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Embryonal tumor with multilayered rosettes: illustrative case and review of the literature

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Abstract

Background

Embryonal tumor with multilayered rosettes (ETMR) is a very rare entity and has seldom been reported. It has been newly defined tumor entity included in the latest update (revised fourth edition) of WHO 2016 Classification of Tumors of the Central Nervous System which portends a uniform dismal prognosis and survival even with the best of multimodality approaches.

Illustrative case

This report documents the presentation of a 2-year-old girl with voluminous intracranial ETMR in the right parieto-occipital region. We describe clinical diagnosis, histological aspects, radiological features, and current management of this very aggressive tumor.

Conclusion

Pediatric intracranial ETMR is a highly aggressive neoplasm, and it should be considered in the differential diagnosis of pediatric brain tumors.

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Authors and Affiliations

  1. Department of Neurosurgery, National Institute of Neurology ”Mongi Ben Hmida”, Tunis, Faculty of Medicine, University of Tunis el MANAR, Tunis, Tunisia

    Sofiene Bouali, Imed Ben Said, Jalel Kallel & Hafedh Jemel

  2. Department of Histopathology la Rabta, Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia

    Alia Zehani

  3. Department of Neuroradiology, National Institute of Neurology Tunis, Tunisia Faculty of Medicine, University of Tunis el MANAR, Tunis, Tunisia

    Maha Mahmoud

Authors
  1. Sofiene Bouali
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  2. Alia Zehani
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  4. Imed Ben Said
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Correspondence to Sofiene Bouali.

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Bouali, S., Zehani, A., Mahmoud, M. et al. Embryonal tumor with multilayered rosettes: illustrative case and review of the literature. Childs Nerv Syst 34, 2361–2369 (2018). https://doi.org/10.1007/s00381-018-3972-x

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