cerebellar-tumors

Question 1

Von Hipple linday Disease.Q: What’s inhirited cause?

Answer 1

Autosomal dominant, mutation of VHL tumor Suppressor gene 3p chromosome (O2 Sensation of The cell).

Question 2

Q: Hemangioblastoma Form

Answer 2

Sporadic 70%30% associated with VHL 30.grad I tumor

Question 3

Describe Hemangioblastoma gross

Answer 3

60%- well deferentiated, Highly vascularized, partially cysted40% compeletly Solidclassic gross : Cyst+ Solid Vascular nodule.

Question 4

Microscopic appearance

Answer 4

Neoplastic cells Larg and vaculated.Many Vascular cell.Thin numerous blood vessels.Hemorrahge.

Question 5

Medullo blastoma grade IVQ: Genetic cause

Answer 5

1- Loss short arm of 17p . →dublication of Long arm →isochromose 17pmost Favorable genetic subtype is WNT-activated and recognized by B-catenin

Question 6

Gross description

Answer 6

In vermis:pink, Friable mass Fill 4th ventricleIn Hemisphere : Firm, more deferentiated Small foci of necrosis

Question 7

Describe Microscopic appearance

Answer 7

1- Classic 2- Medullo blastoma with extensive nodularity3- Desmoplastic nodular medulloblastoma4- Anaplastic Medulloblastoma / Large cells

Question 8

Describe classic medulloblastoma

Answer 8

Most: Dense paked small round cellMild: Moderate nuclear pleomorphism High mitotic count.Some: Homer wright rosette Neutrophile surrounding by primitive tumor cells