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Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy

BACKGROUND: Ewing sarcoma is the second most common bone tumor, occurring mainly in children and young adults. It shows a typical primitive, small round cell morphology and a characteristic fusion oncogene involving EWSR1 and members of the ETS family in most of the cases. Neuronal maturation after...

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Autores principales: Salet, Maria Carolina Wilhelmina, Vogels, Rob, Brons, Paul, Schreuder, Bart, Flucke, Uta
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4979129/
https://www.ncbi.nlm.nih.gov/pubmed/27506465
http://dx.doi.org/10.1186/s13000-016-0516-0
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author Salet, Maria Carolina Wilhelmina
Vogels, Rob
Brons, Paul
Schreuder, Bart
Flucke, Uta
author_facet Salet, Maria Carolina Wilhelmina
Vogels, Rob
Brons, Paul
Schreuder, Bart
Flucke, Uta
author_sort Salet, Maria Carolina Wilhelmina
collection PubMed
description BACKGROUND: Ewing sarcoma is the second most common bone tumor, occurring mainly in children and young adults. It shows a typical primitive, small round cell morphology and a characteristic fusion oncogene involving EWSR1 and members of the ETS family in most of the cases. Neuronal maturation after chemotherapy is a rare phenomenon and we herein describe such an exceptional case. CASE PRESENTATION: An 8-year old boy was diagnosed with a Ewing sarcoma in the left femur. On biopsy the morphology was typical and there was an EWSR1-FLI1 gene fusion. He underwent neo-adjuvant chemotherapy and resection of the tumor. On microscopic evaluation, part of the tumor showed ganglioneuroblastoma-like differentiation with expression of neuronal markers. The continued presence of EWSR1 rearrangement in both the blue round cell component and the ganglioneuroblastoma-like component was shown by FISH analysis. CONCLUSIONS: In conclusion, this case describes the possibility of a Ewing sarcoma to differentiate into a ganglioneuroblastoma-like lesion after neo-adjuvant chemotherapy treatment; the prognostic value of this phenomenon remains questionable. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s13000-016-0516-0) contains supplementary material, which is available to authorized users.
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spelling pubmed-49791292016-08-11 Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy Salet, Maria Carolina Wilhelmina Vogels, Rob Brons, Paul Schreuder, Bart Flucke, Uta Diagn Pathol Case Report BACKGROUND: Ewing sarcoma is the second most common bone tumor, occurring mainly in children and young adults. It shows a typical primitive, small round cell morphology and a characteristic fusion oncogene involving EWSR1 and members of the ETS family in most of the cases. Neuronal maturation after chemotherapy is a rare phenomenon and we herein describe such an exceptional case. CASE PRESENTATION: An 8-year old boy was diagnosed with a Ewing sarcoma in the left femur. On biopsy the morphology was typical and there was an EWSR1-FLI1 gene fusion. He underwent neo-adjuvant chemotherapy and resection of the tumor. On microscopic evaluation, part of the tumor showed ganglioneuroblastoma-like differentiation with expression of neuronal markers. The continued presence of EWSR1 rearrangement in both the blue round cell component and the ganglioneuroblastoma-like component was shown by FISH analysis. CONCLUSIONS: In conclusion, this case describes the possibility of a Ewing sarcoma to differentiate into a ganglioneuroblastoma-like lesion after neo-adjuvant chemotherapy treatment; the prognostic value of this phenomenon remains questionable. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s13000-016-0516-0) contains supplementary material, which is available to authorized users. BioMed Central 2016-08-09 /pmc/articles/PMC4979129/ /pubmed/27506465 http://dx.doi.org/10.1186/s13000-016-0516-0 Text en © The Author(s). 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Salet, Maria Carolina Wilhelmina
Vogels, Rob
Brons, Paul
Schreuder, Bart
Flucke, Uta
Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy
title Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy
title_full Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy
title_fullStr Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy
title_full_unstemmed Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy
title_short Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy
title_sort maturation toward neuronal tissue in a ewing sarcoma of bone after chemotherapy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4979129/
https://www.ncbi.nlm.nih.gov/pubmed/27506465
http://dx.doi.org/10.1186/s13000-016-0516-0
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