Primary leiomyosarcoma of the inferior vena cava

1. Leiomyosarcoma of the IVC (IVCLMS) is a very rare retroperitoneal tumor, accounting for only about 0.5 % of adult soft tissue sarcomas. 2. Complete R0 surgical resection of surrounding involved organs is the mainstay of treatment, with medical oncological treatments (CT, RT, and CHRT) incom- pletely verified and partially ineffective. 3. IVC ligation should be abandoned considering the high risk of leg edema and local/peripheral complications. 4. IVCresectionandcompletereconstructionisthetreatmentofchoice,bothusing homologous and prosthetic graft implants. 5. Survival is poor, but radical resection can allow long disease-free intervals and adequate cumulative survivals.