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Pediatric Atypical Teratoid/Rhabdoid Tumor: Diagnosis Using Imaging Techniques and Histopathology

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Pediatric Cancer, Volume 2

Part of the book series: Pediatric Cancer ((PECA,volume 2))

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Abstract

Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system is an aggressive infantile embryonal neoplasm, usually presented as an intra-parenchymatous lesion. On Computed Tomodensitometry (CT) and Magnetic Resonance Imaging (MRI), AT/RT appears as a bulky, heterogeneous lesion, enhanced after contrast material injection. Both CT and MRI findings are similar to those seen in patients with Primitive Neuroectodermal Tumor/Medulloblastoma (PNET/medulloblastoma), consisting in the main differential diagnosis. Very few cases with diffusion, perfusion and spectroscopy data have been reported, but it seems that AT/RTs and PNET/medulloblastomas are not more distinguishable with more recent MRI techniques. One major histopathologic characteristic of AT/RT is the presence of rhabdoid cells, rarely predominant and not specific given that AT/RT contains variable components with primitive neuroectodermal, mesenchymal and epithelial features. Immunohistochemistry and genetic studies allow the differentiation between PNET/medulloblastoma and composite rhabdoid tumors. A precise diagnosis of AT/RT is important as this tumor has to be treated with intensive therapy (surgery, chemotherapy and radiation therapy). In spite of this treatment, the pronostic of AT/RT remains very poor.

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Acknowledgments

I am grateful to Doctor Caroline Salon, neuropathologist, Grenoble University Hospital, for her valuable suggestions and help for this write-up.

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  1. Neuroradiology unit, University Hospital of Grenoble, 38700, Grenoble Cedex 09, France

    Fabrice Bing

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  1. Fabrice Bing
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Correspondence to Fabrice Bing .

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  1. , Kean University, Room 213, Library building, Morris Avenue 1000, Union, 07083, New Jersey, USA

    M.A. Hayat

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Bing, F. (2012). Pediatric Atypical Teratoid/Rhabdoid Tumor: Diagnosis Using Imaging Techniques and Histopathology. In: Hayat, M. (eds) Pediatric Cancer, Volume 2. Pediatric Cancer, vol 2. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-2957-5_5

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