Learning objectives
Our purpose is to clarify the image findings of adult AT/RTs.
The subjects are 4 patients of adult AT/RT (60,
21,
31,
25 y.o.,
Male: Female=1: 1).
CT,
MRI and Angiography were performed for each case and the image findings are retrospectively evaluated.
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...
Imaging findings OR Procedure details
Image Analysis
The subjects are four cases diagnosed as adult onset AT/RT in Yamagata University Hospital from 1999 to 2010 (2 men and 2 women; mean age: 34.3 years old; range: 21–60 years old).
All cases were precisely pathologically diagnosed by biopsy,
operation or autopsy.
We retrospectively evaluated primary sites,
image findings (CT,
MRI and angiography) and survival period.
CT findings were classified into high,
iso or low density.
MRI findings were classified into high,
iso,
low or mixedintensity in each sequence (including DWI,
T1WI,...
Conclusion
Adult onset AT/RT occurs in various sites and showsa variety ofimage findings.
In spite of adult cases,
AT/RTshould be taken into consideration if the tumorshows mixed intensity on T2WI,
high density on CT,
hyperintensity on DWI and strange behavior of the tumor such as markedly rapid growth and metastasis for the other organs.
References
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Ultrastructure of malignant rhabdoid tumor of the kidney.
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Primary malignant rhabdoid tumour of the brain in adults.
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Bison B,
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