Is polycystic kidney disease associated with malignancy in children?. Issue 7 (14th June 2019)

• Record Type: Journal Article
• Authors: Friend, Brian D.; Wolfe Schneider, Kami; Garrington, Timothy; Truscott, Laurel; Martinez‐Agosto, Julian A.; Venick, Robert S.; Tsai Chambers, Eileen; Weng, Patricia; Farmer, Douglas G.; Chang, Vivian Y.; Federman, Noah
• Is Part Of: Molecular genetics & genomic medicine. Volume 7:Issue 7(2019)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2019-06-14
• Subjects: genetic predisposition -- genetic sequencing -- malignancy -- polycystic kidney disease; Medical genetics -- Periodicals; Genomics -- Periodicals; 616.042
• DOI: 10.1002/mgg3.725
• Languages: English
• ISSNs: 2324-9269

Abstract

Abstract: Background: Polycystic kidney disease (PKD) is an inherited condition characterized by progressive development of end‐stage renal disease, hypertension, hepatic fibrosis, and cysts in the kidney, liver, pancreas, spleen, thyroid, and epididymis. While malignancies have been reported in association with PKD in adults, the incidence of malignancies in children with PKD is not currently known. Methods: We report on five patients with a known history of PKD who developed a malignancy as children at the University of California, Los Angeles and the University of Colorado Anschutz Medical Campus. Patients were included from 2012 to 2017. Results: We present five patients with a history of PKD diagnosed with a malignancy during childhood without any additional known mutations to suggest a genetic predisposition to develop cancer. This includes the first reported case of hepatocellular carcinoma in a patient with autosomal recessive polycystic kidney disease. Conclusion: Our report illustrates the potential that PKD may be associated with an increased risk for developing cancer, even in children. Further research is necessary to better understand this relationship. Abstract : Polycystic kidney disease (PKD) is a heterogenous disease and its association with malignancy is not clear from past studies. We present five children with PKD who developed cancer at an early age to demonstrate a potential link between PKD and malignancy.