Department of Neurosurgery, Nagoya University School of Medicine, 65 Tsurumai-Cho, Showa-Ku, Nagoya, 466-8550, Japan.
Brain Tumor Pathol. 2023 Apr;40(2):48-55. doi: 10.1007/s10014-023-00458-5. Epub 2023 Mar 29.
The 2021 World Health Organization (WHO) classification of central nervous system tumors applied molecular criteria and further integrated histological and molecular diagnosis of gliomas. This classification allows for the diagnosis of isocitrate dehydrogenase wild-type (IDHwt) glioblastoma (GBM), and WHO grade 4 with histologically lower-grade gliomas (LrGGs), even in the absence of high-grade histopathologic features, such as necrosis and/or microvascular proliferation. They contain at least one of the following molecular features: epidermal growth factor receptor amplification, chromosome 7 gain/10 loss, or telomerase reverse transcriptase promoter mutation. In the imaging features at the time of histological diagnosis, a gliomatosis cerebri growth pattern was frequently observed in these tumors. Furthermore, this growth pattern was significantly higher in IDHwt GBM, WHO grade 4, with histological grade II gliomas. Although the exact prognosis of IDHwt GBM, WHO grade 4, with histologically LGGs remains unknown, its OS was approximately 1-2 years similar to that of histologically IDHwt GBM, WHO grade 4, despite histopathological features similar to IDHmut LrGGs. These findings reinforce the need for the analysis of molecular features, regardless of presenting similar clinical characteristics and imaging features to IDHmut LrGGs.
2021 年世界卫生组织(WHO)中枢神经系统肿瘤分类采用了分子标准,并进一步整合了胶质瘤的组织学和分子诊断。这种分类允许诊断异柠檬酸脱氢酶野生型(IDHwt)胶质母细胞瘤(GBM)和组织学低级别胶质瘤(LrGGs)的 WHO 分级 4,即使在没有高级别组织病理学特征(如坏死和/或微血管增生)的情况下也是如此。它们至少具有以下分子特征之一:表皮生长因子受体扩增、染色体 7 获得/10 缺失或端粒酶逆转录酶启动子突变。在组织学诊断时的影像学特征中,这些肿瘤中经常观察到弥漫性脑胶质瘤生长模式。此外,在 IDHwt GBM、WHO 分级 4 和组织学 II 级胶质瘤中,这种生长模式明显更高。尽管 IDHwt GBM、WHO 分级 4 和组织学 LGGs 的确切预后仍然未知,但它的 OS 与组织学 IDHwt GBM、WHO 分级 4 相似,约为 1-2 年,尽管其组织病理学特征类似于 IDHmut LrGGs。这些发现强调了无论与 IDHmut LrGGs 具有相似的临床特征和影像学特征,都需要分析分子特征。