头颈部骨外尤文氏肉瘤4例并文献复习

Four cases of extraskeletal Ewing′s sarcoma in the head and neck and literature review

目的:探讨头颈部骨外尤文肉瘤的临床特征、诊疗方法及预后。方法:分析4例头颈部骨外尤文肉瘤的临床资料,并结合相关文献讨论。结果:4例患者中,发生于鼻腔、鼻窦2例,口咽部1例,皮肤软组织1例,所有患者均通过组织病理及免疫组织化学确诊。2例患者接受以手术为主的综合治疗,分别随访7年及7个月无复发和转移;1例单纯手术者随访至6个月时因肺转移死亡;余1例单纯化疗者在治疗过程中因远处转移死亡。结论:头颈部骨外尤文肉瘤极少见,临床表现无特异性,早期诊断困难,多采用综合治疗方案。本病易复发,转移率高,预后不佳。

Objective：To evaluate the clinical features,diagnosis,treatment and prognosis of the extraskeletal Ewing＇s sarcoma in the head and neck. Method：The clinical data of four cases of extraskeletal Ewing＇s sarcoma was analysed and relevant literatures were reviewed. Result： Two cases originated from the nasal cavity and paranasal si nuses, the other two cases originated from the oral pharynx and skin respectively. The tumors were all diagnosed via histopathology and immunohistochemistry. Two cases were treated by combined modality therapy and did not recur or metastasize after follow-up for 7 months and 7 years respectively. Another one was treated by surgery and died after 6 months of follow-up. The remaining one was treated by chemotherapy and died with metastasis during the period of therapy. Conclusion： The extraskeletal Ewing＇s sarcoma, which is rare arising from the head and neck, has non-specific clinical manifestation and difficulty in early diagnosis. The disease can be effectively treated by combined therapy, but the high recurrent and metastatic rates usually result in poor prognosis.