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Clear Cell Renal Cell Carcinoma (CCRCC)

What is clear cell Renal Cell Carcinoma? Clear cell renal cell carcinoma (CCRCC), also known as conventional renal cell carcinoma (RCC), accounts for 80% of renal cell carcinomas (RCC). The other two classifications of RCC include papillary RCC, and chromophobe RCC. CCRCC occurs more commonly in adults after the age of 40, peaking between the ages of 60-80, but also do makeup 2-6% of childhood and young adult renal cancer.

Renal cell carcinomas are tumors of the renal cortex and renal tubular epithelial cells. The term CCRCC is derived from its microscopic appearance, as the cytoplasm often appears clear because of the accumulation of glycogen and lipids. Clear cell RCCs are typically solitary tumors, unless associated with familial syndromes that present with multiple tumors. 

What causes clear cell RCC?

The etiology of CCRCC is most often associated with genetic causes, but may also be associated with some external factors.

Genetic causes of  CCRCC include abnormalities in:

  • Von Hippel-Lindau (VHL) gene and is high risk in those diagnosed with VHL syndrome. Over 90% of cases with CCRCC have a deletion in 3p.
  • Protein polybromo-1 (PBRM-1) gene, which is a tumor suppressor gene.

Risk factors in acquired CCRCC include:

  • Long-term dialysis
  • Smoking
  • Obesity 
  • Diabetes
  • Hypertension
  • Long-term analgesic use

Signs and Symptoms of CCRCC

Most clear cell RCC are incidental findings on imaging for other indications and are asymptomatic. Some may have symptoms, including:

  • Hematuria
  • Pain
  • Weight Loss
  • Fatigue
  • Fever 
  • Palpable lump

Source: Clear Cell Renal Cell Carcinoma. (2020, March 17). National Cancer Institute. Retrieved July 1, 2022, from https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-kidney-tumors/clear-cell-renal-cell-carcinoma#:%7E:text=Clear%20cell%20renal%20cell%20carcinoma%2C%20or%20ccRCC%2C%20is%20a%20type,called%20conventional%20renal%20cell%20carcinoma