Ricerc@Sapienza

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.