嗜酸细胞性乳头状肾癌19例临床病理学分析  

Oncocytic papillary renal cell carcinoma:a clinicopathological analysis of nineteen cases

作　　者：张伟 张龙宵 杨桐 邹玉玮 刘小玲 于文娟[2] 蒋艳霞[2] 李玉军[2] Zhang Wei;Zhang Longxiao;Yang Tong;Zou Yuwei;Liu Xiaoling;Yu Wenjuan;Jiang Yanxia;Li Yujun(Department of Pathology,971 Hospital of PLA Navy,Qingdao 266071,China;Department of Pathology,the Affiliated Hospital of Qingdao University,Qingdao 266003,China)

机构地区：[1]海军第九七一医院病理科,青岛266071 [2]青岛大学附属医院病理科,青岛266003

出　　处：《中华病理学杂志》2022年第10期981-986,共6页Chinese Journal of Pathology

基　　金：青岛市医药科研指导计划项目(2020-WJZD198);青岛大学附属医院“临床医学+X”科研项目(QDFY+X2021017)。

摘　　要：目的探讨嗜酸细胞性乳头状肾癌(oncocytic papillary renal cell carcinoma,OPRCC)的临床病理、免疫表型及分子学特征,并与1型乳头状肾细胞癌(PRCC)比较。方法收集2003年10月至2021年2月青岛大学附属医院(16例)和海军第九七一医院(3例)共19例具有嗜酸细胞形态乳头状肾癌的临床病理资料,进行组织学、免疫组织化学及分子学分析,并与同期诊断的15例1型PRCC比较。结果男性15例,女性4例,年龄47~78岁(中位年龄61岁)。13例系体检发现,4例因无痛性肉眼血尿、2例因腰痛就诊。病理分期:14例pT1期,1例pT2a期,3例pT3a期,1例pT4期。肿瘤直径1.7~14.0 cm,边界清楚,切面灰黄、灰红色,质软。镜下观察:瘤组织呈乳头状(10%~100%)和腺泡(管)状排列,瘤细胞胞质强嗜酸性,核圆形或不规则,核仁多明显[WHO/国际泌尿病理协会(ISUP)核分级Ⅲ级],2例伴有肉瘤样分化,所有病例均可见泡沫样巨噬细胞聚集。免疫组织化学:19例α-甲酰基辅酶A消旋酶(AMACR)均弥漫强阳性,肾细胞癌标志物(RCC,18/19)、CD10(17/19)、波形蛋白(16/19)、PAX8(17/19)在绝大多数肿瘤阳性,细胞角蛋白(CK)7(11/19)在约50%的病例表达。14例荧光原位杂交显示,8例存在第7号染色体三体、7例存在第17号染色体三体,二者同时发生的有7例,13例男性中8例Y染色体缺失。19例随访8~120个月,3例于术后8、62和82个月因转移死亡,1例术后36个月复发。与1型PRCC相比,OPRCC往往具有较高的核分级、间质泡沫细胞聚集更常见(P<0.05),CD10和上皮细胞膜抗原(EMA)在两者表达差异有统计学意义(P<0.01)。两组病例累积生存率差异无统计学意义(P=0.239)。结论OPRCC具有独特的形态学特点,免疫表型与1型PRCC有重叠但存在差异,分子学结果支持其属于PRCC的形态学变异。该肿瘤与1型PRCC具有相似的生物学行为,出现肉瘤样分化时预后较差。Objective To investigate the clinicopathological characteristics,immunophenotype,and molecular signatures of oncocytic papillary renal cell carcinoma(OPRCC),and to compare these findings with those in type 1 papillary renal cell carcinoma(PRCC 1).Methods The clinicopathologic data of 19 patients with OPRCC from the Affiliated Hospital of Qingdao University(16 patients)and the 971 Hospital of People′s Liberation Army Navy(3 patients)from October 2003 to February 2021 were collected.Histologic,immunohistochemical(IHC)and molecular analyses,together with a control group of 15 cases of PRCC I diagnosed in the same period,were assessed.Results The cohort included 15 males and 4 females,with a median age of 61 years(range,47-78 years).In 13 patients the tumors were found at physical examination;four presented with painless gross hematuria and two with low back pain.As for the pathologic stage,14 patients were pT1,one patient was pT2a,three patients were pT3a and one patient was pT4.The tumor size ranged from 1.7-14.0 cm,with clear boundary and soft texture.The cut surface was grayish-yellow and grayish-red.Microscopically,the tumor cells were mainly arranged in papillary(10%-100%)and acinar(tubular)patterns,with strongly eosinophilic cytoplasm,round or irregular nuclei,and prominent nucleoli(WHO/ISUP gradeⅢ).Two cases showed sarcomatoid differentiation.Stromal foamy macrophages were visible in all cases.IHC staining showed diffuse strong positivity for AMACR in all cases.RCC(18/19),CD10(17/19),vimentin(16/19)and PAX8(17/19)were positive in most tumors.CK7 was expressed in about 50%of cases.Fluorescence in situ hybridization identified trisomy 7 in eight patients,trisomy 17 in seven patients,and the two aberrations occurred simultaneously in seven cases.Eight of 13 men had Y chromosome deletion.All patients were followed up for 8-120 months.Three patients died of metastases at 8,62 and 82 months postoperatively,respectively,and one patient relapsed 36 months after surgery.Compared with PRCC1,OPRCC tended to have hig

关 键 词：肾肿瘤 诊断 鉴别 免疫组织化学 预后 

分 类 号：R737.11[医药卫生—肿瘤]