Jouvet Anne, Fauchon François, Liberski Pawel, Saint-Pierre Ghislaine, Didier-Bazes Marianne, Heitzmann Anna, Delisle Marie-Bernadette, Biassette Homa Adle, Vincent Sylvie, Mikol Jacqueline, Streichenberger Nathalie, Ahboucha Samir, Brisson Christine, Belin Marie-Françoise, Fèvre-Montange Michelle
Hôpital Neurologique, BP Lyon Montchat, 69394 Lyon Cedex 03, France.
Am J Surg Pathol. 2003 Apr;27(4):505-12. doi: 10.1097/00000478-200304000-00011.
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-100 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.
中枢神经系统原发性乳头状肿瘤很罕见。我们遇到了一系列6例松果体区乳头状肿瘤，其具有独特特征，似乎代表一种临床病理实体。这些肿瘤发生于4名女性和2名男性，年龄范围为19至53岁。影像学研究显示松果体区有一个边界清楚的大肿块。肿瘤的特征为上皮样生长模式，其中血管被一层肿瘤细胞覆盖。在乳头状区域，肿瘤细胞大，呈柱状或立方形，细胞质清晰。细胞核呈圆形或内陷，通常位于肿瘤细胞的基底部。免疫组化显示，肿瘤细胞对细胞角蛋白、S-100蛋白、神经元特异性烯醇化酶和波形蛋白呈强阳性染色，但对上皮膜抗原和胶质纤维酸性蛋白仅呈弱阳性或无染色。对2例病例的超微结构检查显示，有丰富的粗面内质网，其池扩张并充满分泌产物、微绒毛和核周中间丝。本系列中这些松果体区乳头状肿瘤的形态功能特征非常一致，与连合下器官室管膜细胞所描述的特征相似，松果体区乳头状肿瘤可能起源于这些特殊的室管膜细胞。
