Abstract
Introduction: Leiomyosarcoma of the venous system is rare, even more so in the greater saphenous vein. In the 85 years since van Ree described the first case in 1919 only 25 cases have been reported in the world.Methods: We describe a case of an 85-year-old woman who was successfully treated by excision of the tumour. We also reviewed pertinent literature with regard to age, gender spread, tumour size, survival, occurrence of metastases and therapy.Results: The median age was 54 years (range 2–85 years), with a 3:2 female to male ratio. The median size of the tumours was 4.1 cm (range 2–12 cm) and metastases occurred in seven of the 25 cases. If any form of adjuvant therapy is used it is usually radiotherapy. Chemotherapy seems to be reserved for cases where metastasis occurs. Average survival was 4 years (range 1 month to 17 years). Currently the best treatment seems to be wide excision of the tumour, with selective vascular reconstruction combined with adjuvant radiotherapy.