Wide local excision with negative pathologic margins is the treatment of choice for most desmoid tumors. Function-sparing resection is appropriate because adjuvant radiation therapy can offset the adverse impact of positive margins. Unresectable disease should be treated with definitive radiation therapy.

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A clinical experience with radiotherapy in 18 patients with alveolar soft-part sarcoma is presented. Adjuvant radiotherapy was associated with prolonged local control in six of six patients without metastatic disease at diagnosis; later one patient relapsed systematically. Meaningful palliation was achieved in all patients with extra-skeletal (and possibly skeletal) metastatic disease. Radiation therapy may be beneficial for patients with alveolar soft-part sarcoma by enhancing local control achieved with limited surgery, by retarding progression of metastatic deposits, and by providing meaningful palliation.