Abstract
BACKGROUND: Primary intracranial Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor (EWS/pPNET) is exceedingly rare and easy to misdiagnose. CASE PRESENTATION: We present a case involving a 23-year-old male who presented with headaches and vomiting. The preoperative brain imaging revealed an irregular mass in the left parietal lobe, initially misdiagnosed as meningioma. However, the surgical specimen was ultimately diagnosed as primary intracranial EWS/pPNET. The patient underwent a total tumor resection, followed by adjuvant chemotherapy and radiotherapy. No recurrence or distant metastasis was observed 18 months after the surgery. CONCLUSION: When the imaging features of young patients' lesions are solid, aggressive, and unevenly enhanced masses, physicians should be aware of the possibility of primary intracranial EWS/pPNET, and if possible, Gross Total Resection (GTR) and intensive chemotherapy and radiotherapy are recommended.