Ji R H, Wang X T, Li R, Ye S B, Wang X, Ma H H, Lu Z F, Rao Q, Xia Q Y
Department of Pathology, Nanjing Jinling Hospital, Nanjing University School of Medicine, Nanjing 210002, China.
Zhonghua Bing Li Xue Za Zhi. 2022 Jan 8;51(1):23-27. doi: 10.3760/cma.j.cn112151-20210627-00461.
To study the clinical pathological characteristics, immunophenotype, molecular changes and prognosis of the papillary renal neoplasm with reverse polarity (PRNRP). Nine cases of PRNRP, diagnosed from 2013 to 2019, were retrieved from the Department of Pathology of Nanjing Jinling Hospital, Nanjing University School of Medicine. Histomorphology, immunophenotype and molecular genetics were analyzed with review of the literatures. There were five male and four female patients, aged from 49 to 70 years, with an average age of 60.1 years. During a mean follow-up of 29 months, one patient died for other cause, and the others survived without disease. Microscopically, the tumor cells arranged in papillary structure with a fibrovascular core, the surface of which was covered with a single layer of cuboidal or columnar cells. The most prominent feature was that the tumor nuclei located at the top of the cytoplasm far from the basement membrane, and they were monotonous in size and arranged neatly with no or few nucleoli. Immunohistochemically, all nine cases of PRNRP showed diffuse positive expression of CK7 and E-cadherin, various degrees of P504s expression, and no expression of CD10 and CD117, with a Ki-67 index of 1%-3%. Unlike other papillary renal cell carcinoma, the nine cases of PRNRP all showed characteristic positive expression of GATA3. The fluorescence in situ hybridization assay showed that the majority of PRNRPs (8/9) did not have triploids on chromosomes 7 and 17. The sequencing of the KRAS gene confirmed the presence of a nonsense KRAS mutation in 8 of the 9 cases. PRNRP is a subtype of papillary renal cell carcinoma with characteristic morphological, immunophenotypic and molecular features, and indolent behaviors. More data are needed to define PRNRP as "carcinoma", and a definitive diagnosis of PRNRP is of great significance for proper treatment choice and accurate prognostication.
研究具有反向极性的乳头状肾肿瘤(PRNRP)的临床病理特征、免疫表型、分子变化及预后。从南京大学医学院附属南京金陵医院病理科检索出2013年至2019年诊断的9例PRNRP。回顾文献分析其组织形态学、免疫表型及分子遗传学。患者中男性5例,女性4例,年龄49至70岁,平均年龄60.1岁。平均随访29个月,1例患者因其他原因死亡,其余患者无病生存。显微镜下,肿瘤细胞呈乳头状结构排列,有纤维血管轴心,其表面覆盖单层立方或柱状细胞。最显著的特征是肿瘤细胞核位于远离基底膜的细胞质顶部,大小单一,排列整齐,无或仅有少量核仁。免疫组化显示,9例PRNRP均显示CK7和E-钙黏蛋白弥漫性阳性表达,P504s呈不同程度表达,CD10和CD117无表达,Ki-67指数为1% - 3%。与其他乳头状肾细胞癌不同,9例PRNRP均显示GATA3特征性阳性表达。荧光原位杂交检测显示,大多数PRNRP(8/9)在7号和17号染色体上无三倍体。KRAS基因测序证实9例中有8例存在KRAS无义突变。PRNRP是乳头状肾细胞癌的一种亚型,具有特征性的形态、免疫表型和分子特征,行为惰性。需要更多数据来将PRNRP定义为“癌”,PRNRP的明确诊断对于正确的治疗选择和准确的预后判断具有重要意义。