A Rare Retroperitoneal Mass: Leiomyosarcoma of the Inferior Vena Cava

Abstract

Leiomyosarcoma of the inferior vena cava (IVC) is a rare soft tissue tumor, mesenchymal in origin that arises from smooth muscles of tunica media; it accounts for about 0.5% of all soft tissue sarcomas, and it is the commonest vascular leiomyosarcoma. The tumor progression is slow, and it is asymptomatic until advanced stage in which involvement of surrounding structures even when the symptoms present are nonspecific. Presentation of Case: A 60 years old lady presented with upper abdominal pain for 3 months duration. Past surgical history was significant for Hysterectomy 15 years ago. On examination: soft abdomen, palpable non pulsating right hypochondrial mass. Ultrasound of the abdomen showed tumor of the head of pancreas. CT scan showed large retroperitoneal tumor extending from the head of pancreas to IVC. Trans abdominal CT guided FNAC showed retroperitoneal sarcoma while Immunohistochemistry (IHC) was proved to be Leiomyosarcoma of the IVC. Discussion: Leiomyosarcoma of inferior vena cava (IVC) is a rare soft tissue tumor, mesenchymal in origin that arises from smooth muscles of tunica media; it accounts for about 0.5% of all soft tissue sarcomas, and it is the commonest vascular leiomyosarcoma. The type of surgical management is a matter of debate and includes resection alone, primary repair/cavoplasty, or replacement with a graft. Reconstruction of the IVC is not always required especially in chronic occlusions. Conclusion: Despite all the advanced modalities, surgery remains the most effective method for treatment of Leiomyosarcoma.