Splenic vein leiomyosarcoma: case report and review of the literature.

Background: Primary venous leiomyosarcoma (LMS) is a rare disease, most commonly affecting the retroperitoneal veins and in particular the inferior vena cava. Five-year survival rate ranges between 33% and 68%.

Methods: Complete surgical resection represents the only potentially curative treatment, occasionally achieving long-term survival. LMS of the splenic vein is extremely rare, with only three cases reported in the literature.

Conclusions: We report a case of primary venous LMS arising from the splenic vein and we briefly review the relevant literature.