University of Miami Miller School of Medicine, Miami, FL, United States.
University of Miami Miller School of Medicine, Miami, FL, United States.
Cancer Treat Res Commun. 2022;31:100530. doi: 10.1016/j.ctarc.2022.100530. Epub 2022 Feb 5.
Extraskeletal myxoid chondrosarcoma is a rare form of soft tissue sarcoma characterized by a unique chromosomal translocation involving the NR4A3 gene on chromosome 9. It is most frequently diagnosed in the proximal extremities of older adult males and is notable for its insidious growth with predilection for local recurrence and metastasis. Currently, extraskeletal myxoid chondrosarcoma is managed with wide resection, with recent investigations supporting the utility of adjuvant radiation and novel chemotherapeutic strategies.
A retrospective study was performed with the Surveillance, Epidemiology, and End-Results (SEER) database, which was searched for cases of extraskeletal myxoid chondrosarcoma diagnosed between years 2004 and 2015. Demographic variables were assessed, as well as Collaborative Staging variables including tumor size, metastatic disease, grade, and lymph node involvement. Cases were stratified according to the anatomic site of the primary tumor and were described by therapeutic intervention. A multivariate Cox proportional hazards model evaluated predictive factors for poor survival, and Kaplan-Meier analyses assessed effects of various staging, demographic, and therapeutic variables on overall survival.
There were 270 cases of extraskeletal myxoid chondrosarcoma reviewed in this study, which were diagnosed most frequently in the lower limb or hip of older adult males. The 5-year overall survival was 76.5% and was worse on univariate assessment for patients with age > 60, high histologic grade, pelvic location, tumor size > 8.0 cm, metastatic or nodal spread, and in patients without surgical intervention. The Cox regression predicted significantly worse survival for older age, larger tumor size, non-surgical status, and high tumor grade. Metastasis did not significantly predict worse survival on multivariate assessment, and neither chemotherapy nor radiotherapy provided a discernable improvement in survival in this cohort.
As a rare soft tissue sarcoma, many of the presenting features and survival outcomes of extraskeletal myxoid chondrosarcoma remain poorly defined due to the limited prevalence of this disease. The findings of this study suggest the overall survival may be worse than previously reported, and poor prognostic factors are those associated with worse survival in other soft tissue sarcomas, including high histologic grade, older age, larger tumor size, and lack of wide resection. Radiation and chemotherapy did not demonstrably improve survival for patients with localized or metastatic disease.
extraskeletal myxoid chondrosarcoma 是一种罕见的软组织肉瘤，其特征是涉及 9 号染色体上的 NR4A3 基因的独特染色体易位。它最常发生在老年男性的近端肢体，其生长隐匿，局部复发和转移倾向明显。目前， extraskeletal myxoid chondrosarcoma 通过广泛切除进行治疗，最近的研究支持辅助放疗和新型化疗策略的应用。
使用监测、流行病学和最终结果（SEER）数据库进行回顾性研究，该数据库检索了 2004 年至 2015 年间诊断为 extraskeletal myxoid chondrosarcoma 的病例。评估了人口统计学变量，以及协作分期变量，包括肿瘤大小、转移性疾病、分级和淋巴结受累情况。根据原发性肿瘤的解剖部位对病例进行分层，并根据治疗干预进行描述。多变量 Cox 比例风险模型评估了不良生存的预测因素，Kaplan-Meier 分析评估了各种分期、人口统计学和治疗变量对总生存的影响。
本研究共回顾了 270 例 extraskeletal myxoid chondrosarcoma 病例，这些病例最常发生在老年男性的下肢或臀部。5 年总生存率为 76.5%，在单因素评估中，年龄>60 岁、组织学分级高、骨盆位置、肿瘤大小>8.0cm、有转移或淋巴结扩散以及未接受手术治疗的患者预后较差。Cox 回归预测年龄较大、肿瘤较大、未手术状态和高肿瘤分级与显著较差的生存相关。多因素评估中，转移并未显著预测生存较差，而在该队列中，化疗和放疗均不能显著改善生存。
作为一种罕见的软组织肉瘤， extraskeletal myxoid chondrosarcoma 的许多表现特征和生存结果由于该疾病的患病率较低而仍未得到明确界定。本研究的结果表明，总体生存率可能比以前报道的更差，不良预后因素与其他软组织肉瘤（包括组织学分级高、年龄较大、肿瘤较大和广泛切除不足）相关的不良预后因素相同。放疗和化疗未能明显改善局限性或转移性疾病患者的生存。
