IDH mutant diffuse and anaplastic astrocytomas have similar age at presentation and little difference in survival: a grading problem for WHO.
IDH mutant diffuse and anaplastic astrocytomas have similar age at presentation and little difference in survival: a grading problem for WHO.
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IDH突变体弥漫性和变性星形胶质细胞瘤在表现时的年龄相似，而生存的差异很小：谁是谁的评分问题。
DOI：
10.1007/s00401-015-1438-8
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发表时间：
2015-06
影响因子：
12.7
通讯作者：
von Deimling A
中科院分区：
文献类型：
--
作者：
Reuss DE;Mamatjan Y;Schrimpf D;Capper D;Hovestadt V;Kratz A;Sahm F;Koelsche C;Korshunov A;Olar A;Hartmann C;Reijneveld JC;Wesseling P;Unterberg A;Platten M;Wick W;Herold-Mende C;Aldape K;von Deimling A
WHO 2007 年中枢神经系统肿瘤分类区分了弥漫性星形细胞瘤 WHO II 级 (A IIWHO2007) 和间变性星形细胞瘤 WHO III 级 (AA III WHO2007)。与 AA III WHO2007 患者相比，A II WHO2007 患者明显更年轻，存活时间也明显更长。到目前为止，分类和分级仅依赖于形态学基础，尚未考虑 IDH 状态（一种与预后相关的分子标记）。我们在这里证明，当应用于携带 IDH 突变的星形细胞瘤时，WHO 2007 分级在预测预后方面表现不佳。三个独立系列包括总共 1360 个具有 IDH 突变的成人弥漫性星形细胞胶质瘤，其中包含 683 个 A II IDHmut、562 个 AA III IDHmut 和 115 GBM IDHmut 的年龄分布和生存率。在所有三个系列中，患有 A II IDHmut 和 AA III IDHmut 的患者发病时年龄相同（36-37 岁），并且各级别之间的生存期差异比 A II WHO2007 与 AA III WHO2007 报告的生存期差异小得多（A II IDHmut 为 10.9 年，AA III IDHmut 为 9.3 年）。我们的分析表明，A II WHO2007 和 AA III WHO2007 之间年龄和生存率的差异主要取决于队列中 IDH 非突变星形细胞瘤的比例。该数据对当前星形细胞瘤分级和风险分层的实践提出了重大挑战，并可能对 IDH 突变星形细胞瘤患者的治疗产生深远的影响。
The WHO 2007 classification of tumors of the CNS distinguishes between diffuse astrocytoma WHO grade II (A IIWHO2007) and anaplastic astrocytoma WHO grade III (AA III WHO2007). Patients with A II WHO2007 are significantly younger and survive significantly longer than those with AA III WHO2007. So far, classification and grading relies on morphological grounds only and does not yet take into account IDH status, a molecular marker of prognostic relevance. We here demonstrate that WHO 2007 grading performs poorly in predicting prognosis when applied to astrocytoma carrying IDH mutations. Three independent series including a total of 1360 adult diffuse astrocytic gliomas with IDH mutation containing 683 A II IDHmut, 562 AA III IDHmut and 115 GBM IDHmut have been examined for age distribution and survival. In all three series patients with A II IDHmut and AA III IDHmut were of identical age at presentation of disease (36–37 years) and the difference in survival between grades was much less (10.9 years for A II IDHmut, 9.3 years for AA III IDHmut) than that reported for A II WHO2007 versus AA III WHO2007. Our analyses imply that the differences in age and survival between A II WHO2007 and AA III WHO2007 predominantly depend on the fraction of IDH-non-mutant astrocytomas in the cohort. This data poses a substantial challenge for the current practice of astrocytoma grading and risk stratification and is likely to have far-reaching consequences on the management of patients with IDH-mutant astrocytoma.