Imaging recommendation · Neuro · Head & Neck

Von Hippel-Lindau (VHL) disease surveillance

Q: What's the alternative imaging for von hippel-lindau (vhl) disease surveillance when pheochromocytoma screening?

MRI — Plasma metanephrines + abdominal MRI (iv contrast).

Q: What's the alternative imaging for von hippel-lindau (vhl) disease surveillance when endolymphatic sac tumor work-up?

MRI — MRI temporal bones with thin sections (iv contrast).

Recommended: MRI Brain + Entire Spine + Abdomen surveillance per VHL Alliance protocol is the preferred imaging study for von hippel-lindau (vhl) disease surveillance. Multi-organ tumor predisposition: CNS hemangioblastomas, retinal hemangiomas, RCC, pheochromocytoma, pancreatic NETs.

Recommended study

MRI Brain + Entire Spine + Abdomen surveillance per VHL Alliance protocol

MRI IV contrast Preferred Neuro · Head & Neck OutpatientScreening Reviewed 2026-05

Multi-organ tumor predisposition: CNS hemangioblastomas, retinal hemangiomas, RCC, pheochromocytoma, pancreatic NETs.

If the default doesn't apply

Pheochromocytoma screening

MRI Plasma metanephrines + abdominal MRI IV contrast

Endolymphatic sac tumor work-up

MRI MRI temporal bones with thin sections IV contrast

Watch-outs

Cerebellar hemangioblastoma + polycythemia

Cyst with enhancing mural nodule; can secrete EPO causing polycythemia.

Multifocal RCC

Nephron-sparing surgery is preferred for RCC < 3 cm; surveillance imaging guides timing.

Pearls

Three main organs: CNS (hemangioblastomas), kidney (RCC + cysts), pancreas (cysts + NETs).
Pheochromocytoma in up to 20% — screen with metanephrines.
Retinal screening is critical — refer to ophthalmology.