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Diagn Pathol. 2016 Aug 9;11(1):74. doi: 10.1186/s13000-016-0516-0.

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

Author information

1
Department of Pathology, Radboud University Medical Center, P.O. Box 9101, Nijmegen, 6500 HB, The Netherlands. Myrthe.Salet@radboudumc.nl.
2
Department of Pathology, Radboud University Medical Center, P.O. Box 9101, Nijmegen, 6500 HB, The Netherlands.
3
Department of Pediatric Oncology, Radboud University center, P.O. Box 9101, Nijmegen, 6500 HB, The Netherlands.
4
Department of Orthopedics, Radboud University Center, P.O. Box 9101, Nijmegen, 6500 HB, The Netherlands.

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.

KEYWORDS:

Differentiation; Ewing sarcoma; Maturation; Neural; Pathology

PMID:
27506465
PMCID:
PMC4979129
DOI:
10.1186/s13000-016-0516-0
[Indexed for MEDLINE]
Free PMC Article

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