[889] 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.

TABLE 1
 ADPKDACKD-ESRD
RCC PREVALANCE5.4%40%
PATHOLOGYADPKDACKD-ESRD
Clear cell RCC316
PRCC17
ACKD ASSOCIATED RCC07
CHROMOPHOBE01
SARCOMATOID RCC01
PAPILLARY ADENOMA817
CN-MEST42
ONCOCYTOMA01
AML10
CD ADENOMA01



Conclusions: In our series, the prevalence of RCC in ACKD-ESRD was 40% while, in APKD, it was 5.4%; this represents a 100-fold and 10-fold increase respectively, when compared with the general population. Patients with ACKD-ESRD and ADPKD were, on average, 1 decade younger than the general population with RCC. While ACKD-ESRD patients may harbor tumors that are unique to this entity, the spectrum of neoplasia in ADPKD appears to be similar to that in the general population. Further studies are needed to establish whether active surveillance of patients with ADPKD should be considered.
Category: Genitourinary (including renal tumors)

Tuesday, March 1, 2011 1:00 PM

Platform Session: Section A, Tuesday Afternoon

 

Close Window