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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

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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.

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Abbreviations

AT/RT:

Atypical teratoid/rhabdoid tumor

NF-2:

Neurofibromatosis type II

CPA:

Cerebellopontine angle

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Authors and Affiliations

  1. Division of Pediatric Neurosurgery, Nationwide Childrenʼs Hospital, 700 Childrenʼs Drive, Columbus, OH, 43205, USA

    Joel S. Katz, Pier Paolo Peruzzi & Jeffrey R. Leonard

  2. Department of Neurosurgery, Grant Medical Center, Columbus, OH, USA

    Joel S. Katz

  3. Department of Neurosurgery, Ohio State University, Columbus, OH, USA

    Pier Paolo Peruzzi, Jonathan L. Finlay & Jeffrey R. Leonard

  4. Department of Pathology and Laboratory Medicine, Nationwide Children’s Hospital, Columbus, OH, USA

    Christopher R. Pierson

  5. Department of Pathology and Division of Anatomy, The Ohio State University, Columbus, OH, USA

    Christopher R. Pierson

  6. Department of Hematology and Oncology, Nationwide Childrenʼs Hospital, Columbus, OH, USA

    Jonathan L. Finlay

Authors
  1. Joel S. Katz
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  2. Pier Paolo Peruzzi
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  3. Christopher R. Pierson
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  4. Jonathan L. Finlay
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  5. Jeffrey R. Leonard
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Correspondence to Jeffrey R. Leonard.

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Katz, J.S., Peruzzi, P.P., Pierson, C.R. et al. 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, 833–838 (2017). https://doi.org/10.1007/s00381-017-3384-3

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  • DOI: https://doi.org/10.1007/s00381-017-3384-3

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