A case of atypical teratoid/rhabdoid tumor in an adult, with long survival

Kenta Takahashi, Hiroshi Nishihara, Masahito Katoh, Tomoaki Yoshinaga, Roshan Mahabir, Hiromi Kanno, Taichi Kimura, Mishie Tanino, Jun Ikeda, Yutaka Sawamura, Kazuo Nagashima, Shinya Tanaka

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27 Citations (Scopus)

Abstract

Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant tumor that mostly occurs in early childhood and has poor prognosis despite aggressive therapy. Adult cases are rare and, as far as we are aware, only 30 cases have been reported to date. Here we present the case of a 27-year-old female with left parietal AT/RT with the chief complaint of numbness of the right superior limb. First, the tumor was surgically removed and the diagnosis was grade II glioma. With additional radiotherapy, the clinical course after surgery was favorable. After 6 years, she had an operation for recurrence and the diagnosis was grade III glioma. Temozolomide was prescribed, and a disease-free period of 2 years followed. Surgery was performed for a third time for second recurrence with histology of diffuse growth of rhabdoid cells. Immunohistochemistry was partially positive for vimentin and epithelial membrane antigen. Ki-67 labeling index was extremely high and tumor cells showed no staining of INI1 suggestive of diagnosis of AT/RT. We re-evaluated past specimens and none had immunoreactivity of INI1. Ki-67 labeling index and O-6 methylguanine DNA methyltransferase (MGMT) staining were also re-examined and both increased gradually. She is still alive without recurrence for more than 1 year. As far as we are aware, this is the second longest survival of an adult with AT/RT.

Original languageEnglish
Pages (from-to)71-76
Number of pages6
JournalBrain tumor pathology
Volume28
Issue number1
DOIs
Publication statusPublished - 2011 Feb 1
Externally publishedYes

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Keywords

  • Adult
  • Atypical teratoid/rhabdoid tumor
  • INI1
  • Immunohistochemistry
  • Long survival
  • O-6 methylguanine DNA methyltransferase

ASJC Scopus subject areas

  • Oncology
  • Clinical Neurology
  • Cancer Research

Cite this

Takahashi, K., Nishihara, H., Katoh, M., Yoshinaga, T., Mahabir, R., Kanno, H., Kimura, T., Tanino, M., Ikeda, J., Sawamura, Y., Nagashima, K., & Tanaka, S. (2011). A case of atypical teratoid/rhabdoid tumor in an adult, with long survival. Brain tumor pathology, 28(1), 71-76. https://doi.org/10.1007/s10014-010-0008-y