Acta Neuropathologica

, Volume 99, Issue 5, pp 482–488

Atypical teratoid/rhabdoid tumor of the central nervous system: a comparative study with primitive neuroectodermal tumor/medulloblastoma

  • D. M.-T. Ho
  • C.-Y. Hsu
  • T.-T. Wong
  • L.-T. Ting
  • H. Chiang
Regular paper

DOI: 10.1007/s004010051149

Cite this article as:
Ho, D., Hsu, C., Wong, T. et al. Acta Neuropathol (2000) 99: 482. doi:10.1007/s004010051149

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).

Key words Atypical terotoid/rhabdoid tumorRhabdoid tumorPrimitive neuroectodermal tumorMedulloblastoma

Copyright information

© Springer-Verlag Berlin Heidelberg 2000

Authors and Affiliations

  • D. M.-T. Ho
    • 1
  • C.-Y. Hsu
    • 1
  • T.-T. Wong
    • 3
  • L.-T. Ting
    • 1
  • H. Chiang
    • 1
  1. 1.Department of Pathology and Laboratory Medicine, Veterans General Hospital-Taipei, 201 Shih-Pai Rd., Sec. 2, Taipei, 11217 Taiwan, R.O.C. e-mail: mtho@vghtpe.gov.tw, Tel.: +886-2-28757448, Fax: +886-2-28757056TW
  2. 2.Department of Pathology, National Yang-Ming University School of Medicine, Taiwan, R.O.C.TW
  3. 3.Division of Pediatric Neurosurgery, Neurological Institute, Veterans General Hospital-Taipei, Taiwan, R.O.C.TW
  4. 4.Department of Surgery, National Yang-Ming University School of Medicine, Taiwan, R.O.C.TW