Abstract

Objectives: The relationship between autosomal dominant polycystic kidney disease (ADPKD) and renal cell carcinoma (RCC) is investigated to determine a link that would guide management due to elevated RCC risk. Current literature is inconclusive on this topic. Methods: This study is a retrospective chart review of patients having undergone nephrectomy. Those with pathology and history consistent with ADPKD were reviewed for presence of RCC. Results: The review at this institution revealed RCC in 18% of ADPKD patients who underwent nephrectomy. These rates are significantly higher than those found in the general population, and even greater than those would be expected in patients suffering end-stage renal disease (ESRD). Conclusions: Due to the increased prevalence of RCC in ADPKD, clinicians managing patients with ADPKD should maintain a high index of suspicion. Our data suggest a link between ADPKD and RCC, most likely at the genetic level. In light of this, we feel that further genetic research is needed to potentially discover the link between these two disease processes.

Highlights

  • Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal cystic diseaseHow to cite this paper: Hansen, C.C., Derrick, M., Warriach, I., Underwood, L.F., Cammack, J.T. and de Riese, W. (2015) The Association between Autosomal Dominant Polycystic Kidney Disease and Renal Cell Carcinoma

  • Pubmed was systematically reviewed using “renal cell carcinoma” and “autosomal polycystic kidney disease” as keywords and subsequent survey for case reports and case series, yielding 42 papers published within the last 10 years regarding the incidence of Renal cell cancer (RCC) in ADPKD patients

  • Of these cases there were 2 out of 16 pathology reports that revealed the presence of RCC (13%)

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Summary

Introduction

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal cystic diseaseHow to cite this paper: Hansen, C.C., Derrick, M., Warriach, I., Underwood, L.F., Cammack, J.T. and de Riese, W. (2015) The Association between Autosomal Dominant Polycystic Kidney Disease and Renal Cell Carcinoma. (2015) The Association between Autosomal Dominant Polycystic Kidney Disease and Renal Cell Carcinoma. Renal cell cancer (RCC), on the other hand, is a fairly rare disorder affecting only 10 - 20 per 100,000 [4]. The connection between these two diseases has been postulated, but currently, ADPKD is not held to be a risk factor for the development of RCC. This may be secondary to the limited number of cases, as well as confounding factors. The goal of this study is to update the data and to review the current literature regarding this clinical entity

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