We report a rare case of oligodendroglioma with gangliocytic differentiation. A 31-year-old male without a past medical history was admitted with a sudden seizure. On magnetic resonance imaging, an approximately 7-cm mass with necrosis was noted in the right frontal lobe. The patient underwent surgical resection. On microscopy, two morphologically distinct areas with oligodendroglioma- and ganglioglioma-like features were found. Immunohistochemistry showed an absence of CD34 expression, whereas isocitrate dehydrogenase 1 (IDH1) was positive in the glial component. Moreover, IDH1 was positive in the ganglion-like cells as well as in the glial component. Subsequent 1p/19q co-deletion was confirmed by fluorescence in situ hybridization. Finally, a diagnosis of oligodendroglioma with gangliocytic differentiation was made. IDH1/2 molecular test would be basic and essential diagnostic tool in central nervous system tumor of young patients.

中文翻译：

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一名31岁男性中少有神经节细胞分化的少突胶质细胞瘤：IDH1 / 2基因检测的重要性。

我们报告少有神经节细胞分化的少突胶质细胞瘤的情况。一名没有病史的31岁男性因突然发作而入院。在磁共振成像中，在右额叶中发现约7厘米肿块伴坏死。该患者接受了手术切除。在显微镜下，发现了两个形态学不同的区域，具有少突胶质细胞瘤和神经节胶质瘤样特征。免疫组织化学显示不存在CD34表达，而异柠檬酸脱氢酶1（IDH1）在神经胶质成分中呈阳性。此外，IDH1在神经节样细胞以及神经胶质成分中均为阳性。通过荧光原位杂交证实随后的1p / 19q共缺失。最后，诊断为少神经胶质瘤伴神经节细胞分化。