Type:
Educational Exhibit
Keywords:
Neuroradiology brain, Neuroradiology peripheral nerve, Neuroradiology spine, CT, MR, Imaging sequences, Image verification
Authors:
M. Kanoto, Y. Toyoguchi, A. Oda, T. Hosoya; Yamagata/JP
DOI:
10.1594/ecr2013/C-0765
Background
Atypical teratoid/ rhabdoid tumor (AT/RT) is a rare tumor occurring in less than 3 years old children.
At first,
this tumor had been reported as the pediatric renal tumor and,
afterward,
was reported as the pediatric central nervous system tumor,
AT/RT which was different from the primitive neuroectodermal tumor (PNET).
The pathological features are proliferation of rhabdoid cells and multiple systemic marker positive.
Especially,
most characteristic pathological feature is the frequent deletion and/or mutation of hSNF5/INI-1 gene in 22nd chromosome (22q11).
The prognosis is very poor (mean survival period; approximately 6 months).
AT/RT in more than 3 years old rarely occurs and adult onset AT/RT is especially extremely rare.
Therefore,
the systematic review on AT/RT has never been performed so far although several cases have been reported.
We have experienced five AT/RT cases so far.
However,
of five cases,
four cases are adult onset AT/RT ones in single institute.
Our purpose is to clarify the image findings of adult AT/RTs.