背景:在腹膜后平滑肌肉瘤(RPLMS)中,主要问题是远处转移(DM)。我们试图确定与该结果和疾病特异性死亡(DSD)相关的变量。
方法:回顾性收集了2002年至2023年在高容量中心治疗的原发性RPLMS患者的数据。对于下腔静脉(IVC)起源的肿瘤,在每个切除标本上重新评估宏观血管侵犯的程度,并与术前横断面成像相关.估计DM和DSD的粗累积发生率,并进行单变量和多变量模型。
结果:在157名研究患者中,中位肿瘤大小为11.0cm,96.2%的病例为中高级.所有患者均接受完全切除,56.7%接受化疗(43.9%新辅助治疗),14.6%接受放疗。仅肿瘤大小和等级,而不是肿瘤起源的部位(例如,IVCvs.其他)与DM和DSD相关(p<0.05)。在64例IVC起源肿瘤患者中,基于内膜破坏的水平设计了一种新的三层分类,与DM(p=0.007)和DSD(0.002)相关。
结论:在主要RPLMS中,只有肿瘤大小和分级可预测DM和DSD。在IVC起源的肿瘤中,宏观血管侵犯的程度也能强烈预测这些结局.
BACKGROUND: In retroperitoneal leiomyosarcoma (RP LMS), the predominant issue is distant metastasis (DM). We sought to determine variables associated with this outcome and disease-specific death (DSD).
METHODS: Data were retrospectively collected on patients with primary RP LMS treated at a high-volume center from 2002 to 2023. For inferior vena cava (IVC)-origin tumors, the extent of macroscopic vascular invasion was re-assessed on each resection specimen and correlated with preoperative cross-sectional imaging. Crude cumulative incidences were estimated for DM and DSD and univariable and multivariable models were performed.
RESULTS: Among 157 study patients, median tumor size was 11.0 cm and 96.2% of cases were intermediate or high grade. All patients underwent complete resection, 56.7% received chemotherapy (43.9% neoadjuvant) and 14.6% received radiation therapy. Only tumor size and grade and not site of tumor origin (e.g., IVC vs. other) were associated with DM and DSD (p < 0.05). Among 64 patients with IVC-origin tumors, a novel 3-tier classification was devised based on the level of intimal disruption, which was associated with both DM (p = 0.007) and DSD (0.002).
CONCLUSIONS: In primary RP LMS, only tumor size and grade are predictive of DM and DSD. In IVC-origin tumors, the extent of macroscopic vascular invasion is also strongly predictive of these outcomes.