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== Contributors ==

== Contributors ==

Daynna Wolff PhD FACMG

Daynna Wolff, PhD FACMG

Yajuan Liu, PhD

Yajuan Liu, PhD

Rajyasree Emmadi, MD

Rajyasree Emmadi, MD

Banumathy Gowrishankar, PhD

Banumathy Gowrishankar, PhD

Jane Houldsworth, PhD

Jane Houldsworth, PhD

'''Clear Cell RCC'''

'''Clear Cell RCC'''

This tumor type accounts for ~70% of all renal cell carcinoma cases (Eble JN, Sauter G, Epstein JI, Sesterhenn IA. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs. Lyon: IARC Press; 2004).

This tumor type accounts for ~70% of all renal cell carcinoma cases.<ref name=eble>Eble JN, Sauter G, Epstein JI, Sesterhenn IA. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs. Lyon: IARC Press; 2004.</ref>

== Description ==

== Description ==

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== Mutations (SNV/INDEL) ==

== Mutations (SNV/INDEL) ==

== Prognosis ==

== Prognosis ==

Loss of 14, loss of 9p, loss of 18q and highly complex genotype are associated with high risk histological features and adverse clinical outcome, also significantly associated with high risk histologic features: loss of 1p, 4p, 9q, 15q, 16q, 17p, 18p, 21q, 22q and gains of 3q, 5p, 7p, 7q, 17q

Loss of 14, loss of 9p, loss of 18q and highly complex genotype are associated with high risk histological features and adverse clinical outcome, also significantly associated with high risk histologic features: loss of 1p, 4p, 9q, 15q, 16q, 17p, 18p, 21q, 22q and gains of 3q, 5p, 7p, 7q, 17q.<ref>Wolff, Nicholas C, Andrea Pavía-Jiménez, Vanina T Tcheuyap, Shane Alexander, Mridula Vishwanath, Alana Christie, Xian-Jin Xie, Noelle S Williams, Payal Kapur, Bruce Posner, Renée M Mckay, and James Brugarolas. "High-throughput Simultaneous Screen and Counterscreen Identifies Homoharringtonine as Synthetic Lethal with Von Hippel-Lindau Loss in Renal Cell Carcinoma." Oncotarget 6.19 (2015): 16951-62.</ref> Losses of 1p, 4p, 4q, 8p, 9p, 9q, 13q, 14q and 18q correlate with higher grade and/or stage of the tumors <ref name=zhang>Zhang, Zhongfa, Bill Wondergem, and Karl Dykema. "A Comprehensive Study of Progressive Cytogenetic Alterations in Clear Cell Renal Cell Carcinoma and a New Model for CcRCC Tumorigenesis and Progression." Advances in Bioinformatics 2010 (2010): 14.</ref><ref>Wilhelm, Mónica, Joris A Veltman, Adam B Olshen, Ajay N Jain, Dan H Moore, Joe C Presti, Gyula Kovacs, and Frederic M Waldman. "Array-based Comparative Genomic Hybridization for the Differential Diagnosis of Renal Cell Cancer." Cancer Research 62.4 (2002): 957-60.</ref><ref>Moore LE, Jaeger E, Nickerson ML, Brennan P, De Vries S, Roy R, et al. Genomic copy number alterations in clear cell renal carcinoma: associations with case characteristics and mechanisms of VHL gene inactivation. Oncogenesis. 2012;1 doi: 10.1038/oncsis.2012.14.</ref><ref name=arai>Arai, Eri, Saori Ushijima, Hitoshi Tsuda, Hiroyuki Fujimoto, Fumie Hosoda, Tatsuhiro Shibata, Tadashi Kondo, Issei Imoto, Johji Inazawa, Setsuo Hirohashi, and Yae Kanai. "Genetic Clustering of Clear Cell Renal Cell Carcinoma Based on Array-comparative Genomic Hybridization: Its Association with DNA Methylation Alteration and Patient Outcome." Clinical Cancer Research : An Official Journal of the American Association for Cancer Research 14.17 (2008): 5531-9.</ref><ref>Gunawan, Huber, Holtrup, Von Heydebreck, Efferth, Poustka, Ringert, Jakse, and Füzesi. "Prognostic Impacts of Cytogenetic Findings in Clear Cell Renal Cell Carcinoma: Gain of 5q31-qter Predicts a Distinct Clinical Phenotype with Favorable Prognosis." Cancer Research 61.21 (2001): 7731-8.</ref><ref name=monzon>Monzon, Federico A, Karla Alvarez, Lief Peterson, Luan Truong, Robert J Amato, Joan Hernandez-Mcclain, Nizar Tannir, Anil V Parwani, and Eric Jonasch. "Chromosome 14q Loss Defines a Molecular Subtype of Clear-cell Renal Cell Carcinoma Associated with Poor Prognosis." Modern Pathology : An Official Journal of the United States and Canadian Academy of Pathology, Inc 24.11 (2011): 1470-9.</ref> and loss of 4, 9p and 14q have been reported as independent prognostic factors for survival in ccRCC.<ref name=zhang></ref><ref name=monzon></ref><ref>Klatte, Tobias, Allan J Pantuck, Jonathan W Said, David B Seligson, Nagesh P Rao, Jeffrey C Larochelle, Brian Shuch, Amnon Zisman, Fairooz F Kabbinavar, and Arie S Belldegrun. "Cytogenetic and Molecular Tumor Profiling for Type 1 and Type 2 Papillary Renal Cell Carcinoma." Clinical Cancer Research : An Official Journal of the American Association for Cancer Research 15.4 (2009): 1162-9.</ref><ref name=arai></ref><ref>Andrei Alimov, Maria Kost-Alimova, Jian Liu, Chunde Li, Ulf Bergerheim, Stefan Imreh, George Klein, and Eugene R Zabarovsky. "Combined LOH/CGH Analysis Proves the Existence of Interstitial 3p Deletions in Renal Cell Carcinoma." Oncogene 19.11 (2000): 1392.</ref><ref>Moch, H., J C Presti, G. Sauter, N. Buchholz, P. Jordan, M J Mihatsch, and F M Waldman. "Genetic Aberrations Detected by Comparative Genomic Hybridization Are Associated with Clinical Outcome in Renal Cell Carcinoma." Cancer Research 56.1 (1996): 27-30.</ref>

<ref>Wolff et al.</ref>.

Losses of 1p, 4p, 4q, 8p, 9p, 9q, 13q, 14q and 18q correlate with higher grade and/or stage of the tumors <ref>Zhang et al, Adv Bioinform, 2010

</ref><ref>Wilhelm et al, Cancer res 2002; Moore et al, Oncogenesis, 2012</ref>

<ref>Arai et al, Clin Canc Res 2008</ref><ref>Gunawan et al. Cancer Res 2001</ref>

<ref>Monzon et al, Mod Path 2011</ref>  

and loss of 4, 9p and 14q have been reported as independent prognostic factors for survival in ccRCC<ref>Zhang 2010</ref><ref>Monzon 2011</ref><ref>Klatte 2009</ref><ref>Arai 2008</ref><ref>Alimov et al Int J Oncol 2004; Moch et al 1996</ref>

== Therapeutics ==

== Therapeutics ==

[[Birt-Hogg-Dube syndrome]] ([[FLCN]] 17p11.2)

[[Birt-Hogg-Dube syndrome]] ([[FLCN]] 17p11.2)

==References==

==References==

{{Reflist}}

{{Reflist}}