Nandoliya Khizar R, Sadagopan Nishanth S, Thirunavu Vineeth, Houskamp Ethan J, Karras Constantine L, Chaliparambil Rahul K, Sriram Nikhil, Jamshidi Pouya, Raleigh David R, Lukas Rimas V, Magill Stephen T
Department of Neurological Surgery, Malnati Brain Tumor Institute, Northwestern University, Chicago, IL 60611, USA.
Department of Pathology, Northwestern University, Chicago, IL 60611, USA.
Cancers (Basel). 2023 Jun 27;15(13):3374. doi: 10.3390/cancers15133374.
Most of the literature on pineoblastoma consists of case reports and single-institution series. The goal of this systematic review and individual patient data (IPD) analysis was to summarize the existing literature, identify factors associated with overall survival (OS), and provide a contemporary update on prognosis for patients with pineoblastoma. Forty-four studies were identified with 298 patients having IPD. Kaplan-Meier analyses were used to report survival outcomes based on age, tumor metastases, extent of resection (EOR), adjuvant therapy, and publication year. Cox regression was performed to identify independent predictors of time to mortality. Multivariable recursive partitioning analysis was used to identify the most important subgroups associated with mortality. Patients were classified based on publication year before and after the last systematic review on this topic (pre-2012 and 2012 onwards) and compared using univariate and multivariable analyses. This study demonstrates that EOR less-than-gross total resection, metastatic presentation, adjuvant chemotherapy without radiation, and tumor presentation in children less than three years old are associated with poorer prognosis. Since 2012, the 5-year actuarial OS has improved from 32.8% to 56.1%, which remained significant even after accounting for EOR, age, and adjuvant therapy. Pineoblastoma remains a severe rare disease, but survival outcomes are improving.
大多数关于松果体母细胞瘤的文献都是病例报告和单机构系列研究。本系统评价和个体患者数据（IPD）分析的目的是总结现有文献，确定与总生存期（OS）相关的因素，并提供松果体母细胞瘤患者预后的当代最新情况。共纳入44项研究，其中298例患者有IPD。采用Kaplan-Meier分析报告基于年龄、肿瘤转移、切除范围（EOR）、辅助治疗和发表年份的生存结果。进行Cox回归分析以确定死亡时间的独立预测因素。采用多变量递归划分分析来确定与死亡率相关的最重要亚组。根据关于该主题的最后一次系统评价之前和之后的发表年份（2012年之前和2012年及以后）对患者进行分类，并使用单变量和多变量分析进行比较。本研究表明，EOR小于全切除、转移表现、无放疗的辅助化疗以及3岁以下儿童的肿瘤表现与较差的预后相关。自2012年以来，5年精算OS从32.8%提高到56.1%，即使在考虑EOR、年龄和辅助治疗后，这一改善仍具有显著性。松果体母细胞瘤仍然是一种严重的罕见疾病，但生存结果正在改善。
