title: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Nephrology, Section of Preventive Medicine, Albert Ludwigs University, Freiburg, Germany. cordula.jilg@uniklinik-freiburg.de
authors.forename: Cordula A
authors.initials: CA
authors.lastname: Jilg
authors.forename: Vanessa
authors.initials: V
authors.lastname: Drendel
authors.forename: Janina
authors.initials: J
authors.lastname: Bacher
authors.forename: Przemyslaw
authors.initials: P
authors.lastname: Pisarski
authors.forename: Hannes
authors.initials: H
authors.lastname: Neeff
authors.forename: Oliver
authors.initials: O
authors.lastname: Drognitz
authors.forename: Malte
authors.initials: M
authors.lastname: Schwardt
authors.forename: Sven
authors.initials: S
authors.lastname: Gläsker
authors.forename: Angelica
authors.initials: A
authors.lastname: Malinoc
authors.forename: Zoran
authors.initials: Z
authors.lastname: Erlic
authors.forename: Mercedes
authors.initials: M
authors.lastname: Nunez
authors.forename: Alexander
authors.initials: A
authors.lastname: Weber
authors.forename: Pablo
authors.initials: P
authors.lastname: Azurmendi
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Schultze-Seemann
authors.forename: Martin
authors.initials: M
authors.lastname: Werner
authors.forename: Hartmut P H
authors.initials: HP
authors.lastname: Neumann
mesh_terms: D002292:Carcinoma, Renal Cell / Q000453:epidemiology* / Q000473:pathology* / Q000601:surgery; D015897:Comorbidity; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D007680:Kidney Neoplasms / Q000453:epidemiology* / Q000473:pathology* / Q000601:surgery; D008297:Male; D008875:Middle Aged; D016891:Polycystic Kidney, Autosomal Dominant / Q000453:epidemiology* / Q000473:pathology* / Q000601:surgery; D015995:Prevalence; D006435:Renal Dialysis / Q000706:statistics & numerical data*; D018570:Risk Assessment