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.
Childs Nerv Syst
; 33(5): 833-838, 2017 May.
Article
em En
| MEDLINE
| ID: mdl-28332155
ABSTRACT
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.
Palavras-chave
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Teratoma
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Neuroma Acústico
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Tumor Rabdoide
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Nervos Cranianos
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Imagem de Difusão por Ressonância Magnética
Tipo de estudo:
Prognostic_studies
Limite:
Child, preschool
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Female
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Humans
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Infant
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Male
Idioma:
En
Revista:
Childs Nerv Syst
Assunto da revista:
NEUROLOGIA
/
PEDIATRIA
Ano de publicação:
2017
Tipo de documento:
Article
País de afiliação:
Estados Unidos