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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Melanocytic medulloblastoma with ganglioneurocytomatous differentiation: a case report.
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Melanocytic medulloblastoma with ganglioneurocytomatous differentiation: a case report.

机译：具有神经节神经细胞瘤分化的黑素细胞性髓母细胞瘤：1例。

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摘要

Melanotic or melanocytic medulloblastoma is a rare variant of medulloblastoma, especially when the tumor shows advanced neuronal differentiation. We report a case of this tumor, which developed in the cerebellar vermis in an 8-year-old girl. Initial biopsy specimens were identified as classical medulloblastoma with a high MIB1 index. Surgical removal of the tumor was performed after chemo-radiotherapy, and black pigments were noticed on the tumor surface. Histologically, the tumor was composed of classical medulloblastoma with the presence of pigmented epithelial cells forming tubules and clusters. Immunohistochemically, the pigmented tumor cells were positive for S100 protein, HMB45, and MART1, indicating that the pigments were derived from melanosomes, and these features were compatible with melanocytic medulloblastoma. Interestingly, some of the non-pigmented or amelanotic tumor cells were also positive for HMB45 and S100 protein. Although the tumor showed an unusual cell combination, it was distinguished from atypical teratoid/rhabdoid tumor (AT/RT) by nuclear expression of INI1/BAF45 protein. The tumor also possessed ganglion-like cells within the neuropil matrix, which resembled small mature ganglion cells, and was consequently designated as ganglioneurocytoma. The melanotic medulloblastoma and part of the ganglioneurocytomatous area were fused with each other. Hence, the present case provides new information indicating that melanocytic medulloblastoma differs from AT/RT, and that it can exhibit advanced neuronal differentiation. In addition, reduction of the tumor MIB1 index was observed after chemo-radiotherapy.
机译：黑色素细胞或黑色素细胞性髓母细胞瘤是髓母细胞瘤的罕见变体，尤其是当肿瘤显示晚期神经元分化时。我们报告了此肿瘤的病例，该肿瘤在一个8岁女孩的小脑ver部发育。最初的活检标本被鉴定为具有高MIB1指数的经典髓母细胞瘤。化学放疗后手术切除肿瘤，并在肿瘤表面发现黑色素。从组织学上讲，该肿瘤由经典的髓母细胞瘤组成，有色素上皮细胞形成小管和簇。免疫组织化学观察，色素沉着的肿瘤细胞对S100蛋白，HMB45和MART1呈阳性，表明这些色素来源于黑素体，并且这些特征与黑素细胞髓母细胞瘤相容。有趣的是，一些非色素或搪瓷肿瘤细胞也对HMB45和S100蛋白呈阳性。尽管该肿瘤显示出异常的细胞组合，但通过核表达INI1 / BAF45蛋白将其与非典型类畸形/类人瘤（AT / RT）区分开。该肿瘤在神经纤维基质内还具有神经节样细胞，类似于小的成熟神经节细胞，因此被称为神经节神经细胞瘤。黑色素神经母细胞瘤和部分神经节神经细胞瘤区域融合在一起。因此，本案提供了新的信息，表明黑素细胞性髓母细胞瘤不同于AT / RT，并且可以表现出晚期神经元分化。另外，化学放射治疗后观察到肿瘤MIB1指数降低。

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