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一名儿科患者的原发性脊柱硬膜内骨外尤文肉瘤:病例报告及文献综述

Primary Spinal Intradural Extraosseous Ewing Sarcoma in a Pediatric Patient: Case Report and Review of the Literature.

作者信息

Scantland Joshua T, Gondim Mercia J, Koivuniemi Andrew S, Fulkerson Daniel H, Shih Chie-Schin

机构信息

Indiana University School of Medicine, Indianapolis, Indiana, USA.

Department of Pathology and Laboratory Medicine, Indiana University Health, Indianapolis, Indiana, USA.

出版信息

Pediatr Neurosurg. 2018;53(4):222-228. doi: 10.1159/000488767. Epub 2018 May 25.

Abstract

Ewing sarcoma (ES) is an aggressive, primary bone malignancy with occasional soft tissue extension. Purely extra-osseous ES is rare. A primary intraspinal, intradural ES without bone involvement is exceedingly rare. ES may be differentiated from other primitive neuroectodermal tumors by molecular analysis. The authors report the case of a 14-year-old female who suffered an acute neurologic decline from a hemorrhagic, intraspinal, intradural ES. The patient has been tumor free for 2 years after the initial emergency surgery. Our management of the patient and a review of the literature are provided. Considering only those cases with molecular or genetic confirmation of ES, our patient is the fifth pediatric case reported in the English literature.

摘要

尤因肉瘤(ES)是一种侵袭性原发性骨恶性肿瘤,偶尔会累及软组织。纯骨外ES较为罕见。原发性椎管内、硬脊膜内且无骨质受累的ES极其罕见。通过分子分析,ES可与其他原始神经外胚层肿瘤相鉴别。作者报告了一例14岁女性患者,因出血性椎管内、硬脊膜内ES导致急性神经功能衰退。该患者在初次急诊手术后已无瘤生存2年。本文介绍了对该患者的治疗情况并对文献进行了综述。仅考虑那些经分子或基因确诊为ES的病例,我们的患者是英文文献中报道的第五例儿科病例。

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