Prevalence and Pathology of Renal Tumors in Autosomal Dominant Polycystic Kidney Disease (ADPKD) Versus Acquired Cystic Kidney Disease in End-Stage Renal Disease (ACKD-ESRD).
Vikas Mehta, Maria M Picken. Loyola University Medical Center, Maywood, IL
Background: ACKD-ESRD is a known risk factor for the development of renal cell carcinoma (RCC). In contrast, the risk for RCC in ADPKD is perceived to be small. The current prevalance of RCC in the general population of the USA is approximately 0.4%. ADPKD occurs in approximately 1-2/1000 live births and accounts for 10% of patients requiring dialysis/renal transplantation. The objective of this study is to compare the prevalence/pathology of renal tumors in ADPKD vs ACKD-ESRD vs the general population.
Design: We reviewed, retrospectively, all nephrectomies done at our Institution between 1985-2010, in patients (pts) with ADPKD and ACKD-ESRD. We compared the prevalence and types of tumors in both groups of patients and compared the data with published epidemiological data.
Results: The ADPKD group comprised 73 kidneys from 42 pts (19 M/23F); in 7/42, both kidneys were removed, but not simultaneously. Mean age was 50.3 yrs (range, 31-71yrs). The ACKD-ESRD group comprised 97 kidneys/80 pts (44M/36F). Mean age: 47.1 yrs (range 19-78 yrs). Four ADPKD kidneys showed RCC (5.4%): clear cell in 3/4, papillary RCC in 1/4; all in male patients. 13 ADPKD kidneys (17%) showed benign tumors: papillary adenomas -8, cystic nephroma-mixed epithelial and spindle cell tumors (CN-MEST) – 4, angiomyolipoma (AML) -1. Out of 80 ACKD-ESRD pts, RCC was seen in 32 pts (25M/7F): clear cell RCC in 16/32 pts (50%), papillary RCC in 7/32 pts (21.8%), acquired cystic disease associated RCC in 7/32 pts (21.8%), sarcomatoid RCC and chromophobe RCC in 1 pt each (3.1%). Of 97 kidneys with ACKD-ESRD, 17 contained papillary adenomas, 2 CN, 1 oncocytoma and 1 collecting duct (CD) adenoma.
|Clear cell RCC||3||16|
|ACKD ASSOCIATED RCC||0||7|