title: Atypical teratoid/rhabdoid tumor of the central nervous system: a comparative study with primitive neuroectodermal tumor/medulloblastoma.
abstract: Eleven atypical teratoid/rhabdoid tumors (AT/RT) and 121 primitive neuroectodermal tumors/medulloblastomas (PNET/MB) were included in this study for evaluation of the histopathological features of AT/RT and comparison between AT/RT and PNET/MB. Histopathological studies of AT/RT showed that in addition to the commonly recognized components, i.e., rhabdoid cells, small (PNET/MB) cells, spindle cells and epithelial components, there was a previously unrecognized component, sickle-shaped embracing cells, which were present in all cases and could be useful as a histological marker of this tumor. Immunohistochemical studies showed divergent differentiation of the tumor cells and among the 16 antibodies studied, vimentin, neuron-specific enolase, epithelial membrane antigen and glial fibrillary acidic protein were most commonly reactive. The frequency of AT/RT expressed as a ratio of AT/RT to PNET/MB was 1:11 in general and increased to 1:3.8 among patients younger than 3 years old. The AT/RT patients were younger than those with PNET/MB and had a female predominance. The MIB-1 labeling index of AT/RT was significantly higher than that of PNET/MB (mean 63.9 vs 40.1), which correlated with a shorter survival in patients with AT/RT than those with PNET/MB (median survival time 15.4 months vs 156.4 months).
authors.affiliation: Department of Pathology and Laboratory Medicine, Veterans General Hospital-Taipei, Taiwan, ROC. mtho@vghtpe.gov.tw
authors.forename: D M
authors.initials: DM
authors.lastname: Ho
authors.forename: C Y
authors.initials: CY
authors.lastname: Hsu
authors.forename: T T
authors.initials: TT
authors.lastname: Wong
authors.forename: L T
authors.initials: LT
authors.lastname: Ting
authors.forename: H
authors.initials: H
authors.lastname: Chiang
mesh_terms: D034961:Antigens, Nuclear; D015415:Biomarkers; D001932:Brain Neoplasms / Q000737:chemistry / Q000401:mortality / Q000473:pathology*; D002528:Cerebellar Neoplasms / Q000737:chemistry / Q000401:mortality / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D006801:Humans; D007223:Infant; D019394:Ki-67 Antigen; D008297:Male; D008527:Medulloblastoma / Q000737:chemistry / Q000401:mortality / Q000473:pathology*; D018396:Mucin-1 / Q000032:analysis; D018242:Neuroectodermal Tumors, Primitive / Q000737:chemistry / Q000401:mortality / Q000473:pathology*; D009687:Nuclear Proteins / Q000032:analysis; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D018335:Rhabdoid Tumor / Q000737:chemistry / Q000401:mortality / Q000473:pathology*; D016019:Survival Analysis; D013724:Teratoma / Q000737:chemistry / Q000401:mortality / Q000473:pathology*; D014746:Vimentin / Q000032:analysis
chemical_list: D034961:Antigens, Nuclear; D015415:Biomarkers; D005904:Glial Fibrillary Acidic Protein; D019394:Ki-67 Antigen; D018396:Mucin-1; D009687:Nuclear Proteins; D014746:Vimentin; D010751:Phosphopyruvate Hydratase