Step I Sturge Weber Tuberous sclerosis and VHL

Started by jtre506
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Sturge-Weber: AV malformations of the face (trigeminal nerve distribution) and brain.
Tuberous Sclerosis: hypopigmented skin lesions ("ash leaf spots") and brain hamartomas.
VHL: Bilateral Renal Cell Carcinoma and Pheos
 
Sturge-Weber: AV malformations of the face (trigeminal nerve distribution) and brain.
Tuberous Sclerosis: hypopigmented skin lesions ("ash leaf spots") and brain hamartomas.
VHL: Bilateral Renal Cell Carcinoma and Pheos

Dont forget rhabdomyomas/heart tumers with tuberous sclerosis - this is a classic presentation - and I believe this is seen at a young age.
 
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Tuberous sclerosis

Michael Schumacher Likes CARs= MSLCAR

Mental ******ation
Seizures (epileptic)
Light skin (hypopigmentation)
Candle stick tumor in ventricles (astrocyte proliferation in epedyma)
Angiomyolipoma of the kidney
Rhabdomyosarcoma of the heart..

If you like cars like I do, this should be helpful
Hope that helps
 
Tuberous sclerosis

Michael Schumacher Likes CARs= MSLCAR

Mental ******ation
Seizures (epileptic)

Light skin (hypopigmentation)
Candle stick tumor in ventricles (astrocyte proliferation in epedyma)
Angiomyolipoma of the kidney
Rhabdomyosarcoma of the heart..

If you like cars like I do, this should be helpful
Hope that helps

Guys, please remember the other manifestation of TS -- angiofibromas esp of the nose and cheeks. (you may see this on an NBME exam..😉

In fact, this may be one of the first signs of the dz and be the reason why the parents bring the "poor little dude" (Dr.G) to see the doc/derm!
 
Tuberous sclerosis

Michael Schumacher Likes CARs= MSLCAR

Mental ******ation
Seizures (epileptic)
Light skin (hypopigmentation)
Candle stick tumor in ventricles (astrocyte proliferation in epedyma)
Angiomyolipoma of the kidney
Rhabdomyosarcoma of the heart..

If you like cars like I do, this should be helpful
Hope that helps

ok, I know all these are all important, but really remember the case of a young patient with cancer in the heart muscle. Its such a unique presentation, and popular (nod, nod, wink, wink).