Szpak G M, Papierz W, Liberski P P, Kulczycki J, Kryst-Widźgowska T, Dymecki J
Department of Neuropathology, Institute of Psychiatry and Neurology, Warszawa.
Folia Neuropathol. 1995;33(1):35-40.
The authors present a case of relatively rare tumor of the central nervous system (CNS) in a 19-year-old female, who died 18 months after the first manifestation of meningismus, increased intracranial pressure and secondary hydrocephalus. Brain autopsy revealed abundant neoplastic infiltrations, which spread through the subarachnoid space. Neoplastic infiltrations were also present in the third ventricle and in a form of small subependymal nodules along the whole ventricular system. The microscopical examination showed that neoplasm consisted of small cells, which formed neuroblastic Homer Wright rosettes. Immunohistochemical studies (for synaptophysin, chromogranin A, GFAP, vimentin) together with morphology and localization of neoplasm suggested diagnosis of primitive neuroectodermal tumor (PNET) that spread mainly in the leptomeninges and caused obliteration of subarachnoid space.
作者报告了一例19岁女性相对罕见的中枢神经系统（CNS）肿瘤病例，该患者在首次出现脑膜炎症状、颅内压升高和继发性脑积水18个月后死亡。脑部尸检发现大量肿瘤浸润，这些浸润通过蛛网膜下腔扩散。第三脑室以及沿整个脑室系统存在呈小室管膜下结节形式的肿瘤浸润。显微镜检查显示肿瘤由小细胞组成，这些小细胞形成神经母细胞性霍纳·赖特菊形团。免疫组织化学研究（针对突触素、嗜铬粒蛋白A、胶质纤维酸性蛋白、波形蛋白）以及肿瘤的形态和定位提示诊断为主要在软脑膜扩散并导致蛛网膜下腔闭塞的原始神经外胚层肿瘤（PNET）。
