description about multi-organic disease

1. Von Hippel –Lindau disease
David Sutton
Ch 30 / page 937
Presented by Dr Laith Fadhel Al Hialy
MBchB . PGCR

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. Cerebellar Hemangioblastoma

4. Retinal angioma

5. Pancreatic adenocarcenoma & cysts

6. Pheochromcytoma

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. RCC By CT
On CT / iso – hypo dense to renal tissue , occasionally hyper dense variable
enhancement / 1/3 show calcification

10. 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 }