Scantland Joshua T, Gondim Mercia J, Koivuniemi Andrew S, Fulkerson Daniel H, Shih Chie-Schin
Indiana University School of Medicine, Indianapolis, Indiana, USA.
Department of Pathology and Laboratory Medicine, Indiana University Health, Indianapolis, Indiana, USA.
Pediatr Neurosurg. 2018;53(4):222-228. doi: 10.1159/000488767. Epub 2018 May 25.
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.
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。