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Challenges in Diagnosing Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report
Shigang Luo1, Feifei Wang2, Huan Haung2
1Department of Radiology, The First People's Hospital of Guang Yuan, Sichuan, China.
Current Medical Imaging
|January 6, 2025
Summary
Primary intracranial Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor (EWS/pPNET) is a rare brain tumor that can be misdiagnosed. Early detection and aggressive treatment including surgery, chemotherapy, and radiotherapy are crucial for favorable outcomes.
Area of Science:
- Neuro-oncology
- Pediatric Oncology
- Neurosurgery
Background:
- Primary intracranial Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor (EWS/pPNET) is a rare central nervous system malignancy.
- Misdiagnosis is common due to its rarity and variable presentation.
Observation:
- A 23-year-old male presented with symptoms of increased intracranial pressure.
- Initial brain imaging suggested a meningioma, leading to a misdiagnosis.
- Histopathological examination confirmed primary intracranial EWS/pPNET.
Findings:
- The patient underwent successful Gross Total Resection (GTR) of the tumor.
- Adjuvant chemotherapy and radiotherapy were administered post-operatively.
- No evidence of tumor recurrence or metastasis was noted at 18 months follow-up.
Implications:
- Suspicion of EWS/pPNET is warranted in young patients with solid, aggressive, and unevenly enhancing brain masses.
- Prompt diagnosis and multimodal treatment including GTR, chemotherapy, and radiotherapy are recommended.
- Aggressive management can lead to favorable outcomes in primary intracranial EWS/pPNET.
