Renal Cell Carcinoma (RCC) - Genetic Risk
Who should be tested?
- Genetic testing is recommended for those with a hereditary renal cell carcinoma syndrome based on any of the following:
- 1st or 2nd degree relative with RCC diagnosis
- Diagnosed ≤46 years old
- Bilateral or multifocal tumors
- Multifocal papillary histology
- HLRSS-associated RCC, RCC with Fumarate hydrtas (FH) deficiency
- BHDS related histology (multiple chromophobe, oncocytoma, oncocytic hybrid)
- Angiomyolipomas of the kidney and one TSC criterion in same person
- Succinate dehydrogenase (SDH) deficient RCC histologytment eligible
When and what should be tested?
- Upon or before presentation
- Peripheral blood, saliva or buccal mucosa swab
What tests should be considered?
- NCCN/FDA Approved
- BAP1
- FLCN
- FH
- MET
- SDHA/B/C/D
- TSC1, TSC2
- VHL
*Ensure inclusion into the molecularly targeted set of testing recommendations