More formally known as renal cancer or renal cell carcinoma (RCC), there are four primary forms of inherited kidney cancer:
- Von Hippel Lindau (VHL) for clear cell RCC, based on mutations in the VHL gene;
- VHL clinical features include tumors developing in both kidneys, adrenal glands, pancreas, brain or spine, eyes, or inner ears.
- hereditary papillary renal carcinoma for type 1 papillary RCC, based on mutations in the MET gene;
- Birt Hogg Dube for chromophobe RCC, based on mutations in the FLCN gene;
- hereditary Leiomyomatosis renal cell carcinoma for type 2 papillary RCC, based on mutations in the FH gene.