Magnetic resonance imaging spectroscopy in pediatric atypical teratoid rhabdoid tumors of the brain.
BACKGROUND
Pediatric central nervous system (CNS) atypical teratoid rhabdoid tumors (ATRT) are highly malignant tumors characterized by SMARCB1 gene abnormalities. Despite chemoradiation responsiveness, most children die of disease. No imaging findings distinguish ATRT from other malignant brain tumors. This study sought to describe magnetic resonance spectroscopy (MRS) of childhood CNS ATRT and identify metabolite patterns for diagnosis and disease status monitoring.


METHODS
Data from 7 children diagnosed with CNS ATRT from 2007 to 2010, whose imaging included MRS, were retrospectively reviewed.


RESULTS
Age at diagnosis ranged from 2.5 to 54 months. Tumors were large with calcium and cysts and avid gadolinium enhancement. All were isointense on T1-weighted imaging and mildly hyperintense on T2-weighted imaging. Short-TE MRS showed prominent lactate+lipid and choline, minimal N-acetyl acetate (NAA), and rarely minimal myoinositol and low creatine peaks. Long TE showed prominent choline, minimal NAA, and rarely low lactate peaks.


CONCLUSIONS
The combination of prominent choline and lactate+lipids peaks, and generally absent NAA and myoinositol peaks by MRS in this panel of ATRT expands existing information and provides a potentially distinct metabolite profile from other malignant pediatric brain tumors, including medulloblastoma. Prospective, comparative quantitative MRS of ATRT with other pediatric CNS tumors is warranted.
*Department of Pediatrics, Division of Pediatric Hematology-Oncology, Huntsman Cancer Institute †Department of Medical Imaging, University of Utah School of Medicine and Primary Children's Medical Center, Salt Lake City, UT.
Carol S
CS
Bruggers
Kevin
K
Moore
D001921:Brain / Q000378:metabolism*; D001932:Brain Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism*; D002675:Child, Preschool; D002794:Choline / Q000378:metabolism; D002868:Chromosomal Proteins, Non-Histone / Q000235:genetics; D003401:Creatine / Q000378:metabolism; D004268:DNA-Binding Proteins / Q000235:genetics; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D007294:Inositol / Q000378:metabolism; D019344:Lactic Acid / Q000378:metabolism; D009682:Magnetic Resonance Spectroscopy / Q000379:methods*; D008297:Male; D011300:Preoperative Care / Q000379:methods; D012189:Retrospective Studies; D018335:Rhabdoid Tumor / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism*; D000071796:SMARCB1 Protein; D013724:Teratoma / Q000175:diagnosis / Q000235:genetics / Q000378:metabolism*; D014157:Transcription Factors / Q000235:genetics
D002868:Chromosomal Proteins, Non-Histone; D004268:DNA-Binding Proteins; D000071796:SMARCB1 Protein; C513266:SMARCB1 protein, human; D014157:Transcription Factors; D019344:Lactic Acid; D007294:Inositol; D003401:Creatine; D002794:Choline