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伴有基因易位、酷似脑膜瘤的原发性颅内尤因肉瘤及多学科治疗方法:1例报告及文献系统综述

Primary Intracranial Ewing Sarcoma With Gene Translocation Mimicking a Meningioma and a Multidisciplinary Therapeutic Approach: A Case Report and Systematic Review of Literatures.

作者信息

Hyun Changjun, Lee Yeonju, Kang Ho, Park Hyun Joo, Suh Koung Jin, Choi Byung Se, Choe Gheeyoung, Kim Chae-Yong

机构信息

Department of Premedicine, Seoul National University College of Medicine, Seoul, Korea.

Department of Neurosurgery, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.

出版信息

Brain Tumor Res Treat. 2023 Oct;11(4):281-288. doi: 10.14791/btrt.2023.0030.

Abstract

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, 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.

摘要

尤因肉瘤和外周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为常见,在放射学上常类似脑膜瘤。一名38岁女性患者因右侧耳鸣、右侧偏瘫和平衡失调前来我院就诊。她接受了术前影像学检查,随后被诊断为岩骨嵴脑膜瘤。部分切除术后,证实存在基因融合,她被诊断为ES/pPNET。通过辅助化疗和放疗的多学科方法成功治疗了该肿瘤。该病例值得注意,因为它是颅内ES/pPNET极为罕见的病例,尽管未实现完全切除,但通过多学科治疗方法成功治疗了该肿瘤,我们的临床经验值得分享。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/85af/10641314/77303bc77abd/btrt-11-281-g002.jpg

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