title: Retinoblastoma-like phenotype expressed in medulloblastomas.
abstract: The previous demonstration of rod-opsin and S-antigen (S-Ag), a protein which arrests visual phototransduction, in retinoblastomas and in a subgroup of medulloblastomas has suggested a relationship between these tumors. We examined 17 medulloblastomas for the presence of a retinoblastoma-like phenotype. Overall 41% of the tumors were immunoreactive for S-Ag. Two tumors with well-differentiated Flexner-Wintersteiner rosettes were also immunoreactive for S-Ag, but not for epithelial membrane antigen (EMA). In contrast, most ependymal rosettes in two ependymomas stained positive for EMA along the luminal surface, consistent with a previous study, and were negative for S-Ag. Because calcification in areas of necrosis is a near constant finding in retinoblastomas, the medulloblastomas were evaluated for the presence of calcification, using Von Kossa staining. Forty-one percent showed calcification in areas of necrosis and 29% were positive for both calcification and S-Ag immunoreactivity. There was a statistically significant concordance between calcification and S-Ag immunoreactivity in the medulloblastomas (p < 0.05). Despite similar phenotypic features, a shared mechanism of tumori-genesis for retinoblastomas and the subgroup of medulloblastomas with photoreceptor differentiation could not be identified since all 17 medulloblastomas were found to express functional Rb protein, as indicated by positive nuclear immunoreactivity.
authors.affiliation: Department of Pathology, University of Texas Medical Branch, Galveston, USA.
authors.forename: P B
authors.initials: PB
authors.lastname: Jaffey
authors.forename: G T
authors.initials: GT
authors.lastname: To
authors.forename: H J
authors.initials: HJ
authors.lastname: Xu
authors.forename: S X
authors.initials: SX
authors.lastname: Hu
authors.forename: W F
authors.initials: WF
authors.lastname: Benedict
authors.forename: L A
authors.initials: LA
authors.lastname: Donoso
authors.forename: G A
authors.initials: GA
authors.lastname: Campbell
mesh_terms: D000293:Adolescent; D000328:Adult; D000941:Antigens / Q000032:analysis; D019393:Arrestin; D002114:Calcinosis; D002528:Cerebellar Neoplasms / Q000235:genetics / Q000473:pathology* / Q000648:ultrastructure; D002648:Child; D002675:Child, Preschool; D005134:Eye Neoplasms / Q000235:genetics / Q000473:pathology*; D005136:Eye Proteins / Q000032:analysis; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008297:Male; D008527:Medulloblastoma / Q000235:genetics / Q000473:pathology* / Q000648:ultrastructure; D009336:Necrosis; D010641:Phenotype; D012175:Retinoblastoma / Q000235:genetics / Q000473:pathology*; D016160:Retinoblastoma Protein / Q000032:analysis; D012177:Retinol-Binding Proteins / Q000032:analysis; D017299:Rod Opsins / Q000032:analysis; D016708:Synaptophysin / Q000032:analysis; D000099214:Retinol-Binding Proteins, Interstitial
chemical_list: D000941:Antigens; D019393:Arrestin; D005136:Eye Proteins; D005904:Glial Fibrillary Acidic Protein; D016160:Retinoblastoma Protein; D012177:Retinol-Binding Proteins; D017299:Rod Opsins; D016708:Synaptophysin; D000099214:Retinol-Binding Proteins, Interstitial