CNS patho Tumors Flashcards
Sturge-Weber syndrome is also called
encephalotrigeminal angiomatosis.
portwine stain
(nevus flammeus or nonneoplastic birthmark) in CN V1/V2 distribution
Tuberous sclerosis pathology:
TSC1 (Ch 9) encodes Hemartin & TSC2 (Ch 16) encodes Tuberin → both inhibit mTOR
mutation → ↑mTOR activity →cell growth
Lisch nodules
(pigmented iris hamartomas )
NF-1 pathology
Mutation in NF1 tumor suppressor gene on chromosome 17 (encodes neurofibromin, a negative RAS regulator).
NF-1 also called
von Recklinghausen disease.
NF-1: these benign nerve sheath neoplasms predominantly comprised of ______ → embryologically derived from ______
Schwann cells
Neural crest cells
NF-2 → brain tumors involved
meningiomas, ependymomas.
VHL pathology
Deletion of VHL gene on chromosome 3p.
pVHL ubiquitinates-hypoxia inducible factor 1a.
Fried egg appearance
Oligodendroglioma Multiple myeloma Oligodendrocytes Dysgerminoma Seminoma
Hemangioblastoma: origin and histology
Blood vessel origin.
Closely arranged, thin walled capillaries with minimal intervening parenchyma
Schwannoma histology:
Biphasic
Antoni A pattern (dense, hypercellular areas containing spindle cells) alternating with Antoni B (hypocellular, myxoid areas.)
Collier sign
Bilateral eyelid retraction → sclera visible above the superior corneal limbus (Sunset sign)
Childhood primary tumors according to location Cerebellar hemesphere: Cerebellar vermis: 4th Ventricle: Supratentorial:
Cerebellar hemesphere: Pilocytic astrocytoma
Cerebellar vermis: Medulloblastoma
4th Ventricle: Ependymoma
Supratentorial: Craniopharyngioma
Lumber puncture is contraindicated in space occupying lesion in brain due to _____
risk of herniation
