Department of Orthopedic Surgery, Section of Orthopedic Oncology, Rush University Medical Center, Chicago, Illinois, USA.
J Surg Oncol. 2021 Apr;123(5):1274-1283. doi: 10.1002/jso.26404. Epub 2021 Feb 1.
This investigation sought to describe the outcomes of primary leiomyosarcoma of bone (PLB) compared to soft tissue leiomyosarcoma (SLMS).
This was a review of the Surveillance, Epidemiology, and End Results database from 1975 to 2016. Kaplan-Meier methods were used to estimate disease-specific survival (DSS), and a Cox regression model was used to identify prognostic factors.
Of the 7502 identifiable cases, 1% (n = 74) were PLB and 99% (n = 7428) were SLMS. Survival was the same between PLB and SLMS (p = .209). On multivariable analysis for high-grade SLMS, radiation (neoadjuvant: hazard ratio [HR], 0.56; 95% confidence interval [CI], 0.4-0.8; p = .003; adjuvant: HR, 0.75; 95% CI, 0.6-0.9; p = .008) and surgery (procedure specific) improved DSS. For PLB, wide resection/limb salvage (HR, 0.40; 95% CI, 0.3-0.5; p = .018) and amputation (HR, 0.69; 95% CI, 0.5-0.9; p < .001) were positive prognostic factors. Neither radiation nor chemotherapy were prognostic factors for survival in PLB.
For SLMS, radiation portends a survival advantage. For PLB, however, neither chemotherapy nor radiation were significant prognostic factors, which suggests the optimal treatment for PLB, similar to other primary soft tissue sarcomas originating in bone, remains an unmet medical need.
本研究旨在描述骨原发性平滑肌肉瘤（PLB）与软组织平滑肌肉瘤（SLMS）的预后结果。
这是对 1975 年至 2016 年监测、流行病学和最终结果数据库的一项回顾。采用 Kaplan-Meier 法估计疾病特异性生存率（DSS），并采用 Cox 回归模型确定预后因素。
在 7502 例可识别病例中，1%（n=74）为 PLB，99%（n=7428）为 SLMS。PLB 和 SLMS 之间的生存情况相同（p=0.209）。对于高级别 SLMS，多变量分析显示，放疗（新辅助：风险比 [HR]，0.56；95%置信区间 [CI]，0.4-0.8；p=0.003；辅助：HR，0.75；95%CI，0.6-0.9；p=0.008）和手术（特定手术方式）改善了 DSS。对于 PLB，广泛切除/保肢（HR，0.40；95%CI，0.3-0.5；p=0.018）和截肢（HR，0.69；95%CI，0.5-0.9；p<0.001）是阳性预后因素。放疗和化疗均不是 PLB 生存的预后因素。
对于 SLMS，放疗预示着生存优势。然而，对于 PLB，化疗和放疗都不是显著的预后因素，这表明 PLB 的最佳治疗方法与起源于骨骼的其他原发性软组织肉瘤相似，仍然是一个未满足的医疗需求。
