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[目的]探讨鼻腔鼻窦神经内分泌癌的临床表现、病理特点、治疗方法及预后.[方法]回顾性分析陆军军医大学第一附属医院2007年10月至2015年8月期间收治的14例鼻腔鼻窦神经内分泌癌患者的临床资料,并复习相关文献,探讨本病的临床特点和治疗相关问题.[结果]14例均病理证实为小细胞神经内分泌癌:3例失访;1例于就诊后1个月死亡;1例治疗后5个月出现肝转移,未治疗,随访11个月后死亡;带瘤生存4例;1例治疗后11个月出现局部复发,未治疗,目前随访109个月病情稳定;1例治疗后60个月出现局部复发,目前随访67个月局部放疗中;1例治疗后32个月出现脑转移,再行颅脑放疗及化疗,目前随访44个月病情稳定;1例随访24个月病情稳定;无瘤生存5例,随访24～89个月均未提示肿瘤局部复发及远处转移.[结论]鼻腔鼻窦神经内分泌癌的发病率低,诊断主要取决于组织病理学表现、免疫组化结果及电镜下超微结构特征;需与低分化鳞癌、黑色素瘤和嗅神经母细胞瘤相鉴别;早期诊断、肿瘤分期、侵犯范围、能否手术以及是否结合放化疗均是本病的预后影响因素.“,”[Objective] To investigate the clinical performance,pathological characteristics,treatment and prognosis of sinonasal neuroendocrine carcinoma.[Methods]A retrospectively study was carried out among 14 eases with this tumor treated in the First Affiliated Hospital of Army Medical University from October 2007 to August 2015 through a careful analysis on their clinical data,the clinical characteristics and treatment of the disease was studied on reviewing the relevant literature.[Results]All of the 14 cases were confirmed pathologically as small cell neuroendocrine carcinoma:3 cases were lost to follow-up,1 case died 1 month after treatment;1 case had liver metastasis 5 months after treatment,no treatment,and died after 11 months of follow-up;Tumor bearing survival in 4 eases;Local recurrence occurred in 1 ease after 11 months of treatment and had no treatment.The patient was followed up for 109 months and the condition was stable;Local recurrence occurred in 1 case 60 months after treatment,and was followed up for 67 months,now in the local radiotherapy;Brain metastasis occurred in 1 patient after 32 months of treatment,followed by cranial radiotherapy and chemotherapy.The patient was followed up for 44 months and the condition was stable;1 case was followed up for 24 months,the condition was stable,no tumor survived in 5 cases,followed up for 24～89 months,and did not suggest local recurrence and distant metastasis of tumor.[Conclusion]The incidence of this disease is low,the diagnosis mainly depends on histopathologic manifestations,immunohistochemical results and ultrastructure under electron microscope characteristics;it should be differentiated from the poorly differentiated squamous carcinoma,melanoma and olfactory nerve blastoma and neurospongioma;Early diagnosis,tumor staging,extent of invasion,with or without surgery and combination of chemotherapy and radiotherapy are the prognostic factors of the disease.