Li Zhibin, Zhao Liyan, Liu Hui, Zhao Yao, Han Xuefei, Wang Yubo, Li Yunqian
Department of Neurosurgery, The First Hospital of Jilin University, Changchun, China.
Department of Clinical Laboratory, The Second Hospital of Jilin University, Changchun, China.
Neurosurg Rev. 2025 Jan 20;48(1):65. doi: 10.1007/s10143-025-03214-9.
Atypical teratoid rhabdoid tumor (AT/RT) is a rare embryonal central nervous system tumor with a dismal prognosis that occurs mostly in early childhood. Since recent epidemiological and prognostic information is limited, we aimed to describe and analyze AT/RT-related incidences, temporal trends and prognostic factors. Incidence and survival data between 2001 and 2021 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. Descriptive epidemiology was utilized to assess incidences and rate ratios. Temporal trends were assessed by Joinpoint analysis. Prognostic factors were evaluated by survival analysis. A total of 393 patients were included in our analysis. Most patients developed tumors at less than 1 year of age (33.6%). The overall incidence was 0.084 per 100,000 population. According to the SEER 17 data, AT/RT represented 18.3% of all CNS malignant tumors diagnosed in infants aged < 1 year, with a relatively high incidence of 0.583. The overall incidence trend increased nonsignificantly from 2004 to 2021 (P = 0.572). Older age, more aggressive tumor resection, radiotherapy and chemotherapy administration, and nonmetastasis status were related to improved overall survival (OS) and cancer-specific survival (CSS) via Kaplan-Meier analysis. Multivariable Cox regression analysis indicated that age, extent of resection, radiotherapy, chemotherapy and metastasis status were independent prognostic factors for OS and CSS. AT/RT, particularly in infants under 1 year of age, should receive increased attention from epidemiologists and clinicians. An aggressive multimodal regimen including surgery combined with chemotherapy and radiotherapy is necessary.
非典型畸胎样横纹肌样瘤（AT/RT）是一种罕见的胚胎性中枢神经系统肿瘤，预后较差，主要发生于幼儿期。由于近期的流行病学和预后信息有限，我们旨在描述和分析与AT/RT相关的发病率、时间趋势和预后因素。从监测、流行病学和最终结果（SEER）数据库中提取了2001年至2021年的发病率和生存数据。采用描述性流行病学方法评估发病率和率比。通过Joinpoint分析评估时间趋势。通过生存分析评估预后因素。我们的分析共纳入393例患者。大多数患者在1岁以下发病（33.6%）。总体发病率为每10万人0.084例。根据SEER 17数据，AT/RT占1岁以下婴儿诊断出的所有中枢神经系统恶性肿瘤的18.3%，发病率相对较高，为0.583。2004年至2021年总体发病率趋势无显著增加（P = 0.572）。通过Kaplan-Meier分析，年龄较大、更积极的肿瘤切除、放疗和化疗的应用以及无转移状态与总体生存率（OS）和癌症特异性生存率（CSS）的改善相关。多变量Cox回归分析表明，年龄、切除范围、放疗、化疗和转移状态是OS和CSS的独立预后因素。AT/RT，尤其是1岁以下的婴儿，应受到流行病学家和临床医生更多的关注。包括手术联合化疗和放疗在内的积极多模式治疗方案是必要的。
