ABSTRACT
Vascular leiomyosarcoma (LMS) is an extremely rare disease. Venous lesion is approximately 5 times more common than arterial. LMS is usually found in retroperitoneal veins and in inferior vena cava. Lesion of the splenic vein is extremely rare. A 52-year-old woman with recurrent abdominal pain is reported in the article. Abdominal contrast-enhanced CT revealed a 6 cm tumor of the pancreatic tail. The patient underwent laparoscopic distal pancreatectomy with splenectomy en bloc. Histological and immunohistochemical studies revealed LMS originating from the smooth muscles of the splenic vein. Surgery was followed by adjuvant chemotherapy. There was no local recurrence in 1 year after surgery. In this article, we analyze the available information on leiomyosarcoma of the splenic vein.