title: Papillary tumor of the pineal region.
abstract: 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.
authors.affiliation: Hôpital Neurologique, BP Lyon Montchat, 69394 Lyon Cedex 03, France. Jouvet@laennec.univ-lyon1.fr
authors.forename: Anne
authors.initials: A
authors.lastname: Jouvet
authors.forename: François
authors.initials: F
authors.lastname: Fauchon
authors.forename: Pawel
authors.initials: P
authors.lastname: Liberski
authors.forename: Ghislaine
authors.initials: G
authors.lastname: Saint-Pierre
authors.forename: Marianne
authors.initials: M
authors.lastname: Didier-Bazes
authors.forename: Anna
authors.initials: A
authors.lastname: Heitzmann
authors.forename: Marie-Bernadette
authors.initials: MB
authors.lastname: Delisle
authors.forename: Homa Adle
authors.initials: HA
authors.lastname: Biassette
authors.forename: Sylvie
authors.initials: S
authors.lastname: Vincent
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Mikol
authors.forename: Nathalie
authors.initials: N
authors.lastname: Streichenberger
authors.forename: Samir
authors.initials: S
authors.lastname: Ahboucha
authors.forename: Christine
authors.initials: C
authors.lastname: Brisson
authors.forename: Marie-Françoise
authors.initials: MF
authors.lastname: Belin
authors.forename: Michelle
authors.initials: M
authors.lastname: Fèvre-Montange
mesh_terms: D000328:Adult; D001932:Brain Neoplasms / Q000473:pathology*; D002291:Carcinoma, Papillary / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D010870:Pineal Gland