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Article

Renal Cell Carcinoma in von Hippel–Lindau Disease—From Tumor Genetics to Novel Therapeutic Strategies

8 Feb 2018

Abstract excerpt

Von Hippel Lindau disease (VHL) is an autosomal dominant syndrome caused by mutations in the VHL tumor suppressor gene, leading to the dysregulation of many hypoxia-induced genes. Affected individuals are at increased risk of developing recurrent and bilateral kidney cysts and dysplastic lesions which may progress to clear cell renal cell carcinoma (ccRCC). Following the eponymous VHL gene inactivation, ccRCCs...

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PMID: 29479523
DOI: 10.3389/fped.2018.00016

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