hereditary conditions associated with RCC

What are some hereditary conditions and other risk factors associated with RCC?

  • • von Hippel Lindau (vHL) syndrome is associated with an increased risk of clear cell RCC (occurring in up to 40% of patients).
  • • Hereditary papillary renal carcinoma (HRPC) is an autosomal dominant condition that is associated with an increased risk of papillary RCC.
  • • Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant condition that is associated with various skin tumors and an increased risk of chromophobe RCC and renal oncocytomas. Cystic lung disease is also typically seen on CT.

In the setting of these hereditary conditions, RCC is more frequently bilateral and multifocal, and it tends to present at a younger age.

Other risk factors for RCC include tobacco use, obesity, exposure to various chemical compounds, and acquired cystic renal disease. Patients on long-term dialysis have a 3- to 6-fold increased risk of RCC compared to the general population.

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