Department of Laboratory Medicine and Pathology, Hamad Medical Corporation, Doha, Qatar.
Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
Adv Anat Pathol. 2019 Mar;26(2):124-132. doi: 10.1097/PAP.0000000000000220.
Papillary renal cell carcinoma (PRCC) is the second most common type of renal carcinoma following clear cell renal cell carcinoma. Papillary renal cell carcinoma is usually divided histologically into 2 types namely, type 1 and type 2. This classification, however, is unsatisfactory as many of papillary carcinoma are unclassifiable by the existing criteria. In recent years there has been a remarkable progress in our understanding of the molecular basis of PRCC. These studies have revealed that type 2 PRCCs represent a heterogenous group which may be subdivided into additional subtypes based on the genetic and molecular make up of these tumors and reflecting different clinical course and prognosis. Some of the molecular features such a hypermethylation of CPG islands in the promotor regions of genes and over expression of the antioxidant pathways within tumor cells have been recognized as markers of poor prognosis. Targeted therapies for papillary carcinoma in the past have been unsuccessful because of lack of clear understanding of the molecular basis of these tumors. It is hoped that recent progress in our understanding of the pathogenesis of various subtypes of PRCC, effective targeted therapies will eventually emerge in due course.
乳头状肾细胞癌（PRCC）是继透明细胞肾细胞癌之后第二常见的肾癌类型。乳头状肾细胞癌通常在组织学上分为 2 种类型，即 1 型和 2 型。然而，这种分类并不令人满意，因为许多乳头状癌不符合现有标准的分类。近年来，我们对 PRCC 的分子基础有了显著的认识。这些研究表明，2 型 PRCC 代表了一个异质性群体，根据这些肿瘤的遗传和分子组成，可以进一步细分为其他亚型，反映出不同的临床过程和预后。一些分子特征，如基因启动子区域 CpG 岛的高甲基化和肿瘤细胞内抗氧化途径的过度表达，已被认为是预后不良的标志物。过去针对乳头状癌的靶向治疗之所以不成功，是因为对这些肿瘤的分子基础缺乏清晰的认识。希望我们对各种亚型 PRCC 发病机制的理解的最新进展，最终将出现有效的靶向治疗方法。
