Virginia Tech Carilion School of Medicine, Roanoke, Virginia, USA.
Carilion Clinic, Section of Neurosurgery, Department of Surgery, Roanoke, Virginia, USA.
Oncology. 2024;102(2):183-194. doi: 10.1159/000533508. Epub 2023 Aug 25.
Atypical teratoid rhabdoid tumor (ATRT) is among the most aggressive central nervous system malignancies. Although rare, this tumor typically afflicts young children and results in mortality within months. Here, we aim to determine key clinical features and treatment options that impact the survival of patients with ATRT.
From the year 2000 to 2019, 363 patients with ATRT were identified from the Surveillance, Epidemiology, and End Results database. Univariate analysis was used to identify variables that had a significant impact on the primary endpoint of overall survival (OS). Multivariable analysis was then used to identify independent predictors of survival.
The median OS of the entire cohort was 13 months. Univariate analysis identified ages between 1 and 3 years, ages between 4 and 17 years, years of diagnosis between 2010 and 2019, and the receipt of treatment to have a significant impact on survival. In multivariable analysis, ages between 1 and 3 years and receipt of treatment were the only significant independent predictors of survival. The median OS was significantly greater in patients who received surgical treatment, chemotherapy, or radiation when compared to those who did not receive any treatment. In general, the receipt of any combination of therapies improved the median OS significantly. The receipt of triple therapy had the greatest impact on survival.
This study highlights the survival benefit of a multimodal approach in the treatment of ATRT. The use of triple therapy, including surgery, radiation, and chemotherapy, was found to have the greatest survival benefit for patients. Overall, these findings may guide future care for patients with ATRT.
非典型畸胎样/横纹肌样瘤（ATRT）是最具侵袭性的中枢神经系统恶性肿瘤之一。尽管罕见，但这种肿瘤通常影响幼儿，并在数月内导致死亡。在这里，我们旨在确定影响 ATRT 患者生存的关键临床特征和治疗选择。
从 2000 年到 2019 年，从监测、流行病学和最终结果数据库中确定了 363 例 ATRT 患者。单变量分析用于确定对总生存期（OS）主要终点有显著影响的变量。然后，多变量分析用于确定生存的独立预测因素。
整个队列的中位 OS 为 13 个月。单变量分析确定年龄在 1 至 3 岁、年龄在 4 至 17 岁、诊断年份在 2010 年至 2019 年之间，以及接受治疗对生存有显著影响。在多变量分析中，年龄在 1 至 3 岁和接受治疗是生存的唯一显著独立预测因素。与未接受任何治疗的患者相比，接受手术治疗、化疗或放疗的患者中位 OS 显著更高。一般来说，任何联合治疗的接受都显著提高了中位 OS。接受三联疗法对生存的影响最大。
本研究强调了多模式治疗在 ATRT 治疗中的生存获益。发现使用包括手术、放疗和化疗在内的三联疗法对患者的生存获益最大。总体而言，这些发现可能为 ATRT 患者的未来护理提供指导。
