Atlas One · Host Biology · Scale 07 — System

VHL Disease

Hereditary VHL disease is caused by germline VHL mutations; clear cell RCC, CNS and retinal hemangioblastomas, pheochromocytoma (type 2), and pancreatic NETs; belzutifan (HIF-2α inhibitor) is FDA-approved for VHL-related tumors; type 1/2A/2B/2C classification by pheo risk.

Also known as: VHL disease, von Hippel-Lindau disease, VHL syndrome, VHL hemangioblastoma, VHL RCC, hereditary VHL, VHL pheochromocytoma, VHL belzutifan, von Hippel-Lindau syndrome

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
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vhl-disease

VHL Disease

Hereditary VHL disease is caused by germline VHL mutations; clear cell RCC, CNS and retinal hemangioblastomas, pheochromocytoma (type 2), and pancreatic NETs; belzutifan (HIF-2α inhibitor) is FDA-approved for VHL-related tumors; type 1/2A/2B/2C classification by pheo risk.

Entry Metadata

FieldValue
IDvhl-disease
NameVHL Disease
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toVhl connects-toEgln1 connects-toRenal Cell Carcinoma connects-toPheochromocytoma Paraganglioma connects-toHif 1Alpha connects-toBrain connects-toEye connects-toTuberous Sclerosis Complex connects-toPancreas connects-toErythropoietin

Sources