Von Hippel Lindau Disease

  • Etiology: mutations in the VHL tumor suppressor gene on chromosome 3
  • Imaging:
    — Brain – hemangioblastoma of cerebellum
    — GI – cysts of liver + pancreas
    — GU – cysts of kidney, renal cell carcinoma, pheochromocytoma

Radiology Cases of Von Hippel Lindau Disease

Radiology Cases of Hemangioblastoma in Von Hippel Lindau Disease

MRI of hemangioblastoma in Von Hippel Lindau disease
Axial (above), sagittal (below left) and coronal (below right) T1 MRI with contrast of the brain shows a round, solid, avidly enhancing lesion in the superior aspect of the left cerebellar hemisphere.