具有 EWSR1-FLI1 基因易位的原发性颅内尤文肉瘤模仿脑膜瘤和多学科治疗方法:病例报告和文献系统回顾。
Primary Intracranial Ewing Sarcoma With EWSR1-FLI1 Gene Translocation Mimicking a Meningioma and a Multidisciplinary Therapeutic Approach: A Case Report and Systematic Review of Literatures.
发表日期:2023 Oct
作者:
Changjun Hyun, Yeonju Lee, Ho Kang, Hyun Joo Park, Koung Jin Suh, Byung Se Choi, Gheeyoung Choe, Chae-Yong Kim
来源:
Brain Structure & Function
摘要:
尤文肉瘤和周围原始神经外胚层肿瘤 (ES/pPNET) 是一种未分化的恶性肿瘤,在儿童和年轻人中最常见,放射学上常常类似于脑膜瘤。患者,女,38岁,主诉右侧耳鸣、右侧偏瘫、身体不平衡就诊。她接受了术前影像检查,随后被诊断为岩骨嵴上有脑膜瘤。部分切除后,证实EWSR1-FLI1基因融合,诊断为ES/pPNET。使用辅助化疗和放疗的多学科方法成功治疗了肿瘤。该病例值得注意,因为这是极其罕见的颅内 ES/pPNET 病例,尽管未实现完全切除,但通过多学科治疗方法成功治疗肿瘤,值得分享我们的临床经验。版权所有 © 2023 韩国脑肿瘤学会、韩国神经肿瘤学会和韩国儿科神经肿瘤学会。
Ewing sarcoma and peripheral primitive neuroectodermal tumor (ES/pPNET) is an undifferentiated malignant tumor that is most prevalent in children and young adults and often radiologically mimics a meningioma. A 38-year-old female patient visited our hospital with complaints of right-sided tinnitus, right hemiparesis, and imbalance. She underwent preoperative imaging and was subsequently diagnosed as having a meningioma on the petrous ridge. After partial resection, EWSR1-FLI1 gene fusion was confirmed, and she was diagnosed with ES/pPNET. The tumor was successfully treated using a multidisciplinary approach of adjuvant chemo- and radiotherapy. This case is noteworthy because it is an extremely rare case of an intracranial ES/pPNET, and it is worth sharing our clinical experience that the tumor was successfully treated through a multidisciplinary therapeutic approach even though complete resection was not achieved.Copyright © 2023 The Korean Brain Tumor Society, The Korean Society for Neuro-Oncology, and The Korean Society for Pediatric Neuro-Oncology.