Clear cell renal cell carcinoma

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

Video Clear cell 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.

Maps Clear cell renal cell carcinoma

Microscopy

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

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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.

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Histogenesis

CCRCC is derived from the proximal convoluted tubule.

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Images

Source of the article : Wikipedia