Background: Leiomyosarcoma of the vena cava (LMS-VC) is a rare entity with poor oncological outcomes and a lack of histological staging prognostic factors.
Methods: Outcomes of consecutive patients operated on LMS-VC between March 2003 and May 2022, in two specialized sarcoma centers were reported.
Result: Forty-one patients were identified. Median size of LMS-VC was 9 cm with 68% of complete obstruction. After surgery, severe complication rate was 30%. No postoperative mortality was reported. Microscopic complete excision was obtained for 71% of patients, R1 for 27% and one patient presented an R2 resection. Grade 3 was found in 24%. After a median follow-up of 70 months, 3 years disease-free survival (DFS) and 5 years DFS were 34% and 17%, and 3 years overall survival (OS) and 5 years OS were 74% and 50%. Distant metastasis concerned 54% of recurrences, local 7% and local and distant 5%. Multivariate analysis showed that FNCLCC grade (p < 0.001) and perioperative chemotherapy (p = 0.026) were significant factors for DFS. In multivariate analysis, FNCLCC grade was a significant factor for OS (p = 0.004).
Discussion: Perioperative chemotherapy may have a role to play in lowering the risk of recurrence for LMS-VC, particularly in high-grade tumor.
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