Otani Ryohei, Uzuka Takeo, Ueki Keisuke
Department of Neurologic Surgery, Dokkyo Medical University, Mibu, Tochigi.
Department of Neurologic Surgery, Dokkyo Medical University, Mibu, Tochigi
Jpn J Clin Oncol. 2017 Jan;47(1):2-6. doi: 10.1093/jjco/hyw142. Epub 2016 Oct 31.
Classification of gliomas, first established by Cushing and Bailey in early 20th century, has been based on histological features that were associated with clinical behavior of the tumor fairly well. However, inter-observer variation in the diagnosis and heterogeneous clinical outcome within a single entity have been problematic in some cases. Accumulation of molecular information of gliomas over the past two to three decades gradually elucidated the mechanism of oncogenesis and progression of gliomas at the molecular level, and it now appears to be possible to classify gliomas by the molecular markers, especially in adult diffuse gliomas that constitute ~25-30% of the primary intracranial tumors. Most powerful molecular markers to classify those tumors are those that appear to be involved in the early phases of oncogenesis, including IDH1/2, TP53, TERT, ATRX and 1p/19q co-deletion. Interesting tight negative and positive correlations among those molecular genetic alterations enable clearer definition of entities and better prognosis prediction in adult diffuse gliomas.
胶质瘤的分类最早由库欣（Cushing）和贝利（Bailey）在20世纪初确立，其依据的组织学特征与肿瘤的临床行为相当吻合。然而，在某些情况下，观察者之间诊断的差异以及单一类型内临床结果的异质性一直是个问题。在过去二三十年里，胶质瘤分子信息的积累逐渐在分子水平上阐明了胶质瘤的发生和进展机制，现在似乎可以通过分子标志物对胶质瘤进行分类，尤其是在占原发性颅内肿瘤约25% - 30%的成人弥漫性胶质瘤中。用于分类这些肿瘤的最有力分子标志物是那些似乎参与肿瘤发生早期阶段的标志物，包括异柠檬酸脱氢酶1/2（IDH1/2）、肿瘤蛋白53（TP53）、端粒酶逆转录酶（TERT）、α地中海贫血/智力低下综合征X连锁基因（ATRX）以及1号染色体短臂/19号染色体长臂共缺失（1p/19q co-deletion）。这些分子遗传改变之间有趣的紧密负相关和正相关关系，使得成人弥漫性胶质瘤的实体定义更加清晰，预后预测也更加准确。
