Von Hippel-Lindau disease (VHL)

Question 1

Most common location hemangioblastomas

Answer 1

cerebellar
2nd location retina

Question 2

Subtype VHL

Answer 2

Type I > HGB
Type 2 A > pheochromocytoma
Type 2B > pheochromocytoma+ HGB + RCC
Type 2 C > pheochromocytoma

Question 3

MCC location HCB cerebellum e

Answer 3

cerebellar hemispheres ( posterior superior 1/2)

Question 4

MCC location HGB spine

Answer 4

Cervical and thoracic posterior 1/2
90% spinal HGB are VHL
90 % spinal HGB sryngiomylia

Question 5

Brain stem HGB

Answer 5

posterior medulla oblongata usually around the obex and the region within the area postrema

Question 6

endolymphatic sac tumors (ELST) MCC location VHL

Answer 6

Petrous portion of temporal bone 🦴
Bilateral hearing loss + tinnitus

Question 7

MCC malignant tumor in VHL

Answer 7

RCC
Surgery indicated if size < 3 cm

Question 8

Medical tx for VHL

Answer 8

belzutifan (Welireg), an hypoxia-inducible factor inhibitor
Effective HGB pancreatic neuroendocrine tumors than RCC.

Question 9

SRS in HGB indication

Answer 9

Asymptomatic
> 5 mm cystic or progress in size

Question 10

Radiosurgery CI🚫

Answer 10

HGB with cyst

Question 11

Radiation dose cranial and spine

Answer 11

Cranial 22 GY
Spinal 21 GY

Question 12

VHL present at age

Answer 12

60

Question 13

Gen VHL

Answer 13

germline variants of the VHL tumor suppressor gene 3p 25.3