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Diagnostic Pathology
Published in: Diagnostic Pathology 1/2016

Open Access 01-12-2016 | Case Report

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

Authors: Maria Carolina Wilhelmina Salet, Rob Vogels, Paul Brons, Bart Schreuder, Uta Flucke

Published in: Diagnostic Pathology | Issue 1/2016

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Abstract

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.
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Metadata
Title
Maturation toward neuronal tissue in a Ewing sarcoma of bone after chemotherapy
Authors
Maria Carolina Wilhelmina Salet
Rob Vogels
Paul Brons
Bart Schreuder
Uta Flucke
Publication date
01-12-2016
Publisher
BioMed Central
Published in
Diagnostic Pathology / Issue 1/2016
Electronic ISSN: 1746-1596
DOI
https://doi.org/10.1186/s13000-016-0516-0

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