Clear cell renal cell carcinoma

The Clear Cell Renal Cell Carcinoma (CCRCC) is a type of renal cell carcinoma.

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.

Microscopy

Generally, the cells have a clear cytoplasm, are surrounded by a distinct cell membrane and contain round and uniform nuclei.

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.

Images
  • Clear cell renal cell carcinoma. Macroscopy.
  • Clear cell renal cell carcinoma. HE, x100.
  • Clear cell renal cell carcinoma. Führman Grade = 1. HE, x400.

References
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