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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Rosette-forming glioneuronal tumor of the fourth ventricle with advanced microvascular proliferation--a case report.
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Rosette-forming glioneuronal tumor of the fourth ventricle with advanced microvascular proliferation--a case report.

机译：第四脑室形成玫瑰花结的胶质神经胶质瘤伴晚期微血管增生-病例报告。

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摘要

Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle is a recently described novel type of primary brain tumor that was included into the current WHO classification of CNS tumors. It is a very rare, slowly growing, mixed neoplasm at cerebellar localization with distinctive morphological pattern. We present an unusual case of a 20-year-old patient with RNGT of the fourth ventricle with advanced microvascular proliferation. MRI revealed the solid-cystic tumor mass largely involving the cerebellar vermis and left hemisphere with compression of the fourth ventricle. Microscopically, the tumor showed classical architectural pattern with two distinctive components. The main component consisted of neurocytic rosettes formed by round, isomorphic nuclei arranged around eosinophilic, fibrillar cores with strong synaptophysin expression. The perivascular rosettes with cell arrangement along blood vessels were observed only sporadically. The second neoplastic component consisted of spindle or stellate astroglial cells with piloid process and Rosenthal fibers, strongly resembling pilocytic astrocytoma. Focally, the astroglial cells showed increased cellularity but without marked nuclear atypia. The glial part of the tumor revealed advanced proliferation of microvessels. The vessels of glomeruloid type exhibited multilayered endothelial proliferation and marked mitotic activity. MIB1 labelling index was generally low; however, in areas exhibiting microvascular proliferation its expression was significantly increased up to 20%. This report demonstrates the unique case of RGNT with conspicuous microvascular proliferation of glomeruloid type and extensive endothelial proliferation. As there is still limited clinical experience with RGNT, further studies are necessary to evaluate the biology of this type of tumor.
机译：第四脑室的形成玫瑰花结的神经胶质神经瘤（RGNT）是最近描述的新型原发性脑肿瘤，被纳入当前的WHO中枢神经系统肿瘤分类中。这是一种非常罕见的，缓慢生长的混合肿瘤，位于小脑部位，具有独特的形态学特征。我们提出了一个不寻常的病例，该患者为一名20岁的第四脑室RNGT的晚期微血管增生患者。 MRI显示实体囊性肿瘤肿块主要累及小脑ver部和左半球，并压缩第四脑室。在显微镜下，肿瘤表现出具有两个独特成分的经典建筑模式。主要成分由周围呈嗜酸性的原纤维核心排列的圆形同构核形成的神经细胞玫瑰花结组成，具有强烈的突触素表达。仅偶尔观察到沿细胞排列的沿血管的玫瑰花结。第二个赘生物成分是纺锤状或星状星形胶质细胞，具有倍性过程和罗森塔尔纤维，非常类似于毛细胞星形细胞瘤。星形胶质细胞显示出增加的细胞性，但没有明显的核非典型性。肿瘤的神经胶质部分显示微血管的晚期增殖。肾小球类型的血管表现出多层内皮增殖和明显的有丝分裂活性。 MIB1标记指数普遍较低;然而，在表现出微血管增生的区域，其表达显着增加了高达20％。该报告证明了RGNT的独特情况，其具有肾小球类型的显着微血管增生和广泛的内皮增生。由于RGNT的临床经验仍然有限，因此有必要进行进一步的研究以评估此类肿瘤的生物学特性。

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