Clear-cell renal-cell carcinoma
Appearance
The Clear Cell Renal Cell Carcinoma (CCRCC) is a type of renal cell carcinoma.
Epidemiology
- Most commonly affects male patients in their sixties and seventies.
- Majority of CCRCC arise sporadically.
- Only 2 – 4% of the cases presenting as part of an inherited cancer syndrome, such as von Hippel-Lindau syndrome.
Microscopy
Generally, the cells have a clear cytoplasm, are surrounded by a distinct cell membrane and contain round and uniform nuclei.
Genetics
Cytogenetics
- Alterations of chromosome 3p segments occurs in 70 – 90% of CCRCCs
- Inactivation of von Hippel-Lindau disease (VHL) gene by gene mutation and promoter hypermethylation
- Gain of chromosome 5q
- Loss of chromosomes 8p, 9p, and 14q
Molecular genetics
Several frequently mutated genes were discovered in CCRCC: VHL, KDM6A/UTX, SETD2, KDM5C/JARID1C and MLL2. PBRM1 is also commonly mutated in CCRCC.
Histogenesis
CCRCC is derived from the proximal convoluted tubule.
Images
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Clear cell renal cell carcinoma. Macroscopy.
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Clear cell renal cell carcinoma. HE, x100.
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Clear cell renal cell carcinoma. Führman Grade = 1. HE, x400.