Von Hippel-Lindau syndrome Flashcards

1
Q

inheritance

A

autosomal dominant

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2
Q

on what chromosome is the VHL gene located?

A

Chromosome 3

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3
Q

What does VHL predispose patients to?

A

Neoplasia

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4
Q

What cancers commonly develop in VHL?

A
  • cerebellar haemangiomas
  • retinal haemangiomas
  • renal cysts (premalignant)
  • phaeochromocytoma
  • extra-renal cysts: epididymal, pancreatic, hepatic
  • endolymphatic sac tumours
  • clear-cell renal cell carcinoma
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5
Q
A
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