Atypical teratoid/rhabdoid tumors of the central nervous system: imaging and clinical findings in 16 children.
OBJECTIVE
To investigate the imaging and clinical findings of central nervous system (CNS) atypical teratoid/rhabdoid tumours (AT/RTs) in children.


METHODS
The computed tomography (CT) and magnetic resonance imaging (MRI) findings and clinical records of 16 children with CNS AT/RTs were retrospectively reviewed. Tumour location, size, composition, enhancement pattern, peritumoural oedema, signal intensity (SI) on MRI and CT attenuation were evaluated.


RESULTS
A total of 17 lesions from 16 patients (median age 2.3 years, age range 0.7-15 years) were included in the evaluation. Tumour location was infratentorial for 11 lesions and supratentorial for six lesions. The mean diameter of the largest dimension for a tumour was 4 cm. The tumour was mainly solid in 65% of cases, and solid and cystic or cystic and solid in 35% of cases. The solid component of the tumours had a homogeneous iso SI (n=15) on T2-weighted MRI images and iso SI (n=14) on T1-weighted images. Moderate to strong enhancement of the solid component was noted in most cases. In spite of a large tumour size, peritumoural oedema was minimal or mild except in four cases. Rapid growth of the tumour was demonstrated in three cases. Seven patients died from tumour progression, with a mean survival time of 8.4 months (range 2-12 months).


CONCLUSIONS
Although the AT/RTs had non-specific imaging findings, the tumours tended to be large in size, have iso SI on T1 and T2-weighted MR images with prominent enhancement, and relatively mild peritumoural oedema. Rapid growth of the tumour was seen during the follow-up period.
Lee
I H
IH
Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
Yoo
S-Y
SY
Kim
J H
JH
Eo
H
H
Kim
O H
OH
Kim
I-O
IO
Cheon
J-E
JE
Jung
A Y
AY
Yoon
B J
BJ
D000293:Adolescent; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007090:Image Interpretation, Computer-Assisted / Q000379:methods; D007223:Infant; D007723:Korea; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D012189:Retrospective Studies; D018335:Rhabdoid Tumor / Q000175:diagnosis* / Q000473:pathology; D014057:Tomography, X-Ray Computed / Q000379:methods