Gielen Gerrit H, Baugh Joshua N, van Vuurden Dannis G, Veldhuijzen van Zanten Sophie E M, Hargrave Darren, Massimino Maura, Biassoni Veronica, Morales la Madrid Andres, Karremann Michael, Wiese Maria, Thomale Ulrich, Janssens Geert O, von Bueren André O, Perwein Thomas, Nussbaumer Gunther, Hoving Eelco W, Niehusmann Pitt, Gessi Marco, Kwiecien Robert, Bailey Simon, Pietsch Torsten, Andreiuolo Felipe, Kramm Christof M
Institute of Neuropathology, Medical Center Bonn, Bonn, Germany.
Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Neurooncol Adv. 2022 May 20;4(1):vdac077. doi: 10.1093/noajnl/vdac077. eCollection 2022 Jan-Dec.
The WHO Classification of Tumors of the Central Nervous System has undergone major restructuring. Molecularly defined diagnostic criteria were introduced in 2016 (revised 4th edition) and expanded in 2021 (5th edition) to incorporate further essential diagnostic molecular parameters. We investigated potential differences between specialists in perception of these molecularly defined subtypes for pediatric high-grade gliomas (pedHGG).
We designed a 22-question survey studying the impact of the revised 4th edition of the WHO classification on pedHGG. Data were collected and statistically analyzed to examine the spectrum of viewpoints and possible differences between neuro-oncologists and neuropathologists.
465 participants from 53 countries were included; 187 pediatric neuro-oncologists (40%), 160 neuropathologists (34%), and 118 additional experts (26%). Neuro-oncologists reported issues with the introduction of molecularly defined tumor types, as well as the abolishment or renaming of established tumor entities, while neuropathologists did not to the same extent. Both groups indicated less relevant or insufficient diagnostic definitions were available in 2016. Reported issues were classified and assessed in the 2021 WHO classification and a substantial improvement was perceived. However, issues of high clinical relevance remain to be addressed, including the definition of clinical phenotypes for diffuse intrinsic pontine glioma and gliomatosis cerebri.
Within the WHO classification of pediatric brain tumors, such as pedHGG, rapid changes in molecular characterization have been introduced. This study highlights the ongoing need for cross talk between pathologist and oncologist to advance the classification of pedHGG subtypes and ensure biological relevance and clinical impact.
世界卫生组织中枢神经系统肿瘤分类经历了重大调整。分子定义的诊断标准于2016年（第4版修订版）引入，并于2021年（第5版）扩展，纳入了更多重要的诊断分子参数。我们调查了专家对小儿高级别胶质瘤（pedHGG）这些分子定义亚型认知的潜在差异。
我们设计了一项包含22个问题的调查，研究世界卫生组织第4版修订分类对pedHGG的影响。收集数据并进行统计分析，以考察神经肿瘤学家和神经病理学家的观点范围及可能存在的差异。
纳入了来自53个国家的465名参与者；187名小儿神经肿瘤学家（40%）、160名神经病理学家（34%）和118名其他专家（26%）。神经肿瘤学家报告了分子定义肿瘤类型引入方面的问题，以及既定肿瘤实体的废除或重新命名问题，而神经病理学家在相同程度上未报告此类问题。两组均指出2016年可用的诊断定义相关性较低或不充分。报告的问题在2021年世界卫生组织分类中进行了分类和评估，人们认为有了实质性改进。然而，仍有一些具有高度临床相关性的问题有待解决，包括弥漫性固有脑桥胶质瘤和大脑胶质瘤病临床表型的定义。
在世界卫生组织小儿脑肿瘤分类中，如pedHGG，分子特征方面已引入快速变化。本研究强调病理学家和肿瘤学家之间持续进行交流的必要性，以推进pedHGG亚型的分类，并确保生物学相关性和临床影响。
