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作者

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

作者单位

Affiliations ¹ Shandong First Medical University, Jinan, China. 20232120680@email.sdfmu.edu.cn. ² Shandong Provincial Hospital, Jinan, China. 20232120680@email.sdfmu.edu.cn. ³ Shandong First Medical University, Jinan, China. ⁴ Shandong Provincial Hospital, Jinan, China. ⁵ Shandong First Medical University, Jinan, China. fengshaobin@sdfmu.edu.cn. ⁶ Shandong First Medical University, Jinan, China. guohua@sdfmu.edu.cn.

刊名

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

年份

2026

卷号

Vol.42 No.1

页码

118

ISSN

1433-0350

关键词

Intracranial Ewing sarcoma Near-total resection Treatment strategy.

摘要

Objective: This study investigates the diagnostic procedures, therapeutic strategies, and postoperative surveillance of primary intracranial Ewing Sarcoma , 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,...更多

Objective: This study investigates the diagnostic procedures, therapeutic strategies, and postoperative surveillance of primary intracranial Ewing Sarcoma , 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 and multi-agent chemotherapy . 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.收起

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