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摘要: 目的 探讨鼻腔鼻窦神经内分泌癌(SNEC)的临床特点及影响预后的因素。方法 回顾性分析31例确诊为SNEC患者的临床资料。31例患者中，单纯手术切除3例，手术+放疗4例，手术+化疗4例，手术+放化疗10例，未行手术单纯放化疗10例。随访8~64个月。结果 至随访结束，2例患者失访，17例死亡，12例存活，8例复发，5年总生存率36.4%，高TNM分期、淋巴结转移、颅底浸润以及Ki-67≥55%是其预后不良因素。结论 SNEC是一种罕见的侵袭性肿瘤，预后差、局部复发率高、有转移倾向，且起病隐匿，手术联合放化疗的综合治疗仍是最佳的治疗方式。Abstract: Objective To investigate the characteristics and prognostic factors of sinonasal neuroendocrine carcinoma (SNEC).Methods The clinical data of 31 patients with SNEC were retrospectively analyzed. Among the 31 patients, 3 cases were simply surgically removed, 4 cases were surgery + radiotherapy, 4 cases were surgery + chemotherapy, 10 cases were surgery + chemoradiotherapy, and 10 cases were simply given chemoradiotherapy without surgery. The study follow-up 8-64 months.Results By the end of follow-up, 2 patients were lost to follow-up, 17 died, 12 survival, 8 relapsed and 5-year survival rate was 36.4%. High TNM stage, lymph node metastasis, skull base infiltration and Ki-67≥55% were the negative prognostic factors for survival.Conclusion SNEC is a rare aggressive tumor, with poor prognosis, high local recurrence rate, metastasis tendency, hidden disease. The comprehensive treatment of surgery combined with chemoradiotherapy is still the best treatment.
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Key words:
- neuroendocrine carcinoma /
- nose neoplasms /
- prognosis
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表 1 31例SNEC患者的一般资料
临床特征 例数(%) 临床特征 例数(%) 性别 首发症状 男 25(80.6) 鼻塞 22(71.0) 女 6(19.4) 鼻出血 13(41.9) 年龄/岁 头疼 12(38.7) < 50 12(38.7) 眼部胀痛 9(29.0) ≥50 19(61.3) 面部麻木 3(9.7) 肿瘤位置 TNM分期 左侧 15(48.4) Ⅰ期 1(3.2) 右侧 10(32.3) Ⅱ期 6(19.4) 双侧 6(19.4) Ⅲ期 5(16.1) 淋巴结转移 Ⅳ期 19(61.3) 有 8(25.8) 免疫组织化学 无 23(74.2) Ki-67/% 颅底浸润 < 55 7(22.6) 有 7(22.6) ≥55 24(77.4) 无 24(77.4) CK+ 29(93.5) 病理类型 Syn+ 30(96.8) 小细胞型 30(96.8) CD56+ 25(80.6) 大细胞型 1(3.2) CgA+ 8(25.8) 
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