Wang Harvey Y, Cogua Laura M, Tupper Connor J, Silberstein Peter T
College of Medicine, Creighton University School of Medicine, Phoenix, USA.
Department of Orthopaedic Surgery, Hofstra University/Northwell Health (Zucker) School of Medicine, Hempstead, USA.
Cureus. 2025 Jun 9;17(6):e85606. doi: 10.7759/cureus.85606. eCollection 2025 Jun.
Background Clear cell sarcoma (CCS) is a rare soft tissue cancer that predominantly affects young to middle-aged adults. Current literature lacks recent and accurate estimates of patient outcomes due to the disease's low incidence and the small sample sizes in existing studies. This study aims to examine the incidence and survival of patients with CCS. Methods Patients from the Surveillance, Epidemiology, and End Results (SEER) database diagnosed with CCS between 2000 and 2019 were selected. Additional variables collected included age, sex, race, stage, metastases, tumor size, treatment status for surgery, radiation, and chemotherapy, median household income, and population size. Descriptive statistics, population-based incidence, chi-square tests, and Cox regression analyses were performed. Results A total of 268 patients were included. The population-adjusted incidence ranged from 0.012/100,000 to 0.027/100,000. The total percent change over the study period was 16.751%, and the annual percent change was 0.561%. The survival rates at one, three, and five years were 78.4%, 62.0%, and 57.1%, respectively. Cox regression results showed that Black patients (p = 0.007), tumor size greater than 4.0 cm (p = 0.033), and the presence of metastases (p = 0.040) were all associated with shorter survival. Significance The findings showed that CCS incidence has remained unchanged in recent years and that prognosis is poor. Black patients were found to have shorter survival durations. Contrary to prior findings, staging and tumor size were only significantly associated with survival in univariate analyses. Limitations include a small sample size and the constraints of variables available in the SEER database. Nonetheless, future research will benefit from assessing whether race is an independent risk factor for CCS survival and from exploring ways to improve prognosis.
背景 透明细胞肉瘤(CCS)是一种罕见的软组织癌,主要影响年轻至中年成年人。由于该疾病发病率低且现有研究样本量小,当前文献缺乏对患者预后的最新准确估计。本研究旨在探讨CCS患者的发病率和生存率。方法 选取2000年至2019年期间在监测、流行病学和最终结果(SEER)数据库中诊断为CCS的患者。收集的其他变量包括年龄、性别、种族、分期、转移情况、肿瘤大小、手术、放疗和化疗的治疗状态、家庭收入中位数和人口规模。进行描述性统计、基于人群的发病率、卡方检验和Cox回归分析。结果 共纳入268例患者。人群调整后的发病率在0.012/10万至0.027/10万之间。研究期间的总变化百分比为16.751%,年变化百分比为0.561%。1年、3年和5年生存率分别为78.4%、62.0%和57.1%。Cox回归结果显示,黑人患者(p = 0.007)、肿瘤大小大于4.0 cm(p = 0.033)和存在转移(p = 0.040)均与较短的生存期相关。意义 研究结果表明,近年来CCS发病率保持不变,预后较差。发现黑人患者的生存期较短。与先前的研究结果相反,分期和肿瘤大小仅在单变量分析中与生存显著相关。局限性包括样本量小和SEER数据库中可用变量的限制。尽管如此,未来的研究将受益于评估种族是否是CCS生存的独立危险因素以及探索改善预后的方法。