Abstract
Renal cell carcinoma is a term that represents multiple different disease processes, each driven by different genetic alterations, with distinct histology, and biological potential which necessitates divergent management strategies. This review discusses the genetic alterations seen in several forms of hereditary kidney cancer and how that knowledge can dictate when and how to intervene with a focus on the surgical management of these tumors.
Keywords:
Birt-Hogg-Dubé; Von Hippel-Lindau; genetic alterations; hereditary leiomyomatosis and renal cell cancer; kidney cancer; precision surgery; renal cell carcinoma.
Publication types
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Research Support, N.I.H., Intramural
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Review
MeSH terms
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Carcinoma, Renal Cell / classification
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Carcinoma, Renal Cell / genetics*
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Carcinoma, Renal Cell / pathology
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Carcinoma, Renal Cell / surgery
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Humans
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Kidney Neoplasms / classification
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Kidney Neoplasms / genetics*
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Kidney Neoplasms / pathology
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Kidney Neoplasms / surgery
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Leiomyomatosis / classification
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Leiomyomatosis / genetics*
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Leiomyomatosis / pathology
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Leiomyomatosis / surgery
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Mutation / genetics
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Neoplastic Syndromes, Hereditary / classification
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Neoplastic Syndromes, Hereditary / genetics*
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Neoplastic Syndromes, Hereditary / pathology
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Neoplastic Syndromes, Hereditary / surgery
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Skin Neoplasms / classification
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Skin Neoplasms / genetics*
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Skin Neoplasms / pathology
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Skin Neoplasms / surgery
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Succinate Dehydrogenase / genetics
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Tumor Suppressor Proteins / genetics
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Ubiquitin Thiolesterase / genetics
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Uterine Neoplasms / classification
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Uterine Neoplasms / genetics*
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Uterine Neoplasms / pathology
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Uterine Neoplasms / surgery
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Von Hippel-Lindau Tumor Suppressor Protein / genetics
Substances
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BAP1 protein, human
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Tumor Suppressor Proteins
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Succinate Dehydrogenase
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Von Hippel-Lindau Tumor Suppressor Protein
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Ubiquitin Thiolesterase
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VHL protein, human
Supplementary concepts
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Hereditary leiomyomatosis and renal cell cancer