儿童筛窦尤文氏肉瘤1例报告并文献复习

Primary Extraosseous Ewing Sarcoma of the Ethmoid Sinus in a Child- A Case Report And Literatrue Review

摘要:

目的：探讨儿童鼻窦尤文肉瘤的临床特征、诊疗方法及预后。方法：回顾性分析1例筛窦尤文肉瘤的临床资料，并结合相关文献进行讨论。结果：该患者为原发性筛窦尤文氏肉瘤，术前诊断缺乏特异性临床表现和影像学特征。最终通过组织病理及免疫组织化学确诊。患者接受手术联合化疗、放疗的综合治疗，随访8个月无复发和转移。 结论：儿童筛窦尤文氏肉瘤极罕见，临床表现无特异性，病理特征与鼻腔其他小圆细胞肿瘤相似，建议采用免疫标记物CD99+NKX2.2的方法诊断。鼻窦尤文氏肉瘤多采用综合治疗方案，预后较好。

Abstract:

Objective To study the clinical features, diagnosis, treatments and prognosis of Ewing’s sarcoma of the ethmoid sinus in a child . Methods The clinical data of a case of Ewing's sarcoma of the ethmoid sinus were analyzed and discussed in combination with relevant literatures. Results The diagnosed of primary Ewing’s sarcoma of the ethmoid sinus was difficult before the surgery for the clinical manifestations and imaging characteristics of the patient lacked specificity. It was eventually confirmed by the results of the postoperative examination, including the result of pathology and immunohistochemistry. The patients had no tumor recurrence or metastasis by surgery , radiation and chemotherapy.treatments followed up for 8 months . Conclusion Ewing Sarcoma of the Ethmoid Sinus is extremely rare and lack of typical and specificity clinical manifestations,so Early diagnosis was difficult , and often a comprehensive treatment plan was adopted.