Cerebellopontine angle tumors in young children, displaying cranial nerve deficits, and restricted diffusion on diffusion-weighted imaging: a new clinical triad for atypical teratoid/rhabdoid tumors.
Atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system (CNS) are rare, highly malignant neoplasms that carry a poor prognosis. Even with prompt diagnosis, gross total resection and early initiation of intensive adjuvant therapy, the majority of patients will succumb within 9-12 months of diagnosis. The CPA location in children harbors lesions along a wide spectrum varying from benign to highly malignant. Imaging features of lesions within the CPA that aid the diagnostic process will help to initiate early treatment in higher-grade lesions. We report three cases, in very young children, all with cranial nerve deficits, who displayed CPA lesions with restricted diffusion on diffusion-weighted imaging (DWI) with pathology confirming AT/RT. We propose that in young children with a CPA tumor diffusion-weighted imaging should be routinely evaluated to aid in prompt management. In addition, the diagnosis of AT/RT should be highly suggestive in infants presenting with cranial nerve findings as well as DWI restricted diffusion within the CPA.
Division of Pediatric Neurosurgery, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH, 43205, USA.|Department of Neurosurgery, Grant Medical Center, Columbus, OH, USA.
Joel S
JS
Katz
Division of Pediatric Neurosurgery, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH, 43205, USA.|Department of Neurosurgery, Ohio State University, Columbus, OH, USA.
Pier Paolo
PP
Peruzzi
Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, Columbus, OH, USA.|Department of Pathology and Division of Anatomy, The Ohio State University, Columbus, OH, USA.
Christopher R
CR
Pierson
Department of Neurosurgery, Ohio State University, Columbus, OH, USA.|Department of Hematology and Oncology, Nationwide Children's Hospital, Columbus, OH, USA.
Jonathan L
JL
Finlay
Division of Pediatric Neurosurgery, Nationwide Children's Hospital, 700 Children's Drive, Columbus, OH, 43205, USA. Jeffrey.Leonard@nationwidechildrens.org.|Department of Neurosurgery, Ohio State University, Columbus, OH, USA. Jeffrey.Leonard@nationwidechildrens.org.
Jeffrey R
JR
Leonard
D002675:Child, Preschool; D003391:Cranial Nerves / Q000000981:diagnostic imaging*; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009464:Neuroma, Acoustic / Q000150:complications / Q000000981:diagnostic imaging* / Q000628:therapy; D018335:Rhabdoid Tumor / Q000150:complications / Q000000981:diagnostic imaging* / Q000628:therapy; D013724:Teratoma / Q000150:complications / Q000000981:diagnostic imaging* / Q000628:therapy
Atypical teratoid rhabdoid tumor; Cerebellopontine angle; Diffuse-weighted imaging