摘要：乳头状肾细胞癌（papillary renal cell carcinoma，PRCC）有2种组织学亚型。近一半病例由于不完全符合其中某一亚型的所有形态学标准，被归为非特殊类型（not otherwise specified，NOS)PRCC。目前尚无标记物能解决这部分PRCC NOS的分类问题。准确的分类可以更好地指导临床治疗。在既往研究中，我们提出了相关的标记物对PRCC进行分型。本研究选取108例PRCC患者样本，对之前从基因组中筛选出的一组潜在鉴别标记物进行免疫组化分析。这组标记物在2种经典PRCC亚型之间的表达具有显著差异，且能对PRCC NOS病例（45%)再次分类。不仅如此，我们应用这些标记物鉴别出了第3种PRCC亚型PRCC3(占病例的35%)。我们利用miRNA表达和拷贝数变异分析证实这3种亚型存在3种不同的分子特征。单变量分析显示，PRCC1的无病生存期显著长于PRCC2和PRCC3(P=0.047)。多变量分析也显示亚型间有显著差别（P=0.025；风险比，6；95%置信区间，1.25~32.2)。结合形态学、免疫组织化学和分子表型，我们提出一种新的PRCC分型系统。最新提出的PRCC3具有与PRCC1和PRCC2重叠的形态学特征，在现有的分型系统中被归为NOS。PRCC3具有独特的分子信号，其临床表现与PRCC2相似。按照新分型系统对PRCC进行分组，这将对指导临床治疗具有重要意义。
关键词：乳头状肾细胞癌，乳头状肾细胞癌NOS，ABCC2
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