Papillary tumor of the pineal region
Papillary tumor of the pineal region
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DOI：
10.1097/00000478-200304000-00011
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发表时间：
2003-04-01
影响因子：
5.6
通讯作者：
Fevre-Montange, M
中科院分区：
文献类型：
--
作者：
Jouvet, A;Fauchon, F;Fevre-Montange, M
摘要原发性乳头状肿瘤在中枢神经系统是罕见的。我们遇到了一系列的六个乳头状肿瘤的松果体区与独特的特点，似乎代表了一个临床病理实体。肿瘤发生在4名女性和2名男性中，年龄从19岁到53岁不等。影像学检查显示在松果体区有一个大的边界清楚的肿块。肿瘤的特征是上皮样生长模式，其中血管被一层肿瘤细胞覆盖。在乳头状区，肿瘤细胞较大，呈柱状或立方状，胞浆透明。细胞核圆形或内折，多位于肿瘤细胞的基底极。免疫组化显示肿瘤细胞角蛋白、S-I 00蛋白、神经元特异性烯醇化酶和波形蛋白染色较强，而上皮膜抗原和胶质细胞酸性蛋白染色较弱或不染色。超微结构检查发现两例丰富的粗面内质网与扩张的池充满分泌产物，微绒毛和核周中间丝。松果体区乳头状肿瘤的形态功能特征与连合下器室管膜细胞相似，在本系列中非常一致，松果体区乳头状肿瘤可能来源于这些特化的室管膜细胞。
Primary papillary tumors of the central nervous system are rare. We have encountered a series of six papillary tumors of the pineal region with distinctive features that appear to represent a clinicopathologic entity. The tumors occurred in four women and two men, ranging in age from 19 to 53 years. Imaging studies showed a large well-circumscribed mass in the pineal region. The tumors were characterized by an epithelial-like growth pattern, in which the vessels were covered by a layer of tumoral cells. In papillary areas, the neoplastic cells were large, columnar or cuboidal, with a clear cytoplasm. Nuclei, round or infolded, were found generally at the basal pole of tumoral cells. Immunohistochemically, the tumor cells showed strong staining for cytokeratin, S-I 00 protein, neuron-specific enolase, and vimentin but only weak or no staining for epithelial membrane antigen and glial fibrillary acid protein. Ultrastructural examination of two cases revealed abundant rough endoplasmic reticulum with distended cisternae filled with secretory product, microvilli, and perinuclear intermediate filaments. The morphofunctional features of these papillary tumors of the pineal region, remarkably uniform within this series, are similar to those described for ependymal cells of the subcommissural organ, and the papillary tumors of the pineal region may be derived from these specialized ependymocytes.