How does VHL mutation type predict lesion risk?

Type 1 (truncating/missense): hemangioblastomas + RCC, low pheochromocytoma risk. Type 2A (missense): hemangioblastomas + pheochromocytoma, low RCC risk. Type 2B: all manifestations including high RCC risk. Type 2C: pheochromocytoma only. VHL type determines surveillance intensity for different organs.

What is the belzutifan indication in VHL disease?

Belzutifan (HIF-2α inhibitor) is FDA-approved for VHL-associated RCC, CNS hemangioblastomas, and pancreatic NETs not requiring immediate surgery. In the LITESPARK-004 trial, ORR was 49% for RCC, 30% for CNS lesions with durable responses, reducing need for surgical procedures.

Von Hippel-Lindau Lesion Risk Calculator

Estimate risk of VHL-associated lesions (hemangioblastomas, ccRCC, pheochromocytoma, pancreatic NETs) based on mutation type classification (VHL type 1, 2A, 2B, 2C) and age.

Use the full interactive calculator below, or

Von Hippel-Lindau Lesion Risk Calculator

Frequently Asked Questions

How does VHL mutation type predict lesion risk?

What is the belzutifan indication in VHL disease?