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Keywords:

  • desmoids;
  • aggressive fibromatosis;
  • systemic therapy;
  • nonsurgical treatment

Abstract

Background and Objectives

Surgery is the main treatment for extra-abdominal desmoid tumors, but the results of further management remain uncertain. Therefore, a retrospective analysis was undertaken to evaluate the toxicity and efficacy of treatment with interferon-alpha (IFN-α) ± tretinoin in this setting.

Methods

Thirteen patients with extra-abdominal desmoid tumors and a median age of 32 years (range, 15–73) received IFN-α. Seven of these patients received a combination of IFN-α and tretinoin in order to test further enhancement.

Results

After a mean observation period of 27 ± 15 months (mean ± standard deviation) under treatment with IFN-α ± tretinoin, local control was seen in 11 of 13 patients (85%). Seven patients had no evidence of disease at a mean disease-free interval of 22 ± 18 months; in two patients progressive disease occurred after only 7 and 9 months, respectively, of observation. In another four patients, progression of the desmoid tumor was stabilized.

Conclusions

The data of this retrospective, nonrandomized study on therapy with IFN-α ± tretinoin suggest that such treatment may be effective in prolonging the disease-free interval of patients after intralesional or marginal surgery. Because of the encouraging response rate, this regimen appears to be another nonsurgical treatment alternative. J. Surg. Oncol. 2000;73:21–25. © 2000 Wiley-Liss, Inc.