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Diagnosis, treatment, and postoperative follow-up of intracranial Ewing sarcoma (EWS): a case report
Kaidi Liu1,2, Xiaolei Wang3,4, Shuo Zhang3,4
1Shandong First Medical University, Jinan, China. 20232120680@email.sdfmu.edu.cn.
Insights
Primary intracranial Ewing Sarcoma (EWS) is rare and challenging to diagnose. Multimodal treatment including surgery, radiotherapy, and chemotherapy offers favorable short-term outcomes for pediatric posterior fossa EWS.
Area of Science:
- Pediatric neuro-oncology
- Molecular diagnostics in oncology
- Surgical oncology
Background:
- Intracranial Ewing Sarcoma (EWS) is an exceptionally rare primary brain tumor in children.
- Nonspecific radiological features present significant diagnostic challenges.
- Posterior fossa location is uncommon for intracranial EWS.
Purpose of the Study:
- To investigate diagnostic procedures, therapeutic strategies, and postoperative surveillance for primary intracranial EWS.
- To highlight a pediatric case of posterior fossa EWS.
- To conduct a comparative literature review on intracranial EWS.
Main Methods:
- Case presentation of a 6-year-old girl with posterior fossa mass.
- Diagnostic workup including MRI, histopathology, and molecular analysis (EWSR1-FLI1 fusion gene).
- Maximal safe surgical resection followed by adjuvant radiotherapy and chemotherapy (VDC/IE regimen).
Main Results:
- Near-total tumor resection achieved.
- Histopathological and molecular analysis confirmed EWS.
- Postoperative adjuvant therapy completed with no evidence of recurrence at 8-month follow-up.
- Patient remained neurologically stable.
Conclusions:
- Integrated pathological and molecular testing is crucial for definitive diagnosis of intracranial EWS.
- Multimodal treatment combining maximal safe surgical resection, radiotherapy, and chemotherapy yields favorable short-term oncological outcomes.
- Tumor location and extent of resection are important prognostic factors.
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.
