鼻腔鼻窦神经内分泌癌8例并文献复习

Neuroendocrine carcinoma of the nasal cavity and paranasal sinus:eight cases and literature review

目的探讨鼻腔鼻窦神经内分泌癌的临床特征、病理表现、治疗手段、预后及影响预后的因素。方法回顾经病理证实的8例原发性鼻腔鼻窦神经内分泌癌患者的临床病例资料。结果随访5~70个月,中位随访时间23个月,8例中典型类癌1例、不典型类癌4例、小细胞神经内分泌癌2例、复合型神经内分泌癌1例(低分化神经内分泌癌伴内翻性乳头状瘤);平均46岁(34~57岁);T2期2例,T4期6例;接受手术7例,接受放疗6例,接受化疗6例,以手术+术后放化疗为最常见的治疗方式。结论神经内分泌癌最终确诊依靠术后病理和免疫组化指标,主要影响生存率的不良预后因素包括病理亚型和相关的治疗方式的选择。典型类癌、不典型类癌首选手术治疗,对于低分化亚型的神经内分泌癌,综合治疗对肿瘤局部控制和延长生存期有帮助。

ObjectiveTo investigate the clinical and pathological features,treatment,prognosis,and prognostic factors of neuroendocrine carcinoma of the nasal cavity and paranasal sinus.MethodsThe clinical data of 8 pathologically confirmed primary neuroendocrine carcinomas of the nasal cavity and paranasal sinus were reviewed.ResultsThe follow-up durations were 5-70 months,with a median of 23 months.Eight cases of nasal sinus neuroendocrine carcinoma were reviewed:1 case of typical carcinoid tumor,4 cases of atypical carcinoid tumor,2 cases of small cell neuroendocrine carcinoma,and 1 case of complex neuroendocrine carcinoma(poorly differentiated neuroendocrine carcinoma with varus papilloma).The mean age of the patients was 46 years(34-57 years).The stage of the malignant tumor in the nasal cavity and paranasal sinus was T2 in 2 patients and T4 in 6 patients.The cases were treated as follows:surgery,7 cases;radiotherapy,6 cases;and chemotherapy,6 cases.Surgery plus postoperative chemoradiotherapy was the most common treatment.Conclusion The final diagnosis of neuroendocrine carcinoma depends on postoperative pathological and immunohistochemical indicators.The major adverse prognostic factors affecting the survival rate included the pathological subtypes and the associated treatment options.Surgical treatment is the first choice for typical and atypical carcinomas.For poorly differentiatedsubtypesof neuroendocrine carcinoma,comprehensive treatment is the most effective method for local tumor control and prolonged survival.