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Objective <p>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.</p> Case report <p>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.</p> Results <p>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&#xa0;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.</p> Conclusion <p>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.</p>

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

  • 期刊论文

  • 发表日期：

    2026年3月15日

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  • Kaidi Liu,
  • Xiaolei Wang,
  • Shuo Zhang,
  • Shaobin Feng,
  • Hua Guo

摘要

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.