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

Maria Carolina Wilhelmina Salet*, Rob Vogels, Paul P. T. Brons, Bart Schreuder, Uta Flucke

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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.

Original languageEnglish
Article number74
JournalDiagnostic Pathology
Volume11
Issue number1
DOIs
Publication statusPublished - 9 Aug 2016

Keywords

  • Differentiation
  • Ewing sarcoma
  • Maturation
  • Neural
  • Pathology

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