Von Hippel-Lindau syndrome Flashcards
1
Q
inheritance
A
autosomal dominant
2
Q
on what chromosome is the VHL gene located?
A
Chromosome 3
3
Q
What does VHL predispose patients to?
A
Neoplasia
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
5
Q
A