颈部骨外尤文氏肉瘤1例报道并文献复习

Extraskeletal Ewing's sarcoma: a case report and literature review

摘要:

目的 探讨骨外尤文氏肉瘤（EES）的疾病特征，了解其临床表现、诊断及治疗方案。方法 对1例颈部EES患者的临床资料进行回顾性分析，复习相关文献并进行讨论。结果 该例EES患者临床表现及影像学特征缺乏特异性，确诊依赖于术后病检，其中免疫组化结果为PAS（+）、CD99（+）、Vimentin（+），基因检测EWSR1基因易位，治疗采用手术为主的综合治疗，随访8个月后复发。结论 颈部EES极其罕见，EES侵袭性强、恶性程度高，强调早期诊断和采用综合性治疗方案，但预后较差。

Abstract:

Objective To study the clinical features, diagnosis and treatment of extraskeletal Ewing’s sarcoma (EES).Methods The clinical data of a patient with EES were analyzed, and the relevant literatures were reviewed and discussed.Results The clinical manifestations and imaging characteristics of the patient with EES lacked specificity. The diagnosis of EES in this case was dependent on postoperative examination, in which the immunohistochemical results were PAS (+), CD99 (+) and Vimentin (+), and the translocation of EWSR1 gene was detected by gene test. Optimal treatment consisted of surgery in combination with chemotherapy.Relapse occurred after 8 months of the follow-up.Conclusions EES in the neck is extremely rare, which has the parameter of strong invasion and highly malignant.Early diagnosis and comprehensive treatment should be emphasized, but the prognosis were worse.