Clear cell renal cell carcinoma

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

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

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