Article Text
Summary
Ewing’s sarcoma is a highly aggressive malignant tumour most commonly affecting long bones in children and adolescents. It is part of the Ewing’s sarcoma family of tumours (ESFTs) that also include peripheral primitive neuroectodermal tumour and Askin’s tumours. ESFTs share common cytogenetic aberrations, antigenic profiles and proto-oncogene expression with an overall similar clinical course. In 99% of ESFTs, genetic translocation with molecular fusion involves the EWSR1 gene on 22q12. Approximately 30% of ESFTs are extraosseous, most commonly occurring in the soft tissues of extremities, pelvis, retroperitoneum and chest wall. Primary presentation in solid organs is very rare but has been described in multiple sites including the pancreas. Accurate diagnosis of a Ewing’s sarcoma in a solid organ is critical in facilitating correct treatment. We report the case of a 17-year-old girl with cytogenetically confirmed primary pancreatic Ewing’s sarcoma and provide a brief review of the published literature.
- Primary pancreatic Ewing’s sarcoma
- Extraosseous Ewing’s sarcoma
- Primitive neuroectodermal tumor
- peripheral primitive neuroectodermal tumor (pPNET)
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Footnotes
Contributors AG conceptualised the manuscript, acquired data and wrote the manuscript.
SR acquired data and edited the manuscript.
BH participated in clinical pathological work, acquired data and edited the manuscript.
SHS conceptualised the manuscript, acquired data and edited the manuscript.
All authors have seen and approved the final version of the manuscript.
All authors agree to be accountable for the accuracy and integrity of the manuscript.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.