目的:探讨鼻神经内分泌癌的临床表现、治疗方法及预后。方法:回顾性分析四川大学华西医院2009-02-2012-02期间收治的8例鼻神经内分泌癌患者的临床资料。结果:3例原发于上颌窦,其中2例分别于术后8个多月及14个多月死亡,1例随访至今10个多月仍存活。原发于鼻腔5例,1例在随访至7个多月时复发,行放疗后,已随访20个多月,未见局部及远处转移;余4例,分别随访13、20、27、28个多月,未见复发。结论:本病发病率较低,临床表现及预后与发病部位有关,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、黑色素瘤及嗅神经母细胞瘤鉴别;早期诊断和综合治疗是提高本病生存率的关键。
Objective:To study the diagnosis,treatment and prognosis of sinonasal neuroendocrine carcinoma.Method:Eight patients with sinonasal neuroendocrine carcinoma from February 2009 to February 2012 were retrospectively analyzed and the related literatures were reviewed.Result:There were seven males and one female.Three cases were treated by surgery only,one case received surgery followed by radiotherapy,and four cases were treated by combined treatment(surgery followed by radiotherapy and chemotherapy).There were three patients with a primary tumor originating from the maxillary sinus,two cases died after 8 and 14 months,another patient was survived in 10 months of follow-up,and the carcinomas did not recur.There were five patients with primary neuroendocrine carcinoma from the nasal cavity,one patient recurred after the surgery and after radiotherapy,the patient did not recur after 20 months of follow-up,and the other four patients did not recur,in 13,20,27 and 28 months of follow-up.Conclusion: Neuroendocrine carcinomas of the sinuses are rare malignant tumors.Neuroendocrine carcinomas cases with the lesions at different sites differ in the clinical manifestations and prognosis,pathology,immunocytochemistry and electron microscopy,It should be differentiated from poorly differentiated squamous carcinoma melanoma,olfactory nerve blastoma and neurospongioma.The key to improve the survival rate of the disease is early accurate diagnosis and combined treatment.