CONTEXT: 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%. CASE REPORT: 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. CONCLUSION: We report a case of primary venous LMS arising from the splenic vein and we briefly review the relevant literature.
CONTEXT: 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%. CASE REPORT: 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. CONCLUSION: We report a case of primary venous LMS arising from the splenic vein and we briefly review the relevant literature.