACKGROUND
Desmoid tumors are mesenchymal nonmetastasizing neoplasms. Although rare in the general population, they are a common extracolonic manifestation of familial adenomatous polyposis (FAP). Because of high tumor recurrence rates, surgery has been less than satisfactory in the treatment of desmoid tumors. In the current study, high doses of tamoxifen in combination with sulindac were used to treat severe desmoid tumors to avoid surgery.
METHODS
Since 1992, 25 patients at Heinrich Heine University (Dusseldorf, Germany) were treated with a combination of tamoxifen and sulindac. In the current study, 17 patients with FAPโassociated and 8 patients with sporadic desmoid tumors received 120 mg of tamoxifen and 300 mg of sulindac daily. Every 6 months, the protracted course of desmoid growth was measured by computed tomography and/or magnetic resonance imaging scans. Tumor responses were characterized as progressive disease, stable disease (SD), partial regression (PR), and complete regression (CR).
RESULTS
Of the group of patients who received tamoxifen and sulindac as a primary treatment, all three patients with sporadic desmoid tumors demonstrated cessation of growth, and 10 of the 13 patients with FAPโassociated tumors achieved either a PR or CR. In the sporadic desmoid tumor group, eight of nine patients developed tumor recurrences after undergoingsurgery at other institutions. Of these, two patients had SD and two patients had a PR to CR.
CONCLUSIONS
The patients with desmoid tumors who were managed conservatively with highโdose tamoxifen and sulindac had the best outcome. Desmoid tumor recurrence after surgery was high and in the FAPโassociated tumor group, therapy with tamoxifen and sulindac was found to be less successful. Based on this experience, the authors recommended highโdose tamoxifen and sulindac as the primary treatment for patients with FAPโassociated desmoid tumors. However, to our knowledge, the best approach after surgical intervention for patients with sporadic desmoid tumors remains to be determined. Cancer 2004. ยฉ 2003 American Cancer Society.