原发性肾神经内分泌肿瘤的临床病理特点及预后分析  

Clinical characteristics and prognostic analysis of renal primary neuroendocrine neoplasms

作　　者：文春森 张龙[1] 刘宇[1] 吴启哲 姚文诚[1] 李松超[1] 贾占奎[1] 杨锦建[1] Wen Chunsen;Zhang Long;Liu Yu;Wu Qizhe;Yao Wencheng;Li Songchao;Jia Zhankui;Yang Jinjian(Department of Urology,First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院泌尿外科,郑州450052

出　　处：《中华泌尿外科杂志》2022年第6期423-429,共7页Chinese Journal of Urology

摘　　要：目的探讨原发性肾神经内分泌肿瘤的临床病理特点及预后情况。方法回顾性分析2011年10月至2021年6月郑州大学第一附属医院收治的42例肾神经内分泌肿瘤患者的临床资料。男17例,女25例;中位年龄60.0(50.0,67.0)岁;表现为腰腹痛20例,血尿7例,腹胀4例,无症状11例;肿瘤位于左侧23例,右侧19例;肿瘤直径8.0(4.8,10.5)cm。CT增强扫描病变呈轻度强化,强化程度低于正常肾实质。临床分期:T1期6例,T2期11例,T3期11例,T4期14例;淋巴结转移17例;远处转移11例。36例行手术治疗,其中27例行根治性肾切除术,5例行肾部分切除术,4例行介入栓塞手术;6例未行手术治疗,其中5例行单纯化疗,1例行支持治疗。根据2016 WHO泌尿系统与男性生殖器官肿瘤分类,肾神经内分泌肿瘤分为高分化肾神经内分泌肿瘤(简称为NET,包含类癌和不典型类癌)和高级别肾神经内分泌癌(简称为NEC,包含小细胞神经内分泌癌和大细胞神经内分泌癌)。比较两种病理类型患者的临床病理特征并采用log-rank检验对总体进行单因素分析,采用Cox回归模型分析影响患者预后的危险因素。结果NET组19例,其中类癌12例,不典型类癌7例;NEC组23例,均为小细胞神经内分泌癌。全组42例均获随访,中位随访时间60(35,99)个月,中位生存时间25(15,60)个月;3、5年总生存率分别为40.0%和21.2%。NET组3、5年总生存率分别为72.6%和42.3%。NEC组3、5年总生存率分别为6.3%和0。NET组Ki-67指数中位值3%(2%,10%),术后化疗2例,初诊后3例发生早期(6个月内)进展。NEC组Ki-67指数中位值60%(40%,80%),术后化疗10例,初诊后13例发生早期进展。两组在初始治疗方式、术后化疗、Ki-67指数、病情早期进展方面差异均有统计学意义(P<0.05)。单因素分析结果显示,性别、年龄、病情早期进展、初始治疗方式、肿瘤分化、Ki-67指数均是影响患者预后的因素(P<0.05);多因素分析结果显示,NEC(HR=13.964,PObjective To investigate the clinicopathological characteristics,treatment methods and prognosis of renal primary neuroendocrine neoplasms.Methods The clinical data of 42 patients with renal neuroendocrine neoplasms admitted to the First Affiliated Hospital of Zhengzhou University from October 2011 to June 2021 were retrospectively analyzed.There were 17 males and 25 females.The median age was 60.0(50.0,67.0)years old.The CT enhancement scan lesion was slightly intensified with less intensification than normal renal parenchyma.The clinic manifestation included lumbar abdominal pain in 7 cases,hematuria in 3 cases,abdominal distension in 1 cases,and asymptomatic in 8 cases.The average diameter of tumor was 8.0(4.0,10.0)cm.The tumor of 13 cases was in the left,and 6 cases was in the right.6 cases were in T1 stage,11 cases were in T2,11 cases were in T3,and 14 cases were in T4.17 cases had lymph node metastasis,11 cases had distant metastasis.The surgical method was radical nephrectomy in 27 cases,nephrectomy in 5 cases and interventional embolization in 4 case,and no operation in 6 cases,including 5 with chemotherapy alone and 1 with supportive care.Patients were classified by WHO Classification of renal tumors of the urinary system and the male reproductive organs(2016)into high-differentiated renal neuroendocrine tumors(NET,including carcinoid and atypical carcinoids)and high-grade renal neuroendocrine carcinoma(NEC,including small cell neuroendocrine carcinoma and large cell neuroendocrine carcinoma).The clinicopathological characteristics and prognosis of the 2 groups were compared,and the Cox proportional regression risk model was used to analyze the clinical factors affecting the prognosis.Results In the NET group,12 cases were carcinoids and 7 cases were atypical carcinoids.In the NEC group,23 cases were small cell carcinomas.The mean Ki-67 index of 42 cases was 35%(4.5%,62.5%).The proportion of positive expression of the neuroendocrine markers CD56,chromogranin A(CgA),and synapsin(Syn)were(37/42),(15/42),an

关 键 词：肾肿瘤 癌 神经内分泌肿瘤 预后 

分 类 号：R737.11[医药卫生—肿瘤]