Diagnosis, treatment, and postoperative follow-up of intracranial Ewing sarcoma (EWS): a case report.

[OBJECTIVE] This study investigates the diagnostic procedures, therapeutic strategies, and postoperative surveillance of primary intracranial Ewing Sarcoma (EWS), with a focus on a pediatric case in the posterior fossa and a comparative literature review.

[CASE REPORT] We present the case of a 6-year-old girl admitted with a 1-month history of worsening headaches and signs of elevated intracranial pressure. Preoperative MRI identified a large, heterogeneously enhancing mass in the left posterior fossa, initially suspected to be a medulloblastoma. The patient underwent maximal safe surgical resection via a posterolateral paramedian approach. Histopathological and molecular analysis confirmed the diagnosis of EWS, revealing the characteristic EWSR1-FLI1 fusion gene.

[RESULTS] Near-total resection of the tumor was achieved, with no gross residual tumor observed under the operating microscope. The patient subsequently received adjuvant radiotherapy (30 Gy) and multi-agent chemotherapy (VDC/IE regimen). An 8-month follow-up cranial MRI showed no evidence of tumor recurrence, and the patient remained neurologically stable.

[CONCLUSION] Intracranial EWS is exceptionally rare and poses a significant diagnostic challenge due to its nonspecific radiological features. Definitive diagnosis relies on integrated pathological and molecular genetic testing. This case underscores that a multimodal treatment strategy centered on maximal safe surgical resection, combined with radiotherapy and chemotherapy, can achieve favorable short-term oncological outcomes. A review of the literature highlights the prognostic importance of tumor location and extent of resection.