A rosette-forming glioneuronal tumor of the fourth ventricle - Infratentorial form of dysembryoplastic neuroepithelial tumor?
A rosette-forming glioneuronal tumor of the fourth ventricle - Infratentorial form of dysembryoplastic neuroepithelial tumor?
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DOI：
10.1097/00000478-200205000-00004
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发表时间：
2002-05-01
影响因子：
5.6
通讯作者：
Hirose, T
中科院分区：
文献类型：
--
作者：
Komori, T;Scheithauer, BW;Hirose, T
本文报告11例后颅窝肿瘤，患者年龄12-59岁，临床表现为头痛和/或共济失调。神经影像学检查显示一个相对离散的局灶性强化肿块，主要累及导水管、第四脑室和小脑蚓部。7例患者出现脑积水，2例为多中心病变。在两个病例中，记录了肿瘤大小的显著增加。全切除或次全切除10例。1例仅行活检，另1例接受术后放疗。组织学上，两种成分Acre在所有病例中均被识别。一种由神经细胞组成，形成神经细胞和/或血管周围的假卵巢，部分微囊基质。第二，星形细胞成分类似于10例毛细胞型星形细胞瘤，由纤维化的梭形细胞组成，核呈卵圆形，偶见Rosenthal纤维、颗粒体、肾小球样毛细血管和微钙化。在区域上，这一成分更为弥漫和无模式，由圆形至椭圆形的少突胶质细胞样细胞组成。4例可见少见的神经节细胞。梭形细胞呈突触素和MAP-2免疫反应阳性，而梭形细胞呈S-100蛋白和胶质细胞酸性蛋白阳性。总的来说，细胞分裂是最小的，没有发现有丝分裂，Ki 67标记指数低。超微结构上，神经细胞的突起含有微管，偶尔还有致密的核心颗粒。在所研究的四个病例中的一个中发现了成熟的突触。虽然这种独特的肿瘤的组织学特征表面上类似于胚胎发育不良性神经上皮肿瘤，玫瑰花结形成的神经元细胞，经常出现的毛细胞性星形细胞瘤的成分，和不断增长的病变的性质反对的诊断，偶尔多灶性。
Eleven cases of a distinctive tumor of the posterior fossa are described, The patients (age range 12-59 years) presented with headache and/or ataxia. Neuroimaging revealed a relatively discrete, focally enhancing mass(es) primarily involving the aqueduct, fourth ventricle, and cerebellar vermis. Hydrocephalus was present in seven cases, and two lesions were multicentric. In two cases a significant increase in tumor size was documented. Gross total or subtotal resections were achieved in 10 cases. One patient underwent biopsy alone and another received postoperative irradiation, Histologically, two components Acre identified in all cases. One consisted of neurocytes forming neurocytic and/or perivascular pseudorosettes in a fibrillary, partly microcystic matrix. The second, astrocytic component resembled pilocytie astrocytoma in 10 cases and consisted of fibrillated spindle cells With oval nuclei associated with occasional Rosenthal fibers, granular bodies, glomeruloid capillaries, and microcalcifications. Regionally, this component was more diffuse and patternless, consisting of sheets of round to oval, oligodendrocyte-like cells. Rare ganglion cells Were seen in four cases. The rosettes were consistently synaptophysin and MAP-2 immunoreactive, whereas the spindle cells were positive for S-100 protein and glial fibrillary acidic protein. Overall, atypia was minimal; no mitoses were found, and Ki67 labeling indices were low. Ultrastructurally, the neurocytic cells featured processes containing microtubules and occasional dense core granules. Mature synapses were found in one of the four cases studied. Although the histologic features of this unique tumor superficially resemble those of dysembryoplastic neuroepithelial tumor, rosette formation by neuronal cells, the frequent presence of a pilocytic astrocytoma component, and the growing nature of the lesion argue against that diagnosis, as does occasional multifocality.