Renal cell carcinoma causes

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Farima Kahe M.D. [2]

Overview

The causes of renal cell carcinoma include von hippel-lindau (VHL), hereditary paragangliomas, leiomyomatosis, birt-hogg-dube syndrome, and several other genetic factors.

Causes

Sporadic development of renal cell carcinomas contributes to the majority of cases of the most common renal cell carcinoma, the conventional (or clear cell) carcinoma.[1][2][3]

The following tables show the sporadic and hereditary renal-cell carcinomas and associated causes:

Sporadic Forms of Renal Cell Carcinoma

Sporadic Forms of Renal Cell Carcinoma[4]
Histologic Appearance Incidence Gene Frequency
Conventional (Clear Cell) 75 VHL 60 %
Papillary 12 MET
TFE3
13 %
< 1 %
Chromophobe 4
Oncocytoma 4
Collecting Duct < 1
Unclassified 3 - 5
Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma.N Engl J Med. 2005; 353:2477-90

Hereditary Forms of Renal Cell Carcinoma

Hereditary Forms of Renal Cell Carcinoma[4]
Syndrome Associated Gene Common Histological Appearance
VHL Disease VHL Conventional (Clear Cell)
FCRC Chromosome 3p translocation Conventional (Clear Cell)
Hereditary Paraganglioma SDHB Conventional (Clear Cell)
HPRC MET Papillary
HLRCC FH Papillary
Birt-Hogg-Dube Syndrome BHD Chromophobe / Oncocytoma
Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma.N Engl J Med. 2005; 353:2477-90

References

  1. Haas NB, Nathanson KL (January 2014). "Hereditary kidney cancer syndromes". Adv Chronic Kidney Dis. 21 (1): 81–90. PMC 3872053Freely accessible. PMID 24359990. doi:10.1053/j.ackd.2013.10.001. 
  2. Linehan WM, Srinivasan R, Schmidt LS (May 2010). "The genetic basis of kidney cancer: a metabolic disease". Nat Rev Urol. 7 (5): 277–85. PMC 2929006Freely accessible. PMID 20448661. doi:10.1038/nrurol.2010.47. 
  3. Linehan WM (November 2012). "Genetic basis of kidney cancer: role of genomics for the development of disease-based therapeutics". Genome Res. 22 (11): 2089–100. PMC 3483538Freely accessible. PMID 23038766. doi:10.1101/gr.131110.111. 
  4. 4.0 4.1 Cohen HT, McGovern FJ (2005). "Renal-cell carcinoma.". N Engl J Med. 353 (23): 2477–90. PMID 16339096. doi:10.1056/NEJMra043172. 

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