Cancer Report

Cancer : Kidney: Clear cell renal cell carcinoma
Summary
Cancer Name Kidney: Clear cell renal cell carcinoma
Cancer Alias Common renal cell carcinoma
Conventional renal cell carcinoma, Non papillary renal cell carcinoma
Description Clear cell renal cell carcinoma (cRCC) is a distinct subtype of renal cell carcinoma, possibly originating from mature renal tubular cells in the proximal tubule of the nehpron.
 
Clinics and Pathology
Epidemiology It comprises 70-75% of cases.They show a male preponderance of 2:1.
Pathology The tumor mass of cRCC is multicolored, with a predominantly yellow surface with white or gray foci. It usually shows a solid growth pattern, but in some cases cystic or alveolar appearance is seen. The cytoplasm is clear, due to an intensive intracytoplasmatic accumulation of glycogen and lipids. The nuclei are condensed and hyperchromatic. Electronmicroscopical features resembling the proximale tubule can be found i.e. brush border formation and basal infoldings. Tumor cells express antigens of the proximal tubule.

Variants can be assigned which are characterized by augmentation of mitochondria leading to a stronger eosinophilia or granularity, respectively, of the cytoplasm. Spindle-shaped/pleomorphic variants as a result of sarcomatoid transformation can also be found.


 
Related Genes
Gene Symbol SBK
Description From COSMIC:
 
Gene Symbol TOPORS
Description From COSMIC:
 
Gene Symbol DDR2
Description From COSMIC:
 
Gene Symbol INSRR
Description From COSMIC:
 
Gene Symbol FLT4
Description From COSMIC:
 
Gene Symbol PRKWNK3
Description From COSMIC:
 
Gene Symbol RIOK2
Description From COSMIC:
 
Gene Symbol ATM
Description From COSMIC:
 
Gene Symbol MERTK
Description From COSMIC:
 
Gene Symbol MAPK8
Description From COSMIC:
 
Gene Symbol TEK
Description From COSMIC:
 
Gene Symbol ALS2CR7
Description From COSMIC:
 
Gene Symbol MAST4
Description From COSMIC:
 
Gene Symbol VHL
Description From COSMIC:
 
Gene Symbol ICK
Description From COSMIC:
 
Gene Symbol RB1
Description From COSMIC:
 
Gene Symbol CDKN2A
Description From COSMIC:
 
Gene Symbol CTNNB1
Description From COSMIC:
 
Gene Symbol PTENP1
Description From COSMIC:
 
Gene Symbol IGF1R
Description From COSMIC:
 
Gene Symbol BMPR1A
Description From COSMIC:
 
Gene Symbol RASSF1
 
Cytogenetics
Cytogenetics Morphological cRCC is characterized by loss of (part of) the short arm of chromosome 3 due to (a) deletion(s) or unbalanced translocation(s) and restricted to this type. Regions frequently lost are 3p12-14, 3p21 and 3p25. Loss of at least two of these regions is necessary for kidney cells to develop into clear cell renal cell carcinoma, and loss of 3p21 is obligatory. Therefore, if a tumor shows only one deletion at 3p, either 3p14 or 3p25, it should be designated common type renal cell adenomas.

Other aberrations frequently found in common RCC are (partial) trisomy of chromosome 5, especially the 5q22-qter segment. Trisomy 12, and 20, and loss of chromosomes 8, 9, 13q, 14q, and structural abnormalities of the long arm of chromosomes 6 and 10 are also found and correlated with progression.

In general, cRCCs are sporadic tumors but also syndromic in patients with the von Hippel-Lindau (VHL) disease (germ line mutations in the VHL tumor suppressor gene assigned to 3p25). Also familial cRCC familial cRCC has been reported. All have in common the presence of abnormalities involving chromosome 3.

 
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Source and Citation
Source Atlas of Genetics and Cytogenetics in Oncology and Haematology
Citation van den Berg E, Störkel S . Kidney: Clear cell renal cell carcinoma. Atlas Genet Cytogenet Oncol Haematol. July 2004 .
URL : http://AtlasGeneticsOncology.org/Tumors/ClearCellRenalCC5020.html
URL http://AtlasGeneticsOncology.org/Tumors/ClearCellRenalCC5020.html