儿童眶底及上颌窦前壁原始神经外胚层肿瘤/尤文氏肉瘤的临床分析  

Primitive neuroectodermal tumor/Ewing sarcoma of the orbital floor and anterior maxillary Sinus in children:A case report and literature review

作　　者：焦红叶 潘晗[1] 施涛 汪小霞[2] 周玫[1] 陈伟[1] 张勇[1] JIAO Hong-ye;PAN Han;SHI Tao;WANG Xiao-xia;ZHOU Mei;CHEN Wei;ZHANG Yong(Department of ENT,General Hospital of Eastern Theater Command,PLA,Nanjing 210002,Jiangsu,China;Department of Pathology,General Hospital of Eastern Theater Command,PLA,Nanjing 210002,Jiangsu,China)

机构地区：[1]东部战区总医院(原南京军区南京总医院)耳鼻咽喉头颈外科,南京210002 [2]东部战区总医院(原南京军区南京总医院)病理科,南京210002

出　　处：《东南国防医药》2021年第1期17-20,共4页Military Medical Journal of Southeast China

基　　金：江苏省自然科学基金(BK20161388)。

摘　　要：目的探讨眶底及上颌窦前壁原始神经外胚层肿瘤/尤文氏肉瘤(ESFTs)这一罕见疾病的临床特点。方法报道1例儿童ESFTs的临床特征、影像学表现、病理学特征,探讨其诊断、鉴别诊断、治疗及预后,并通过文献复习了解该病早期诊断要点。结果该例患儿肿瘤病理上以小圆细胞为主要结构,细胞免疫组化为CD99的高表达。治疗方法为手术切除加放化疗,患者术后随访21个月未出现肿瘤复发及远处转移征象。结论头颈部肿瘤高度恶性肿瘤ESFTs影像学检查有一定的诊断意义,但是没有特异性,明确诊断主要依靠免疫组化检查。确诊后应尽可能早期手术,以免贻误治疗时机。预后较差,手术切除加放化疗能改善患者预后。Objective To reportthe clinical data of patients with primitive neuroectodermal tumor(PNET/Ewing sarcoma)in the orbital floor and the anterior wall of the maxillary Sinus for better understanding of this rare disease.Methods The clinical features,imaging features and pathological features of a child PNET/Ewing sarcoma was reported.The diagnosis,differential diagnosis,treatment and prognosis of the child PNET/Ewing sarcoma were examined.The key points of early diagnosis of the child PNET/Ewing sarcoma were reviewed.Results In this case,the tumor was mainly composed of small round cells,and the cell immunohistochemistry showed high expression of CD99.The treatment method was surgical resection plus chemoradiotherapy.At present,the patients were followed up 21 months without tumor recurrence or distant metastasis.Conclusion PNET/Ewing sarcoma is a highly malignant tumor of head and neck.Imaging examination has certain diagnostic significance,but there is no specificity.The clear diagnosis mainly relies on immunohistochemical examination.After the diagnosis should be as early as possible operation,so as not to delay the treatment opportunity.The prognosis is poor,and surgical resection combined with chemoradiotherapy can improve the prognosis of patients.

关 键 词：原始神经外胚层肿瘤/尤文肉瘤 诊断 治疗 

分 类 号：R734.2[医药卫生—肿瘤]