title: [Hereditary renal tumors: More common than expected?].
abstract: Renal cell carcinomas are associated with hereditary tumor syndromes in approximately 5 % of cases. In patients with a hereditary predisposition, tumors show an earlier age of onset, often with a multicentric and bilateral manifestation. While some patients with renal cell carcinoma can be classified into well-characterized kidney cancer syndromes others have a genetic background which is still poorly understood. Most of the specific tumor syndromes are associated with a histopathologically distinct renal cell tumor phenotype. The recognition of patients with hereditary renal cell carcinoma and the identification of individual family members with a higher risk of development of renal tumors is important for early tumor detection and treatment. This manuscript reviews the clinical pathological and molecular findings of hereditary renal cell carcinoma syndromes.
authors.affiliation: Pathologisches Institut, Universität Erlangen-Nürnberg, Krankenhausstraße 8-10, 91054, Erlangen, Deutschland.
authors.forename: A
authors.initials: A
authors.lastname: Agaimy
authors.affiliation: Pathologisches Institut, Universität Erlangen-Nürnberg, Krankenhausstraße 8-10, 91054, Erlangen, Deutschland. arndt.hartmann@uk-erlangen.de.
authors.forename: A
authors.initials: A
authors.lastname: Hartmann
mesh_terms: D002292:Carcinoma, Renal Cell / Q000453:epidemiology* / Q000235:genetics* / Q000473:pathology; D003430:Cross-Sectional Studies; D042241:Early Diagnosis; D020022:Genetic Predisposition to Disease / Q000235:genetics; D005820:Genetic Testing; D006801:Humans; D007680:Kidney Neoplasms / Q000453:epidemiology* / Q000235:genetics* / Q000473:pathology; D006623:von Hippel-Lindau Disease / Q000453:epidemiology / Q000235:genetics / Q000473:pathology
keywords: Early tumor recognition; Genetic predisposition; Human genetic clarification; Renal cell carcinoma; Von Hippel-Lindau syndrome