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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Research ArticlePediatrics

Pediatric Atypical Teratoid/Rhabdoid Tumors of the Brain: Identification of Metabolic Subgroups Using In Vivo 1H-MR Spectroscopy

B. Tamrazi, S. Venneti, A. Margol, D. Hawes, S.Y. Cen, M. Nelson, A. Judkins, J. Biegel and S. Blüml
American Journal of Neuroradiology May 2019, 40 (5) 872-877; DOI: https://doi.org/10.3174/ajnr.A6024
B. Tamrazi
aFrom the Departments of Radiology (B.T., M.N., S.B.)
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S. Venneti
dDepartment of Pathology (S.V.), University of Michigan, Ann Arbor, Michigan
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A. Margol
bPediatrics (A.M.) and Division of Hematology Oncology
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D. Hawes
cPathology (D.H., A.J., J.B.), Children's Hospital Los Angeles, Los Angeles, California
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S.Y. Cen
eDepartment of Radiology and Neurology (S.Y.C.), University of Southern California, Los Angeles, California
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M. Nelson
aFrom the Departments of Radiology (B.T., M.N., S.B.)
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A. Judkins
cPathology (D.H., A.J., J.B.), Children's Hospital Los Angeles, Los Angeles, California
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J. Biegel
cPathology (D.H., A.J., J.B.), Children's Hospital Los Angeles, Los Angeles, California
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S. Blüml
aFrom the Departments of Radiology (B.T., M.N., S.B.)
fRudi Schulte Research Institute (S.B.), Santa Barbara, California.
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Abstract

BACKGROUND AND PURPOSE: Atypical teratoid/rhabdoid tumors are rare, aggressive central nervous system tumors that are predominantly encountered in very young children. Our aim was to determine whether in vivo metabolic profiles correlate with molecular features of central nervous system pediatric atypical teratoid/rhabdoid tumors.

MATERIALS AND METHODS: Twenty confirmed patients with atypical teratoid/rhabdoid tumors who underwent MR spectroscopy were included in this study. In vivo metabolite levels of atypical teratoid/rhabdoid tumors were compared with molecular subtypes assessed by achaete-scute homolog 1 expression. Additionally, brain-specific creatine kinase levels were determined in tissue samples.

RESULTS: In vivo creatine concentrations were higher in tumors that demonstrated achaete-scute homolog 1 expression compared with those without achaete-scute homolog 1 expression (3.42 ± 1.1 versus 1.8 ± 0.8 IU, P < .01). Additionally, levels of myo-inositol (mI) (9.0 ± 1.5 versus 4.7 ± 3.6 IU, P < .05) were significantly different, whereas lipids approached significance (44 ± 20 versus 80 ± 30 IU, P = .07) in these 2 cohorts. Higher brain-specific creatine kinase levels were observed in the cohort with achaete-scute homolog 1 expression (P < .05). Pearson correlation analysis showed a significant positive correlation of brain-specific creatine kinase with absolute creatine (P < .05) and myo-inositol (P < .05) concentrations.

CONCLUSIONS: In vivo MR spectroscopy may predict key molecular features of atypical teratoid/rhabdoid tumors at initial diagnosis, leading to timely patient risk stratification and accelerating the development of targeted therapies.

ABBREVIATIONS:

ASCL1
achaete-scute homolog 1
AT/RT
atypical teratoid/rhabdoid tumor
CKB
brain-specific creatine kinase
Cr
creatine
mI
myo-inositol
Cho
choline
SMARCB1
SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1
  • © 2019 by American Journal of Neuroradiology
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American Journal of Neuroradiology: 40 (5)
American Journal of Neuroradiology
Vol. 40, Issue 5
1 May 2019
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Cite this article
B. Tamrazi, S. Venneti, A. Margol, D. Hawes, S.Y. Cen, M. Nelson, A. Judkins, J. Biegel, S. Blüml
Pediatric Atypical Teratoid/Rhabdoid Tumors of the Brain: Identification of Metabolic Subgroups Using In Vivo 1H-MR Spectroscopy
American Journal of Neuroradiology May 2019, 40 (5) 872-877; DOI: 10.3174/ajnr.A6024

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Pediatric Atypical Teratoid/Rhabdoid Tumors of the Brain: Identification of Metabolic Subgroups Using In Vivo 1H-MR Spectroscopy
B. Tamrazi, S. Venneti, A. Margol, D. Hawes, S.Y. Cen, M. Nelson, A. Judkins, J. Biegel, S. Blüml
American Journal of Neuroradiology May 2019, 40 (5) 872-877; DOI: 10.3174/ajnr.A6024
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