Surgical treatment as well as radiation and chemotherapy may improve survival in selected cases but probably have their value much more in terms of local disease control and improvement life quality of patients who probably already suffer from an aggressive systemic disease at time of nodal involvement.

In patients with high-risk soft tissue sarcomas, IORT +/- EBRT after limb-preserving surgery achieves high local control rates. The risk of normal tissue toxicities is comparable to conventional limb-sparing treatment.

BackgroundLeiomysarcoma of intravascular origin is an exceedingly rare entity of malignant soft tissue tumors. They are most frequently encountered in the retroperitoneum arising from the inferior vena cava and are scarcely found to arise from vessels of the extremities. These tumors were analysed with particular reference to treatment outcome and prognosis. The aim of this article is to broaden the knowledge of the clinical course of this rare malignancy.MethodDuring 2000 and 2009 twelve patients were identified with an intravascular origin of a leiomyosarcoma. Details regarding the clinical course, follow-up and outcome were assessed with focus on patient survival, tumor relapse and metastases and treatment outcome. 3 year survival probability was calculated using Kaplan-Meier method.ResultsVascular leiomyosarcomas accounted for 0.7% of all malignant soft tissue tumors treated at our soft tissue sarcoma reference center. The mean follow up period was 38 months. Tumor relapse was encountered in six patients. 6 patients developed metastatic disease. The three year survival was 57%.ConclusionVascular leiomysarcoma is a rare but aggressive tumor entity with a high rate of local recurrence and metastasis.