软组织透明细胞肉瘤(CCS),上皮样肉瘤,滑膜肉瘤是历史上被确定为淋巴结转移高风险的罕见肿瘤。这项研究调查了患有这些亚型的儿童和年轻人的淋巴结转移和相关生存率。
使用国家癌症数据库(2004-2015),我们建立了一个由1303名接受CCS局部对照治疗的患者(年龄≤25岁)组成的回顾性队列,上皮样肉瘤,和滑膜肉瘤.Kaplan-Meier曲线按亚型估计总生存期(OS)。在亚型上分层,Cox回归通过淋巴结采样状态和淋巴结转移评估OS。
有103例(7.9%)CCS患者,221(17.0%)伴有上皮样肉瘤,滑膜肉瘤979例(75.1%)。与滑膜肉瘤相比,CCS(56.3%)和上皮样肉瘤(52.5%)患者的淋巴结采样频率更高(20.5%,p<.001)。滑膜肉瘤转移到淋巴结的频率低于CCS或上皮样肉瘤(2.1%vs.14.6%和14.9%,p<.001)。在所有亚型中,淋巴结转移与下OS相关(HR2.02,CI1.38-2.95,p<.001)。淋巴结采样与CCS的OS改善相关(HR0.35,CI:0.15-0.78,p=.010),滑膜肉瘤的OS较差(HR1.60,CI:1.13-2.25,p=.007),与上皮样肉瘤的OS无统计学关联。
淋巴结转移在儿童和青年滑膜肉瘤中很少见。对于患有CCS或上皮样肉瘤的患者,未进行一致的淋巴结采样程序。但改进的OS支持对患有CCS的儿童和年轻人进行常规淋巴结采样。
Clear cell sarcoma of soft tissue (CCS), epithelioid sarcoma, and synovial sarcoma are rare tumors historically identified as high risk for lymph node metastasis. This study investigates incident nodal metastasis and associated survival in children and young adults with these subtypes.
Using the National Cancer Database (2004-2015), we created a retrospective cohort of 1303 patients (aged ≤25 years) who underwent local control therapy for CCS, epithelioid sarcoma, and synovial sarcoma. Kaplan-Meier curves estimated overall survival (OS) by subtype. Stratifying on subtype, Cox regressions assessed OS by lymph node sampling status and nodal metastasis.
There were 103 (7.9%) patients with CCS, 221 (17.0%) with epithelioid sarcoma, and 979 (75.1%) with synovial sarcoma. Lymph node sampling was more frequent in patients with CCS (56.3%) and epithelioid sarcoma (52.5%) versus synovial sarcoma (20.5%, p < .001). Synovial sarcoma metastasized to lymph nodes less frequently than CCS or epithelioid sarcoma (2.1% vs. 14.6% and 14.9%, p < .001). Across all subtypes, lymph node metastasis was associated with inferior OS (HR 2.02, CI 1.38-2.95, p < .001). Lymph node sampling was associated with improved OS in CCS (HR 0.35, CI: 0.15-0.78, p = .010), inferior OS in synovial sarcoma (HR 1.60, CI: 1.13-2.25, p = .007), and no statistical association with OS in epithelioid sarcoma.
Lymph node metastasis is rare in children and young adults with synovial sarcoma. Lymph node sampling procedures were not consistently performed for patients with CCS or epithelioid sarcoma, but improved OS supports routine lymph node sampling in children and young adults with CCS.