Background: Leiomyosarcoma of the inferior vena cava (IVC) is a rare mesenchymal tumor with poor prognosis. Surgical resection is currently the only potential curative treatment. This study analyzed long-term outcomes of patients who underwent surgical resection of leiomyosarcoma of the IVC.
Methods: The charts of 12 patients who underwent surgical resection of leiomyosarcoma of the IVC from January 1999 to December 2017 at a single center were retrospectively reviewed.
Results: Of the 12 patients, 10 (83.3%) were women. Median age at diagnosis was 63 years (range 42-67). Leiomyosarcoma involved the middle segment of the IVC in 9 patients (75.5%) and the lower segment in 3 (25.0%). Ten patients underwent IVC resection, followed by reconstruction with polytetrafluoroethylene in 9 patients and a bovine patch in 1. Two patients underwent IVC resection followed by ligation of the IVC. Eleven patients (91.7%) underwent grossly radical resection, with 1 (8.3%) having peritoneal seeding at the first operation. After resection, 8 patients received adjuvant chemotherapy and 7 received adjuvant radiotherapy. No patient experienced regional recurrence at the resection margins of the IVC, but 9 patients (75.5%) experienced distant metastases to sites such as the lungs, liver, bones, pelvis, peritoneum, and scalp. Median follow-up was 41 months (range 6-149). Median disease-free survival (DFS) was 49 months (range 8-88), and median overall survival (OS) was 127 months (range 25-149). The 3- and 5-year DFS rates were 77.9% and 39.0%, respectively, and the 3-, 5-, and 10-year OS rates were 87.5%, 75.0%, and 56.3%, respectively.
Conclusions: Although there is no established treatment for leiomyosarcoma of the IVC and metastasis after surgery is frequent, surgical resection followed by chemotherapy and/or radiotherapy can enhance long-term survival.
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