Embryonal brain tumor with unknown primary lesion and massive cerebrospinal fluid dissemination: A case report.
Embryonal brain tumor with unknown primary lesion and massive cerebrospinal fluid dissemination: A case report.
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原发灶不明且大量脑脊液播散的胚胎性脑肿瘤：一例报告。
DOI：
10.1016/j.jocn.2018.04.046
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发表时间：
2018
期刊：
影响因子：
--
通讯作者：
Kohmura E.
中科院分区：
文献类型：
--
作者：
Tanaka H;Yamamoto D;Ikeda M;Morikawa M;Ueda K;Tanaka K;Sasayama T;Kohmura E.
2007年世界卫生组织中枢神经系统（CNS）肿瘤分类将CNS胚胎性肿瘤分为三类：成神经管细胞瘤、CNS原始神经外胚层肿瘤和非典型畸胎瘤/横纹肌样瘤。由于缺乏特定的组织学特征，有时很难在病理上准确鉴别中枢神经系统胚胎性肿瘤。钆增强磁共振成像显示在脑脊液空间的巨大病变，这强烈建议脑肿瘤的软脑膜传播。组织学显示肿瘤由密集的小细胞组成，细胞质稀少。免疫反应阳性突触素和嗜铬粒蛋白A，和阴性的胶质细胞酸性蛋白，S-100，EMA，和CD 20。由于肿瘤位于多个部位，且大多数位于脑脊液间隙内，因此无法确定原发病灶。我们诊断此病例为中枢神经系统原始神经外胚叶肿瘤，根据患者年龄及病灶主要分布于幕上。在诱导治疗后，世卫组织于2016年发布了其更新的分类。考虑到诊断为髓母细胞瘤的可能性，我们进行了额外的免疫组化分析，并诊断为第3组髓母细胞瘤，因为表达利钠肽受体3。
The2007 World Health Organization Classification of Tumors of the Central Nervous System(CNS) categorized embryonal tumors of the CNS into three classes: medulloblastoma, CNS primitive neuroectodermal tumor, and atypical teratoid/rhabdoid tumor. Due to the lack of specific histological features, it was sometimes difficult to accurately differentiate CNS embryonal tumors pathologically.Here, we report a case of a young man, who presented with headache. Gadolinium-enhanced magnetic resonance imaging demonstrated massive lesions in the cerebrospinal fluid space, which strongly suggested leptomeningeal dissemination of a brain tumor. The histology showed the tumor comprised densely packed, small cells with scant cytoplasm. Immunoreactivities were positive for synaptophysin and chromogranin A, and negative for glial fibrillary acidic protein, S-100, EMA, and CD20. Because the tumors were located in multiple sites and most of them were within the cerebrospinal fluid space, the primary lesion could not be determined. We diagnosed this case as ‘CNS primitive neuroectodermal tumor’ by the patient age and predominantly supratentorial distribution of the lesions. After the induction therapy, WHO published its updated classification in 2016. Considering the possibility that the diagnosis is medulloblastoma, we performed additional immunohistochemical analyses, and diagnosed Group 3 medulloblastoma because of the expression of natriuretic peptide receptor 3.