TY - JOUR
T1 - A case of atypical teratoid/rhabdoid tumor in an adult, with long survival
AU - Takahashi, Kenta
AU - Nishihara, Hiroshi
AU - Katoh, Masahito
AU - Yoshinaga, Tomoaki
AU - Mahabir, Roshan
AU - Kanno, Hiromi
AU - Kimura, Taichi
AU - Tanino, Mishie
AU - Ikeda, Jun
AU - Sawamura, Yutaka
AU - Nagashima, Kazuo
AU - Tanaka, Shinya
N1 - Funding Information:
We thank Eiko Aoyanagi, Miho Nodagashira, and Kazuko Shimizu for their excellent technique. This study is supported by the grant from Ministry of Education, Culture, Sports, Science, and Technology, Japan.
PY - 2011/2
Y1 - 2011/2
N2 - 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.
AB - 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.
KW - Adult
KW - Atypical teratoid/rhabdoid tumor
KW - INI1
KW - Immunohistochemistry
KW - Long survival
KW - O-6 methylguanine DNA methyltransferase
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U2 - 10.1007/s10014-010-0008-y
DO - 10.1007/s10014-010-0008-y
M3 - Article
C2 - 21181449
AN - SCOPUS:79951550305
SN - 1433-7398
VL - 28
SP - 71
EP - 76
JO - Brain tumor pathology
JF - Brain tumor pathology
IS - 1
ER -
