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.

Highlights

  • 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 [1], and it is the commonest vascular leiomyosarcoma [2]

  • According to the site involved IVC leiomyosarcomas are divided into 3 levels, Level 1 proximal to the level of Hepatic veins, Level 2 between the level of renal veins and hepatic veins, Level 3 distal to the entry of renal veins to IVC [4]-[6]

  • The tumor progression is slow [7], and it is asymptomatic until advanced stage in which involvement of surrounding structures and distal metastasis occur [8], even when the symptoms are present are nonspecific, that is why the disease is usually diagnosed after several years [9]

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Summary

Background

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 [1], and it is the commonest vascular leiomyosarcoma [2]. The tumor progression is slow [7], and it is asymptomatic until advanced stage in which involvement of surrounding structures and distal metastasis occur [8], even when the symptoms are present are nonspecific, that is why the disease is usually diagnosed after several years [9]. Imaging studies has a major role in diagnosis, demonstration of origin and extension of such tumors [10]

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