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Von hippel –lindau disease

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description about multi-organic disease

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Von hippel –lindau disease

  1. 1. Von Hippel –Lindau disease David Sutton Ch 30 / page 937 Presented by Dr Laith Fadhel Al Hialy MBchB . PGCR
  2. 2. Von Hippel-Lindau syndrome (VHL) is an autosomal dominant , neurocutaneous disorder , have high incidence of multiple cysts in variety of organs , and tumor association • Kidneys 75% •Liver and spleen 50 % •Pancreas cysts ,insufficiency and DM •RCC 25-40 % multifocal 75 % •Cerebellar Hemangioblastoma •Retinal angioma •Pancreatic adenocarcenoma & adenoma •Epidyimal cyst adenoma •Pheochromcytoma
  3. 3. Cerebellar Hemangioblastoma
  4. 4. Retinal angioma
  5. 5. Pancreatic adenocarcenoma & cysts
  6. 6. Pheochromcytoma
  7. 7. RCC 25-40 % multifocal 75 % On MRI / intermediate signal on T1 , weighted , intermediate or high on T2 , w , high signal on STIR 10-15 % cystic enhance with contrast , destroyed renal structure , DDX infiltrative TCC
  8. 8. RCC By CT On CT / iso – hypo dense to renal tissue , occasionally hyper dense variable enhancement / 1/3 show calcification
  9. 9. RCC By IVU On IVU / soft tissue mass , 10 % show calcification ( dense – central – amorphous ) displaces calyces , distorts renal outline , loss renal function { tumor less than 3 cm not seen by IVU }

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