Management of Great Saphenous Vein and Inferior Vena Cava Leiomyosarcomas: Two Surgical Case Reports and Literature Review

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Background: Vascular leiomyosarcoma (LMS) is an exceptionally rare and aggressive soft tissue sarcoma arising from the smooth muscle cells of the vascular wall. They account for approximately 0.5–2% of adult soft-tissue sarcomas and are the most frequent primary malignancy of vascular origin. Among venous sites, the inferior vena cava (IVC) is the most frequently involved, accounting for more than half of reported vascular LMS cases, with rarer occurrences in peripheral veins, including the internal saphenous vein and the external iliac vein. Case Presentation: We report a case series comprising two distinct presentations of vascular LMS involving the internal saphenous vein and the inferior vena cava, respectively. Each case highlights unique clinical manifestations, radiologic features, histopathologic diagnosis, and therapeutic challenges inherent to the involved vascular territory. Surgical resection with clear margins was the primary treatment modality, complemented by adjuvant therapies tailored according to tumor grade and extent. Literature Review: An updated literature review contextualizes these findings, detailing epidemiology, diagnostic challenges, prognostic factors, and current management approaches. It emphasizes the rarity of leiomyosarcomas originating from major venous pathways and highlights variability in clinical presentation, tumor size, growth patterns, and outcomes. Achieving complete surgical removal with negative margins continues to be the primary treatment goal and the most significant prognostic factor. Conclusions: Given the paucity of cases, our series contributes valuable insights into the clinical spectrum and multidisciplinary approach necessary for optimal outcomes in vascular LMS. Early recognition and aggressive treatment remain paramount to improving survival in this rare malignancy.