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Ewing Sarcoma Displaying Extensive Well Differentiated Neuroblastomatous Differentiation: A Case Report.
Nil Çomunoğlu1, Cem Çomunoğlu2, Rahşan Özcan3
1Department of Pathology, Istanbul University-Cerrahpaşa Carrahpaşa Faculty of Medicine, Istanbul, Turkey.
Fetal and Pediatric Pathology
|May 10, 2022
Summary
A rare tumor with EWSR1/FLI fusion showed extensive neuroblastomatous differentiation. This case highlights the importance of recognizing this phenomenon in Ewing Sarcoma (ES) to ensure accurate oncologic treatment.
Area of Science:
- Pediatric Oncology
- Skeletal Tumors
- Molecular Pathology
Background:
- Ewing Sarcoma (ES) is a malignant bone tumor.
- EWSR1/FLI fusion is a characteristic genetic alteration in ES.
- Neuroblastomatous differentiation can occur in ES.
Observation:
- A 9-year-old female presented with a thoracic paraspinal mass.
- Initial biopsy suggested ganglioneuroblastoma, but later samples showed a prominent small round cell component.
- Immunohistochemistry revealed CD99 and NKX2.2 positivity.
Findings:
- Molecular analysis confirmed EWSR1/FLI fusion.
- The tumor exhibited extensive well-differentiated neuroblastomatous differentiation.
- Diagnosis was revised to Ewing Sarcoma with extensive neuroblastomatous differentiation.
Implications:
- Accurate diagnosis is crucial for effective oncologic treatment.
- Recognizing neuroblastomatous differentiation in ES prevents misinterpretation.
- This finding aids in understanding the spectrum of ES presentations.
