Kawataki Tomoyuki, Sato Eiji, Sato Takashi, Kinouchi Hiroyuki
Department of Neurosurgery, University of Yamanashi, Faculty of Medicine, Chuo, Yamanashi, Japan.
Neurol Med Chir (Tokyo). 2010;50(3):228-31. doi: 10.2176/nmc.50.228.
A 34-year-old man presented with a case of anaplastic ganglioglioma with malignant features in both neuronal and glial components manifesting as seizure episodes over 11 months. The tumor was subtotally removed, followed by irradiation and chemotherapy. The histological diagnosis was anaplastic ganglioglioma. Atypical cells were morphologically estimated as glial and neuronal cells. Though these cells were weakly positive for synaptophysin and glial fibrillary acidic protein, the neural stem cell marker nestin was extremely expressed in both these cells. The MIB-1 index was 15%. Two months later, the tumor recurred with more pleomorphic appearance and higher cellularity with increased nestin expression level. Mitotic cells and multinucleated cells were found in the neuronal components. Cytological examination found dissemination to the leptomeningeal space. The patient died 6 months after the first surgery. This rare case of anaplastic ganglioglioma with both neuronal and glial components, which were extremely positive for nestin, showed progressive worsening of the clinical course. The expression of nestin may suggest that the origin or malignant transformation in anaplastic gangliogliomas is related to the undifferentiated neural stem cells.
一名34岁男性,患有间变性节细胞胶质瘤,其神经元和胶质成分均具有恶性特征,表现为11个月内反复发作癫痫。肿瘤次全切除,随后进行放疗和化疗。组织学诊断为间变性节细胞胶质瘤。非典型细胞在形态学上被判定为胶质细胞和神经元细胞。尽管这些细胞突触素和胶质纤维酸性蛋白呈弱阳性,但神经干细胞标志物巢蛋白在这两种细胞中均极度表达。MIB-1指数为15%。两个月后,肿瘤复发,外观更具多形性,细胞密度更高,巢蛋白表达水平增加。在神经元成分中发现有丝分裂细胞和多核细胞。细胞学检查发现肿瘤播散至软脑膜间隙。患者在首次手术后6个月死亡。这例罕见的间变性节细胞胶质瘤同时具有神经元和胶质成分,且巢蛋白呈极度阳性,临床病程呈进行性恶化。巢蛋白的表达可能提示间变性节细胞胶质瘤的起源或恶性转化与未分化的神经干细胞有关。