Once a tumor has been identified, the general recommendation is for immediate surgical removal due to the aggressive nature of SDH-RCC lesions, and the ‘3 cm rule’ followed in VHL and HPRC is not recommended in SDH-RCC due to the metastatic potential of even smaller lesions well below 3cm, with metastatic risk approaching 30% [102,113,126]. Here, SDHB is linked to renal cell carcinoma.