胆道系统神经内分泌肿瘤的病理和预后

Clinical features and prognosis of extrahepatic biliary neuroendocrine neoplasms

目的探讨肝外胆道系统神经内分泌肿瘤(extrahepatic biliary neuroendocrine neoplasms,EB-NENs)的临床病理特征及影响预后的因素。方法回顾性分析2014年5月至2018年5月收治的21例EB-NENs患者临床资料和生存情况。结果EB-NENs占同期全部胆道系统肿瘤总数的1.6%(21/1313),就诊时淋巴结转移7例(33.3%),远处转移5例(23.8%);至随访结束,中位生存时间(23.23±4.17)个月;单因素分析显示,TNM分期(χ^2=9.066,P=0.003)、淋巴结转移(χ^2=6.399,P=0.011)和远处转移(χ^2=9.808,P=0.002)是影响预后的相关危险因素。多因素分析显示,TNM分期(P=0.008,RR=3.003,95%CI:1.332~6.774)、淋巴结转移(P=0.023,RR=5.382,95%CI:1.261~22.971)和远处转移(P=0.007,RR=7.423,95%CI:1.730~31.851)是影响预后的独立危险因素。结论EB-NENs发病率低,预后差。TNM分期、淋巴结转移和远处转移是影响患者预后的独立危险因素。

Objective To investigate the clinical features and prognostic factors of extrahepatic biliary neuroendocrine neoplasms(EB-NENs).Methods The clinical data and survival of 21 patients with EB-NENs admitted from May 2014 to May 2018 were analyzed retrospectively.Results These 21 EB-NENs patients accounted for 1.6%(21/1313)of all biliary tract neoplasms treated during the study period.Seven(33.3%)cases had lymph node metastasis.Five(23.8%)suffered from distant metastasis.The follow up time was(4~46.5)months with median survival time of(23.23±4.17)months.Univariate analysis showed that the risk factors for survival were tumor TNM stage(χ^2=9.066,P=0.003),lymph node metastasis(χ^2=6.399,P=0.011)and distant metastasis(χ^2=9.808,P=0.002).By multivariate analysis,independent risk factors were tumor TNM stage(P=0.008,RR=3.003,95%CI:1.332~6.774),lymph node metastasis(P=0.023,RR=5.382,95%CI:1.261~22.971)and distant metastasis or not(P=0.007,RR=7.423,95%CI:1.730~31.851).Conclusion EB-NENs was a rare malignant tumor with poor prognosis.Tumor TNM stage,lymph node metastasis and distant metastasis were independent prognostic factors for EB-NENs patients.