Surgical approach, management, and oncologic outcomes of primary leiomyosarcoma of the inferior vena cava: An institutional case series.

Background: Leiomyosarcoma of the inferior vena cava (IVC) is a rare tumor arising from the smooth muscle of vessel walls. Surgery is the only potential curative treatment. Given its rarity, optimal surgical, and oncologic management is not well described. We review our institutional series of primary leiomyosarcomas treated with resection and IVC reconstruction over the last decade.

Methods: Retrospective chart review of all patients who underwent surgical resection of primary leiomyosarcoma of the IVC from November 2009 to March 2020 at a single tertiary care center was performed.

Results: Among the eight patients treated, the majority were female (87.5%) with a median age of 52 years (range, 44-63). Tumor was located in the infrarenal IVC in five patients (62.5%). IVC was reconstructed using a ring-enforced PTFE graft in six patients (75%). All but one patient had an intermediate (grade 2) or high grade (grade 3) tumor, and all resections achieved grossly negative margins. The 1- and 3-year disease-free survival was 85.7% and 64.3%, respectively. There were no disease-specific deaths during a median follow-up of 36 months (interquartile range, 10-51 months).

Conclusions: With a well-coordinated multidisciplinary approach, primary leiomyosarcoma of the IVC can be safely resected with good long-term survival.