Phakomatosis - VHL

Question 1

most common RCC associated with VHL

Answer 1

clear cell

Question 2

inheritance pattern of VHL

Answer 2

AD

Question 3

VHL chromosome

Answer 3

3

Question 4

most common site for VHL hemangioblastomas

Answer 4

cerebellum

cerebellum > retina > spinal cord > brain stem

Question 5

Answer 5

retinal hemangioblastoma - VHL

Question 6

male pt with VHL, ultrasound of the epididymis

Answer 6

papillary cystadenoma of the epididymis

occur in approximately 10%–60% of men with VHL

They are histologically similar to endolymphatic sac cysts and renal cysts

may lead to infertility due to obstructive azoospermia

Question 7

pt with VHL, US of the epididymis

Answer 7

epididymal cysts with ectatic dilated rete testes in a VHL pt (common)

Question 8

Answer 8

Question 9

Answer 9

Question 10

Answer 10

VHL

pheo and spinal hemangioma

Question 11

multiple pancreatic cysts

Answer 11

VHL

Question 12

multiple paragangliomas seen in:

Answer 12

VHL and MEN2

Question 13

endolymphatic sac tumor

Answer 13

VHL

Question 14

petrous apex permeative tumor with moth eaten appearance

Answer 14

endolymphatic sac tumor

Question 15

MRI screening recommendations for VHL

Answer 15

head and abdomen

every 2 years after 10 years old

Question 16

Answer 16

VHL

Question 17

Answer 17

retinal HB in VHL

Question 18

32 yoM

Answer 18

cerebellar HB

Question 19

Answer 19

VHL

multiple cerebellar and spinal hemangioblastoma

Question 20

Answer 20

endolymphatic sac tumor

VHL

Question 21

44yF with VHL

Answer 21

Endolymphatic sac tumor -

Question 22

Answer 22

VHL

Question 23

Answer 23