Department of Neurosurgery, Kohsei Chuo General Hospital, 1-11-7 Mita, Meguro-Ku, Tokyo, 153-0062, Japan.
Department of Neurosurgery, Tokyo Medical University, Tokyo, Japan.
Brain Tumor Pathol. 2023 Apr;40(2):78-84. doi: 10.1007/s10014-023-00454-9. Epub 2023 Apr 6.
Gliomatosis cerebri (GC) is a unique glial tumor that extensively invades the cerebral white matter and has been recognized as an entity of neuroepithelial tumors since the first edition of the WHO classification of brain tumors in 1979. Thereafter, in the fourth edition of the WHO classification in 2007, it was clearly defined as a specific type of astrocytic tumor. However, in the WHO 2016 classification, which was based on the concept of integrated diagnosis using molecular genetics, GC was deleted as it was considered to be only one growth pattern of diffuse glioma and not a specific pathological entity. Since then, there has been criticism by many neuro-oncologists and the establishment of the GC working group at the NIH, and many activities in the world arguing that GC should not be deleted from the clinical discussion of brain tumors. In Japan, positive activities toward multicenter research on GC pathology should be performed, and molecular pathological evidence that can contribute to the WHO classification in the future should be developed. In this article, the author outlined the pathological characteristics of GC, which has been repeated changing since its conception, and also describes his opinion on GC as a neuro-oncologist.
脑胶质瘤病(Gliomatosis cerebri,GC)是一种独特的胶质肿瘤,广泛浸润脑白质,自 1979 年世界卫生组织(World Health Organization,WHO)首次发布脑肿瘤分类以来,一直被认为是神经上皮肿瘤的一种实体类型。此后,在 2007 年发布的第四版 WHO 分类中,它被明确定义为一种特定类型的星形细胞瘤。然而,在 2016 年基于分子遗传学综合诊断概念的 WHO 分类中,GC 被删除,因为它被认为只是弥漫性胶质瘤的一种生长模式,而不是一种特定的病理实体。此后,许多神经肿瘤学家提出了批评,并在美国国立卫生研究院(National Institutes of Health,NIH)成立了 GC 工作组,许多国际活动也认为 GC 不应该从脑肿瘤的临床讨论中删除。在日本,应该积极开展针对 GC 病理学的多中心研究,并开发有助于未来 WHO 分类的分子病理证据。本文概述了 GC 的病理特征,它自概念提出以来一直在不断变化,并描述了作者作为神经肿瘤学家对 GC 的看法。