常染色体显性多囊肾病相关多灶性肾细胞癌：一项叙述性影像学综述
Autosomal Dominant Polycystic Kidney Disease-Related Multifocal Renal Cell Carcinoma: A Narrative Iconographic Review.
作者信息
Sergi Consolato M, Guerra Luis, Hager Josef
机构信息
Anatomic Pathology, Children's Hospital of Eastern Ontario (CHEO), University of Ottawa, Ottawa, ON K1H 8L1, Canada.
Pediatric Urology, Children's Hospital of Eastern Ontario (CHEO), University of Ottawa, Ottawa, ON K1H 8L1, Canada.
出版信息
Int J Mol Sci. 2025 Apr 23;26(9):3965. doi: 10.3390/ijms26093965.
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common inheritable disease of cystic degeneration in the kidney. ADPKD is a significant cause of end-stage renal disease (ESRD). Autosomal Dominant Polycystic Liver Disease (ADPLD) results in substantial PLD with minimal PKD. Currently, there are eight genes which have been associated with ADPKD ( and ), ADPLD (, , , , and ), or both (). The severity of ADPKD can show an extremely broad range, but the evolution to ESRD is doubtless unavoidable. In some patients, carcinogenesis develops with inflammation as a potential promoting factor. In this chapter, we illustrate the severity of ADPKD and the fate to develop renal cell carcinoma (RCC).
常染色体显性多囊肾病（ADPKD）是最常见的肾脏囊性退变遗传性疾病。ADPKD是终末期肾病（ESRD）的一个重要病因。常染色体显性多囊肝病（ADPLD）导致大量肝囊肿而肾囊肿极少。目前，有8个基因与ADPKD（ 和 ）、ADPLD（ 、 、 、 和 ）或两者都有关（ ）。ADPKD的严重程度差异极大，但发展为ESRD无疑是不可避免的。在一些患者中，炎症作为潜在的促发因素会引发癌变。在本章中，我们阐述了ADPKD的严重程度以及发展为肾细胞癌（RCC）的转归。
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