Comparison of the Pathologic Spectrum of Neoplasia in Autosomal Dominant Polycystic Kidney Disease (ADPKD) and Acquired Cystic Kidney Disease in End-Stage Renal Disease (ACKD-ESRD).
Maria M Picken, Vikas Mehta. Loyola University Medical Center, Maywood, IL
Background: ACKD-ESRD and ADPKD are characterized by the development of multiple cysts in the kidneys. The development of renal cell carcinoma (RCC) is a recognized risk factor in ACKD-ESRD and patients are therefore subjected to active surveillance. In contrast, the risk of RCC in ADPKD is perceived to be small and not warranting surveillance. The objective of this study was to compare and contrast the pathologic spectrum of neoplasia as seen in ACKD-ESRD versus ADPKD versus the general population.
Design: We reviewed, retrospectively, all the nephrectomies performed in our department between 1985 and 2010 in patients with ADPKD and ACKD-ESRD. We compared the incidence and types of tumors encountered in ADPKD and ACKD-ESRD, and compared the results with published epidemiological data.
Results: 43 patients (19M/23F) with ADPKD underwent 73 nephrectomies; in 7/42, both kidneys were removed but not simultaneously. Mean age was 50.3 years (range, 31-71years). A review of the pathology revealed that 4 nephrectomies contained malignant tumors (prevalence = 5.4%). The histological subtype of the cancers was as follows: 3/4 clear cell RCC and 1/4 papillary RCC. All carcinomas were seen in males. Of 73 kidneys, 13 (17%) showed benign tumors: papillary adenomas (8), cystic nephroma (2), angiomyolipoma (1), and mixed epithelial and stromal tumor of the kidney (2). Eighty patients with ACKD-ESRD (44 males and 36 females) underwent 97 nephrectomies. Renal cell carcinoma was seen in 32 patients (25 males and 7 females), prevalence = 40%. The histological subtype of the cancers was as follows: clear cell RCC 50% (16/32), papillary RCC 21.8% (7/32), acquired cystic disease-associated RCC 21.8% (7/32), sarcomatoid RCC 3.1% (1/32), and chromophobe RCC 3.1% (1/32). Benign tumors were also identified as follows: Papillary adenomas in 17, cystic nephroma in 2, oncocytoma in 1 and collecting duct adenoma in 1 case.
Conclusions: Renal cell carcinoma arising in the setting of ADPKD as well as ACKD-ESRD is much more common in males. While, among neoplasms arising in ACKD-ESRD, there are tumors that are unique to this condition, tumors arising in APKD are similar to those seen in the general population; this includes both malignant and rarer, benign entities. While the risk of malignancy in ADPKD is smaller than in ACDK-ESRD, it is nevertheless 10x higher than in the general population. In both groups (ADPKD and ACKD-ESRD) the pts with RCC are a decade younger than the median age of pts with RCC in the general population.
Category: Genitourinary (including renal tumors)
Wednesday, March 2, 2011 9:30 AM
Poster Session V # 97, Wednesday Morning