Autosomal Dominant Polycystic Kidney Disease: Prevalence of Renal Neoplasias in Surgical Kidney Specimens

Abstract

Background: The role of autosomal dominant polycystic kidney disease (ADPKD) as a risk factor for renal cell carcinoma (RCC) is still under discussion. Data on prevalence of RCC in ADPKD are limited, especially on a large population scale. The aim of this study was to analyze the prevalence of RCC in ADPKD kidneys and characterize the clinical features of this coincidence. Methods: Based on our histopathological registry for ADPKD and the Else Kröner-Fresenius Registry, we retrospectively reviewed malignant and benign renal lesions in patients with ADPKD who had undergone renal surgery from 1988 to 2011. Results: 240 ADPKD patients underwent 301 renal surgeries. Mean age at surgery was 54 years. Overall, 16 malignant and 11 benign lesions were analyzed in 301 kidneys (5.3%; 3.7%), meaning that 12/240 (5%; 1:20) patients presented with malignant renal lesions. 66.7% (8/12) of these patients had undergone dialysis prior to surgery. We found 10/16 (63%) papillary RCC, 5/16 (31%) clear cell RCC, and 1/16 (6%) papillary noninvasive urothelial cancer. Regarding all renal lesions, 6/17 (35.3%) patients had more than one histological finding in their kidneys. In 2 cases, metachronous metastases were removed. Mean follow-up was 66.7 months. Conclusion: Kidney-related prevalence of RCC in ADPKD kidneys was surprisingly high. Whether or not this is due to chronic dialysis or due to the underlying disease is still speculative. Like other cystic renal diseases with an increased risk for RCC, the attending physician should be aware of the malignant potential of ADPKD, especially with concomitant dialysis.

The Future of Polycystic Kidney Disease Research—As Seen By the 12 Kaplan Awardees

“…42 Yet, in PKD, numerous studies have shown that very little RCC is seen 43 unless the kidneys are removed and examined histologically. 44,45 This very low level of diagnosed RCC in ADPKD suggested that there may be a degree of protection from cancer associated with PKD mutations. As such, we carried out an epidemiologic study to answer this question.…”

Polycystic Kidney Disease and Cancer after Renal Transplantation

“…7,8 This cyst formation is associated with cellular dedifferentiation and is considered a neoplastic process driven by upregulated proto-oncogenes. [9][10][11][12][13] While published case reports document the occurrence of renal cell carcinomas (RCCs) in ADPKD-affected kidneys, 14,15 these tumors may be partly due to acquired renal cystic disease resulting from long-term dialysis. 16 Because there do not appear to be widespread published reports of other cancers in patients with ADPKD, protective mechanisms might exist in ADPKD to prevent malignant transformation.…”

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“…This has been also mentioned as an indication of native nephrectomy. 52,53 In our study, one-fifth of the patients in the deceased-donor group and one-fifth of the patients in the living-donor group underwent native nephrectomy. There was no significant difference between the groups regarding the time of nephrectomy, with most of the nephrectomies that did not occur simultaneously performed unilaterally before kidney transplant.…”