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具有高级别神经元免疫表型的继发性胶质母细胞瘤。

Secondary glioblastoma with advanced neuronal immunophenotype.

作者信息

Shibahara Junji, Fukayama Masashi

机构信息

Department of Pathology, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, 113-0033 Tokyo, Japan.

出版信息

Virchows Arch. 2005 Sep;447(3):665-8. doi: 10.1007/s00428-005-1263-x. Epub 2005 Jun 21.

Abstract

We describe an unusual progression of astrocytoma into secondary glioblastoma exhibiting advanced neuronal immunophenotype. A tumor of the left frontal lobe of a 35-year-old man was diagnosed as astrocytoma. The tumor was treated by partial removal with postoperative chemoradiotherapy, followed by extensive removal of the residual regrowing tumor 5 month later. A secondary tumor was discovered and partially resected 8 years later, but the patient died 11 months following the operation due to extensive tumor progression showing subarachnoidal and intraventricular dissemination. The secondary tumor was small cell-predominant, highly proliferative tumor with an extremely high MIB-1 labeling index (80%). Unexpectedly, most of the tumor cells were positive for neuronal markers (synaptophysin and NeuN), but not for glial fibrillary acidic protein (GFAP). Retrospective examination of the original tumor revealed not only diffuse GFAP expression, but also neuronal marker expressions in small numbers of tumor cells that were hard to discriminate from the other cells on hematoxylin-eosin (HE) stain. This way of malignant progression of astrocytoma was quite unusual. Although the secondary tumor was classified as glioblastoma according to World Health Organization (WHO) classification (2000), it might be categorized into new variants of malignant glioneuronal tumors proposed recently.

摘要

我们描述了一例星形细胞瘤向具有高级神经元免疫表型的继发性胶质母细胞瘤的不寻常进展。一名35岁男性左额叶肿瘤被诊断为星形细胞瘤。该肿瘤接受了部分切除并术后进行放化疗,5个月后对残留复发肿瘤进行了广泛切除。8年后发现继发性肿瘤并部分切除,但患者术后11个月因肿瘤广泛进展,出现蛛网膜下腔和脑室内播散而死亡。继发性肿瘤以小细胞为主,是具有极高MIB-1标记指数(80%)的高增殖性肿瘤。出乎意料的是,大多数肿瘤细胞神经元标志物(突触素和NeuN)呈阳性,但胶质纤维酸性蛋白(GFAP)呈阴性。对原发肿瘤的回顾性检查不仅发现弥漫性GFAP表达,而且在苏木精-伊红(HE)染色中少数肿瘤细胞中也有神经元标志物表达,这些细胞很难与其他细胞区分开来。星形细胞瘤的这种恶性进展方式非常不寻常。尽管根据世界卫生组织(WHO)分类(2000年)继发性肿瘤被归类为胶质母细胞瘤,但它可能属于最近提出的恶性神经胶质神经元肿瘤的新变体。

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