长节段椎管内尤文肉瘤1例报告并文献回顾  

A case report of long segment Ewing’s sarcoma in the spinal canal and literature review

作　　者：郭韩玉 黄涛[2] 甄政 张志强[2] 李聪[2] GUO Hanyu;HUANG Tao;ZHEN Zheng;ZHANG Zhiqiang;LI Cong(Department of Neurosurgery,The Second School of Clinical Medicine,Guangzhou University of Chinese Medicine,Guangzhou,Guangdong 510000,China;Department of Neurosurgery,University Town Hospital of Guangdong Hospital of Traditional Chinese Medicine,Guangzhou,Guangdong 510000,China)

机构地区：[1]广州中医药大学第二临床医学院神经外科,广东广州510000 [2]广东省中医院大学城医院神经外科,广东广州510000

出　　处：《安徽医药》2022年第11期2291-2295,共5页Anhui Medical and Pharmaceutical Journal

摘　　要：目的提高临床医师对椎管内尤文肉瘤(Ewing sarcoma,EWS)的认识,减少误诊,争取早期诊断,早期治疗,改善病人的预后。方法对广州中医药大学第二临床医学院神经外科2020年4月收治的1例长节段椎管内EWS病人的临床资料进行整理并回顾相关文献。结果病人主诉为进行性四肢麻木乏力1月余。术前初步诊断:脊髓占位性病变(C1-T1椎体,血管源性肿瘤?脊膜瘤?神经元性肿瘤?)。入院后9 d行C1-T1椎体占位性病变切除术,术后病理提示:小圆细胞恶性肿瘤;免疫组化:CD99(+)、S-100(+)。结合免疫组化及分子病理结果:符合椎管内EWS。出院在外院行7个疗程CAV/IE化疗,随访1年,病人复发于外院行手术及放化疗。结论椎管内EWS的来源为原始神经上皮,其构成为原始未分化小圆细胞,是一类发生率极低的高度恶性肿瘤,其预后不佳、病程短、容易误诊、进展快。目前确诊主要依赖于病理学和免疫组化。Objective To improve clinicians’understanding of ewing sarcoma(EWS),reduce misdiagnosis,strive for early diagnosis and treatment,and improve the prognosis of patients.Methods The clinical data of a case of long-segment intraspinal EWS admitted to department of Neurosurgery,the Second School of Clinical Medicine,Guangzhou University of Chinese Medicine in April 2020 were analyzed and the relevant literature was reviewed.Results The patient complained of progressive numbness and weakness of limbs for more than 1 month.Preoperative diagnosis:spinal space-occupying lesions(C1-T1 vertebral body,vascularized tumor?Meningioma?Neuronal tumor?).9 d after admission,C1-T1 vertebral body level space occupying lesion resection was performed.Postoperative pathology suggested small round cell malignant tumor.Immunohistochemistry:CD99(+),S-100(+).Combined with immunohistochemistry and molecular pathology:the results were consistent with intraspinal EWS.After discharge,the patient underwent 7 courses of CAV/IE chemotherapy in another hospital,followed up for 1 year,the patient relapsed and underwent surgery and chemoradiotherapy in another hospital.Conclusion sIntraspinal EWS originates from primitive neuroepithelium,which is composed of primitive undifferentiated small round cells.It is a type of highly malignant tumor with very low incidence,with poor prognosis,short course of disease,easy misdiagnosis and rapid progression.At present,diagnosis is mainly dependent on pathology and immunohistochemistry.

关 键 词：肉瘤 EWING 椎管内肿物 原始神经外胚层肿瘤 长节段 文献回顾 

分 类 号：R739.42[医药卫生—肿瘤]