3.P.50 MULTIMODAL THERAPY IN PATIENTS WITH DESMOID TUMOURS OF THE EXTREMITIES

Abstract

Desmoid tumors are rare benign but aggressive lesions. They are characterized by bland-appearing fibroblasts, indistinct margins, and the ability of local invasion and recurrence. Though they are not cancer they may metastasize and can cause significant morbidity. Treatment is primarily surgical, although radiation or systemic therapy can be beneficial if surgery is not feasible. We retrospectively reviewed our patients since 1980 in respect to treatment modalities and outcome.

Between 1980 and 2008 26 patients (16 m, 10 f) with desmoid tumours had been surgically treated. The mean age with diagnosis was 37 years (7 – 69 years). The mean age at surgery was 46 years (10 – 81 years). 17 of the patients had only one resection. In 6 patients two resections, in 2 three resections and in one patient four resections had to be performed. Only 7 patients achieved a R0-situation. In 9 patients adjuvant radiotherapy was used. Two patients had several courses of Vinblastine or MTX based chemotherapy, three patients had Sulindac or Tamoxifen, several other patients combinations of different NSARs. All patients were still alive, one patient developed metastatic disease from the initial lesion in the groin to the foot. After an average of 17 months (7 – 42 months) 11 patients showed recurrent disease. 13 patients are without recurrence after an average of more than 9 years. In 4 patient stable disease is seen without progression in 42 to 156 months (Ø 95 months).

In conclusion desmoid tumours did show an inpredictable course of disease. Due to many alterations in treatment in the 28 years since the first patient of this study and the rareness of this disease no clear predicitve factor could be established. Remarkably no patient did need an amputation. A more detailed analysis regarding the dose effect of irradiated patients is intented.