摘要目的探讨早发性肾癌在亚裔人群中的发病特点和病理特征.方法 回顾性分析2010-2015年录入SEER数据库的亚裔肾癌患者(研究队列)及2014年1月至2018年5月上海交通大学医学院附属仁济医院收治的肾癌患者(验证队列)的临床资料.将两队列患者按年龄分为早发组(≤46岁)和对照组(＞46岁).研究队列3 023例,年龄10 ～ 93岁,平均61岁;早发组占13.4％(406/3 023).验证队列2 702例,年龄15～89岁,平均57岁;早发组占20.2％ (546/2 702).分别比较两队列中早发组与对照组的临床、病理特征.结果早发组中透明细胞癌比例低于对照组[研究队列76.8％ (312/406)与84.8％ (2 219/2 617);验证队列84.3％ (415/492)与88.5％(1 848/2 088)],而嫌色细胞癌比例显著高于对照组[研究队列12.1％(49/406)与6.2％(163/2617);验证队列11.2％(56/492)与4.8％ (101/2 088)],差异均有统计学意义(P＜0.01).验证队列早发组中Xp11易位性肾细胞癌的比例为3.8％(21/546),高于对照组[0.3％ (7/2 156)];Von Hippel-Lindau(VHL)综合征的比例为2.0％(11/546),高于对照组[0.3％,(7/2 156)].早发组早期(T1～72期)肾癌比例显著多于对照组[研究队列85.5％(347/406)与78.1％(2 045/2 617),P=0.037;验证队列96.1％(473/492)与91.2％(1 905/2 088),P＜0.01)],低级别(Ⅰ～Ⅱ级)肾癌比例也明显多于对照组[研究队列58.1％(236/406)与53.4％(1 397/2 617),P=0.043;验证队列85.2％(419/492)与79.4％(1 658/2 088),P＜0.01].研究队列的中位随访时间为26个月(1～71个月),随访率为96.0％(2 901/3 023),早发组1、3、5年生存率分别为94.0％、92.1％、92.1％,对照组分别为89.7％、81.1％、74.2％,两组比较差异有统计学意义(P＜0.01).结论相比于晚发性肾癌,亚裔人群中早发性肾癌具有嫌色细胞癌比例高、Xp11易位性肾细胞癌和VHL综合征发病率高、病理T分期早且肿瘤分级低的特点,预后好于晚发性肾癌.

abstractsObjective To identify clinicopathologic features of early onset RCC in Asian population.Methods Surveillance,Epidemiology,End Results Registry (SEER) database were queried for cases diagnosed as RCC in Asia or pacific islander between 2010 and 2015.Patients diagnosed with RCC in Renji hospital during 2014-2018 were reviewed.All Patients was divided into two group,including early onset group (≤46 years) and control group (＞46 years).There were 3 023 patients with average age of 61 years old,ranging from 10-93 years old in SEER cohort and 2 702 patients with average age of 57 years old,ranging from 15-89 years old in Renji cohort.Early onset group took up 13.4％ (406/3 023) in SEER cohort and 20.2％ (546/2 702) in Renji cohort.Clinicopathologic characteristics were compared between groups in both cohorts.Results The histologic spectrum of early onset group was significantly different from control group comprising fewer clear cell renal cell carcinoma(76.8％ vs.84.8％ in SEER cohort;84.3％ vs.88.5％ in Renji cohort),more chromophobe renal cell carcinoma(12.1％ vs.6.2％ in SEER cohort;11.2％ vs.4.8％ in Renji cohort) (P ＜ 0.01 in both cohorts).21 cases of Xp 11 translocation RCCs were identified in Renji cohort taking up 3.8％ (21/546) of early onset group which was higher than that in control group (0.3 ％,7/2 156),Von Hippel-Lindau syndromes took up 2.0％ (11/546) of early onset group larger than control group (0.3％,7/2 156).In addition,early onset RCC was more likely to be classified into lower pathological T stage(85.5％ vs.78.1％ P =0.037 in SEER cohort;96.1％ vs.91.2％ P ＜ 0.01 in Renji cohort)containing more low-grade tumors (58.1％ vs.53.4％,P =0.043 in SEER cohort;85.2％ vs.79.4％,P ＜0.01 in Renji cohort).The overall follow-up rate of SEER cohort was 96.0％ (2 901/3 023),follow-up time ranges from 1 to 71 months with a median of 26 months.The 1-year,3-year and 5-year overall survival rate were 94.0％ 、92.1％ 、92.1％ in early onset group and 89.7％、81.1％ 、74.2％ in control group,the differences were significant in statistics (P ＜ 0.01).Conclusions Asians who developed early onset RCC present with more ChRCC and fewer ccRCC compared to the older patients.Xp 11 translocation RCCs and VHL disease frequently occurred in younger group rather than the old counterparts.Younger patients diagnosed with RCC usually manifest lower T stage and tumor grade with a favorable prognosis.