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.
RESULTS: 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).
CONCLUSIONS: Perioperative chemotherapy may have a role to play in lowering the risk of recurrence for LMS-VC, particularly in high-grade tumor.
METHODS: Outcomes of consecutive patients operated on LMS-VC between March 2003 and May 2022, in two specialized sarcoma centers were reported.
RESULTS: 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).
CONCLUSIONS: Perioperative chemotherapy may have a role to play in lowering the risk of recurrence for LMS-VC, particularly in high-grade tumor.
摘要：
背景：腔静脉平滑肌肉瘤（LMS-VC）是一种罕见的实体，肿瘤学结果较差，缺乏组织学分期预后因素。
方法：报告了2003年3月至2022年5月在两个专门的肉瘤中心连续接受LMS-VC手术的患者的结果。
结果：确定了41例患者。LMS-VC的中位尺寸为9cm，完全性梗阻占68％。手术后,严重并发症发生率为30%。无术后死亡报告。71％的患者获得了显微镜下的完全切除，R1占27%，一名患者进行R2切除。在24%中发现了3级。经过70个月的中位随访，3年无病生存率(DFS)和5年DFS分别为34%和17%,3年总生存率（OS）和5年OS分别为74％和50％。远处转移与54%的复发有关，当地的7%和当地和遥远的5%。多因素分析显示FNCLCC分级(p<0.001)和围手术期化疗(p=0.026)是影响DFS的重要因素。在多变量分析中，FNCLCC等级是OS的重要因素(p=0.004)。
结论：围手术期化疗可能在降低LMS-VC复发风险方面发挥作用，特别是在高级别肿瘤中。
方法：报告了2003年3月至2022年5月在两个专门的肉瘤中心连续接受LMS-VC手术的患者的结果。
结果：确定了41例患者。LMS-VC的中位尺寸为9cm，完全性梗阻占68％。手术后,严重并发症发生率为30%。无术后死亡报告。71％的患者获得了显微镜下的完全切除，R1占27%，一名患者进行R2切除。在24%中发现了3级。经过70个月的中位随访，3年无病生存率(DFS)和5年DFS分别为34%和17%,3年总生存率（OS）和5年OS分别为74％和50％。远处转移与54%的复发有关，当地的7%和当地和遥远的5%。多因素分析显示FNCLCC分级(p<0.001)和围手术期化疗(p=0.026)是影响DFS的重要因素。在多变量分析中，FNCLCC等级是OS的重要因素(p=0.004)。
结论：围手术期化疗可能在降低LMS-VC复发风险方面发挥作用，特别是在高级别肿瘤中。
