Cancer

Question 1

Where does medulloblastoma metastasize?

Answer 1

Leptomeningeal spaces - patchy CNS involvement typically causes multifocal, asymmetric neurologic signs and symptoms

Question 2

Bilateral vestibular schwannomas are associated with…

Answer 2

Neurofibromatosis type II

Question 3

Von Hippel Lindau triad of tumors

Answer 3

CNS and retinal hemangioblastoma
Pheochromocytoma
RCC (clear cell)

Question 4

ADPKD CNS involvement

Answer 4

Aneurysms

Question 5

What sellar pathology calcifies vs not?

Answer 5

Craniopharyngiomas

Pituitary adenomas and Rathke cleft cysts do not calcify

Question 6

Tuberous sclerosis complex

Answer 6

Autosomal dominant neurocutaneous disorder - benign hamartomas

Seizures
Subependymal nodules
Cortical glioneuronal hamartomas (tubers)

Cardiac rhabdomyoma

Ash leaf spots (hypopigmented macules)
Angiofibromas
Shagreen patches (thick orange peel skin)

Question 7

Astrocytoma prognosis is most affected by what?

Answer 7

Tumor grade (anaplasia, necrosis, neovascularity, etc.), not vascular invasion

Question 8

Pseudopalisading pleomorphic tumor cells bordering central areas of necrosis, hemorrhage, or microvascular proliferation

Answer 8

Astrocytoma

Question 9

“Fried egg” cells - round nuclei with clear cytoplasm

Answer 9

Oligodendroglioma - often in frontal lobes, commonly calcified

Question 10

Spindle cells concentrically in whorled pattern, as well as psammoma bodies (laminated calcifications)

Answer 10

Meningioma - also note dural tails (origin is arachnoid cell)

Question 11

Closely arranged, thin-walled capillaries and minimal intervening parenchyma

Answer 11

Hemangioblastoma - often cerebellar; associated with VHL syndrome when found with retinal involvement

Question 12

S-100+ tumor

Answer 12

Vestibular schwannoma (acoustic neuroma) - Schwann cell origin

Question 13

Tuberous sclerosis - treatment for infantile spasm

Answer 13

Vigabatrin - enhances GABA transmission
ACTH is alternative/adjunctive

Question 14

Tuberous sclerosis - treatment for seizures

Answer 14

Standard antiepileptics
mTOR inhibitors if above failed

Sirolimus may slow disease progression

Question 15

Sturge-Weber syndrome - genetics

Answer 15

Somatic mosaic mutation in GNAQ gene; therefore, not inherited

Question 16

Belzutifan

Answer 16

HIF-2alpha inhibitor used in unresectable VHL tumors

Question 17

Why does ataxia-telangiectasis predispose to infection?

Answer 17

IgA deficiency

Question 18

Which of the neurocutaneous disorders is autosomal recessive inheritance?

Answer 18

Ataxia-telangiectasia; the others (besides Sturge-Weber) are autosomal dominant