Challenges in Diagnosing Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report

TL;DR

This case report highlights the challenges in diagnosing a rare brain tumor, EWS/pPNET, and emphasizes the importance of accurate diagnosis and aggressive treatment.

Contribution

The paper presents a rare case of primary intracranial EWS/pPNET and provides diagnostic and treatment insights for similar cases.

Findings

The patient's tumor was initially misdiagnosed as meningioma but was later confirmed as EWS/pPNET.

The patient underwent successful treatment with no recurrence after 18 months.

Gross Total Resection and adjuvant therapies are recommended for such tumors.

Abstract

Primary intracranial Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor (EWS/pPNET) is exceedingly rare and easy to misdiagnose.

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.

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.

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