Leiomyosarcoma of Intrahepatic IVC - Treatment with Radical Resection and IVC Replacement with PTFE Graft: A Case Report and Review of Literature

Abstract

Leiomyosarcomas of the Inferior Vena Cava (IVC) are rare tumors accounting for only 0.5% of all soft tissue sarcomas in adults with fewer than 300 cases reported. Intraluminal leiomyosarcoma of IVC is rare. The involvement of renal or hepatic veins determines the strategy for vascular reconstruction. Recent studies have shown that radical surgery with adjuvant multimodal therapy improves the cumulative survival rate. We report a case of IVC leiomyosarcoma in a young healthy woman along with diagnostic workup followed by resection of tumor and reconstruction of IVC by Polytetrafluoroethylene (PTFE) graft. She reports no recurrence/complications after 6 cycles of chemotherapy and 40 months of follow-up. The prognosis depends on grade of the tumor, segment of the IVC involved (level 2-better prognosis), size of tumor (size<9 cm better prognosis) and adjuvant therapy. However, overall leiomyosarcoma runs a poor prognosis; over half of the patients with surgical excision develop recurrence. The 5-year survival rate for these patients ranges between 31 and 62%.