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Embryonal tumor with abundant neuropil and true rosettes: an autopsy case-based update and review of the literature
INTRODUCTION: Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a rare subtype of primitive neuroectodermal tumors first reported in 2000. It is rare among the group of embryonal central nervous system tumors with approximately 50 reported cases. ETANTR has been suggested to be a...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer-Verlag
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3620447/ https://www.ncbi.nlm.nih.gov/pubmed/23358909 http://dx.doi.org/10.1007/s00381-013-2037-4 |
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author | Adamek, Dariusz Sofowora, Kolawole D. Cwiklinska, Magdalena Herman-Sucharska, Izabela Kwiatkowski, Stanislaw |
author_facet | Adamek, Dariusz Sofowora, Kolawole D. Cwiklinska, Magdalena Herman-Sucharska, Izabela Kwiatkowski, Stanislaw |
author_sort | Adamek, Dariusz |
collection | PubMed |
description | INTRODUCTION: Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a rare subtype of primitive neuroectodermal tumors first reported in 2000. It is rare among the group of embryonal central nervous system tumors with approximately 50 reported cases. ETANTR has been suggested to be a separate entity among this group of tumors. CASE REPORT: Herein, we present only the second autopsy case of ETANTR, which occurred in a 17-month-old boy, and was located in the brainstem. The tumor was inoperable, and despite chemotherapy, the child died 3 months after initial hospitalization. A brain only autopsy was performed. DISCUSSION: Neuropathological and neuroimaging examinations suggest ETANTR grew by expansion rather than invasion distorting anatomical structures of the posterior fossa. We suggest that the characteristic histopathological picture of the tumor is the result of multifocal and dispersed germinative activity surrounded by mature neuropil-like areas. CONCLUSION: ETANTR is a pediatric tumor occurring in children under 4 with a significantly poor prognosis with more cases and research required to characterize this rare embryonal tumor. |
format | Online Article Text |
id | pubmed-3620447 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2013 |
publisher | Springer-Verlag |
record_format | MEDLINE/PubMed |
spelling | pubmed-36204472013-04-10 Embryonal tumor with abundant neuropil and true rosettes: an autopsy case-based update and review of the literature Adamek, Dariusz Sofowora, Kolawole D. Cwiklinska, Magdalena Herman-Sucharska, Izabela Kwiatkowski, Stanislaw Childs Nerv Syst Case Report INTRODUCTION: Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a rare subtype of primitive neuroectodermal tumors first reported in 2000. It is rare among the group of embryonal central nervous system tumors with approximately 50 reported cases. ETANTR has been suggested to be a separate entity among this group of tumors. CASE REPORT: Herein, we present only the second autopsy case of ETANTR, which occurred in a 17-month-old boy, and was located in the brainstem. The tumor was inoperable, and despite chemotherapy, the child died 3 months after initial hospitalization. A brain only autopsy was performed. DISCUSSION: Neuropathological and neuroimaging examinations suggest ETANTR grew by expansion rather than invasion distorting anatomical structures of the posterior fossa. We suggest that the characteristic histopathological picture of the tumor is the result of multifocal and dispersed germinative activity surrounded by mature neuropil-like areas. CONCLUSION: ETANTR is a pediatric tumor occurring in children under 4 with a significantly poor prognosis with more cases and research required to characterize this rare embryonal tumor. Springer-Verlag 2013-01-29 2013 /pmc/articles/PMC3620447/ /pubmed/23358909 http://dx.doi.org/10.1007/s00381-013-2037-4 Text en © The Author(s) 2013 https://creativecommons.org/licenses/by-nc/2.0/ Open Access This article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited. |
spellingShingle | Case Report Adamek, Dariusz Sofowora, Kolawole D. Cwiklinska, Magdalena Herman-Sucharska, Izabela Kwiatkowski, Stanislaw Embryonal tumor with abundant neuropil and true rosettes: an autopsy case-based update and review of the literature |
title | Embryonal tumor with abundant neuropil and true rosettes: an autopsy case-based update and review of the literature |
title_full | Embryonal tumor with abundant neuropil and true rosettes: an autopsy case-based update and review of the literature |
title_fullStr | Embryonal tumor with abundant neuropil and true rosettes: an autopsy case-based update and review of the literature |
title_full_unstemmed | Embryonal tumor with abundant neuropil and true rosettes: an autopsy case-based update and review of the literature |
title_short | Embryonal tumor with abundant neuropil and true rosettes: an autopsy case-based update and review of the literature |
title_sort | embryonal tumor with abundant neuropil and true rosettes: an autopsy case-based update and review of the literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3620447/ https://www.ncbi.nlm.nih.gov/pubmed/23358909 http://dx.doi.org/10.1007/s00381-013-2037-4 |
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