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Abstract 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.
Bertrand et al. (Sun,) studied this question.