Abstract
Background:
Kidney cancer is not a homogenous entity; it is comprised of many different tumor types, with different biologies and molecular mechanisms leading to disease and therefore different treatment approaches.
Objective:
To describe the genetic basis and biochemical pathways underlying inherited forms of renal cancer, specifically in four described syndromes (von Hippel-Lindau [VHL], hereditary papillary renal cancer [HPRC], Birt-Hogg-Dubé [BHD] and hereditary leiomyomatosis renal cell carcinoma [HLRCC]), and to elucidate how the understanding of these diseases enables the possibility of disease-specific approaches to therapy.
Methods:
A systematic review of the published literature on inherited and sporadic forms of renal cancer was performed.
Conclusion:
Understanding of the biology and mechanisms of different forms of kidney cancer provides an opportunity for development of new treatment options.
Publication types
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Research Support, N.I.H., Intramural
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Review
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Systematic Review
MeSH terms
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Antineoplastic Agents / pharmacology
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Antineoplastic Agents / therapeutic use*
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Carcinoma, Renal Cell / genetics
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Carcinoma, Renal Cell / therapy
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Drug Design
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Fumarate Hydratase / deficiency
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Fumarate Hydratase / genetics
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Fumarate Hydratase / physiology
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Genes, Tumor Suppressor*
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Genetic Predisposition to Disease
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Humans
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Kidney Neoplasms / genetics*
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Kidney Neoplasms / therapy
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Leiomyomatosis / genetics
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Leiomyomatosis / therapy
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Neoplasm Proteins / antagonists & inhibitors
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Neoplasm Proteins / genetics
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Neoplasm Proteins / physiology
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Neoplasms, Multiple Primary / genetics
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Neoplasms, Multiple Primary / therapy
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Neoplastic Syndromes, Hereditary / genetics*
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Neoplastic Syndromes, Hereditary / therapy
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Proto-Oncogene Proteins / antagonists & inhibitors
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Proto-Oncogene Proteins / deficiency
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Proto-Oncogene Proteins / genetics
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Proto-Oncogene Proteins / physiology
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Proto-Oncogene Proteins c-met
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Proto-Oncogenes*
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Receptors, Growth Factor / antagonists & inhibitors
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Receptors, Growth Factor / deficiency
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Receptors, Growth Factor / genetics
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Receptors, Growth Factor / physiology
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Tumor Suppressor Proteins / deficiency
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Tumor Suppressor Proteins / genetics
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Tumor Suppressor Proteins / physiology
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Vascular Endothelial Growth Factor A / antagonists & inhibitors
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Von Hippel-Lindau Tumor Suppressor Protein / genetics
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Von Hippel-Lindau Tumor Suppressor Protein / physiology
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von Hippel-Lindau Disease / genetics
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von Hippel-Lindau Disease / therapy
Substances
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Antineoplastic Agents
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FLCN protein, human
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Neoplasm Proteins
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Proto-Oncogene Proteins
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Receptors, Growth Factor
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Tumor Suppressor Proteins
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VEGFA protein, human
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Vascular Endothelial Growth Factor A
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Von Hippel-Lindau Tumor Suppressor Protein
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MET protein, human
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Proto-Oncogene Proteins c-met
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Fumarate Hydratase
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VHL protein, human