THE MANAGEMENT OF DESMOID TUMORS

Purpose: To determine the efficacy of different treatment modalities f or desmoid tumors. Materials and Methods: We reviewed the treatment of 40 patients with histologically confirmed desmoid tumors seen at Duke University Medical Center between 1974 and 1990. Results: Radiotherap y was administered to 16 patients (Group I)-14 with recurrent disease s/p surgery and in two as initial treatment. The average size of the i rradiated lesions was 9.3 +/- 3.9 X 8.4 +/- 3.5 cm. With a median foll ow-up of 57.5 months and a median administered dose of 5400 cGy (mean 5286 cGy, range 4960-5620 cGy), local control has been obtained in 15/ 16 patients (94%). Complete regression (5/16), partial regression (5/1 6), or stable disease (5/16) was produced in 15 patients while one pat ient failed and was salvaged via gross total resection. Continued regr ession has been seen up to 60 months after treatment. Fourteen patient s underwent primary gross total resection and two underwent subtotal r esection (Group II). None received post-operative radiotherapy. Three of 14 patients (21%) recurred after gross total resection. All three w ere salvaged with subsequent gross total resection. After subtotal res ection, 2/2 patients recurred. With a mean follow-up of 52 months, 14 patients are without evidence of disease, one is dead with disease (un related cause of death), and one was lost to follow-up after recurrenc e. Eight patients have been treated with combinations of chemotherapy, NSAIDS, anti-estrogens, and immunotherapy with mixed results (Group I II). A subset of seven patients with retroperitoneal disease taken fro m all three groups had large tumor burden (mean size 17 X 15 cm), an i nfiltrative nature, as well as a difficult location. The disease was s urgically resectable in three patients. One is without evidence of dis ease 9 years after gross total resection alone. Disease has been stabi lized with radiotherapy in the other two patients after multiple unsuc cessful surgical resections. Of four patients with unresectable diseas e, two are dead of disease, one died of unrelated causes with disease, and regression of disease was obtained in the other with Gamma-interf eron after unsuccessful treatment with tamoxifen and vincristine, doxo rubicin, and cyclophosphamide chemotherapy. Conclusion: Gross total re section is the indicated initial therapy, if it can be performed witho ut significant disfigurement. Radiotherapy is also excellent for obtai ning local control, even in patients with a large burden of recurrent disease. Doses in the range of 50 to 55 Gy give a chance of local cont rol equal to that obtained with higher doses previously reported.