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

Donald Ming Tak Ho, Chih Yi Hsu, Tai-Tong Wong, Ling Tan Ting, Hung Chiang

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112 Citations (Scopus)


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).

Original languageEnglish
Pages (from-to)482-488
Number of pages7
JournalActa Neuropathologica
Issue number5
Publication statusPublished - Jan 1 2000
Externally publishedYes


  • Atypical terotoid/rhabdoid tumor
  • Medulloblastoma
  • Primitive neuroectodermal tumor
  • Rhabdoid tumor

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Cellular and Molecular Neuroscience


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