In VHL-associated Renal Cell Carcinoma, surgical intervention (typically nephron-sparing surgery) is often delayed until the largest tumor reaches 3.0 cm. This balance minimizes the need for repeat surgeries and eventual dialysis while maintaining a low risk of metastasis.
Endolymphatic sac tumors (ELST) are common in VHL and can cause sudden hearing loss. Patients should receive an audiology exam if hearing changes occur.
The VHL Handbook.
VHL surveillance guidelines aim to detect tumors early when they are most treatable. Genetic testing for RET/VHL should be offered to all family members at risk.