31a - Adult Brain Tumors

Question 1

Which genetic syndrome is associated with glioma and medulloblastoma?

Answer 1

Li Fraumeni (Ch.17 , p53 mutation)

Question 2

Which genetic syndrome is associated with subependymal giant cell astrocytoma?

Answer 2

Tuberous Sclerosis (Ch. 9/16, TSC 1/2)

Question 3

Which genetic syndrome is associated with cortical tubers and glioma?

Answer 3

Tuberous sclerosis (Ch.9/16, TSC 1/2)

Question 4

Which genetic syndrome is associated with glioma of the optic nerve, astrocytoma and glioblastoma?

Answer 4

Neurofibromatosis 1 (Ch. 17, NF1)

Question 5

Which genetic syndrome is associated with pituitary cancer?

Answer 5

MEN Type 1 (ch. 11, menin)

Question 6

Which genetic syndrome is associated with meningioma, schwannoma, and ependymoma?

Answer 6

Neurofibromatosis 2 (ch. 22, NF2)

Question 7

Which genetic syndrome is associated with hemangioblastoma?

Answer 7

Von Hippel Lindau (Ch. 3, VHL)

Question 8

Which genetic syndrome is associated with retnioblastoma?

Answer 8

retinoblastoma mutations (ch. 13, RB1)

Question 9

Which genetic syndrome is associated with bilateral acoustic neuroma?

Answer 9

NF2 (ch.22)

Question 10

Which neoplasms is Li Fraumeni associated with?

Answer 10

glioma and medulloblastoma

Question 11

Which neoplasms is Tuberous sclerosis associated with?

Answer 11

subependymal gian cell astrocytoma, corticol tubers, glioma

Question 12

Which neoplasms is a mutation in Ch. 17, p53 gene associated with?

Answer 12

Li fraumeni = glioma and medulloblastoma

Question 13

Which neoplasms is NF1 associated with?

Answer 13

CH 17 mutation. glioma of optic nerve, astrocytoma, glioblastoma

Question 14

Which neoplasms is NF2 associated with?

Answer 14

meningioma, scwhannomma (bilateral acoustic neuroma), ependymoma

Question 15

Which neoplasms is MEN type 1 associated with?

Answer 15

ptiutitary

Question 16

Which neoplasms is von hippel lindau associated with?

Answer 16

hemangioblastoma (ch. 3, of VHL)

Question 17

Which neoplasms is a mutation in ch. 22 asssociated with?

Answer 17

NF2, meningioma, schwannoma, ependymoma

Question 18

Which neoplasms is a mutation in 9/16 associated with?

Answer 18

TSC1/2 - subependymal giant cell astrocytoma, corticol tubers, glioma

Question 19

Which neoplasms is a mutation in ch 11 associated wtih?

Answer 19

MEN1 - menin - pituitary tumors

Question 20

What is most common type of primary brain tumor?

Answer 20

infiltrative astrocytoma and/or Glioblastoma multiforme

Question 21

Which type of MRI makes water appear white?

Answer 21

T2 MRI. CSF, Edema and tumors (water) appear bright. Contrast is dark

Question 22

Which type of MRI makes contrast appear dark?

Answer 22

T2 MRI. Contrast dark, water bright

Question 23

Which type of MRI will show tumors as hypointense?

Answer 23

T1 MRI will show hypointesnse water (CSF, tumor, edema) and bright contrast.

Question 24

What is the equation for cerebral perfusion pressure?

Answer 24

CPP = MAP - ICP

Question 25

What headache symptoms should clue you in to brain tumors?

Answer 25

worse on waking up but better within an hour. Vomiting immediately after headaches. New onset or change in character of headaches, or change in mental status

Question 26

What is gold standard for diagnosing brain tumors?

Answer 26

MRI = gold standard CT is better for calcification/pathology Tissue biopsy is definitive diagnosis EXCEPT for CNS lymphoma because you can sample malignant cells on LP

Question 27

Concerning brain herniations, What is contralateral hemianopsia indicative of?

Answer 27

Ips. PCA compression

Question 28

Concerning brain herniations, what is ipsilateral dilated pupil and ptosis indicative of?

Answer 28

CN III compression

Question 29

Concerning brain herniations, what is ipsilateral paresis indicative of?

Answer 29

Contralateral Crus cerebri compression (Kernohan's notch)

Question 30

What is Duret hemorrhage?

Answer 30

caudal displacement of brainstem, commonly causes paramedian artery rupture

Question 31

What is at risk for compression in a cingulate (subfalcine) herniation?

Answer 31

cingulate passes under falx cerebri. Anterior cerebral artery at risk for compression.

Question 32

What are symptoms of a diencephalic downward (transtentorial) herniation?

Answer 32

brainstem will be compressed so drowsiness, imparied vertical gaze, bilateral small pupils (horner), coma-->death.

Question 33

What should CNIII palsy and both contra/ipsilateral hemiparesis make you think in terms of herniations?

Answer 33

classical uncal herniation. May have contralateral or ipsilateral hemiparesis depending on particular case, but always CN III palsy.

Question 34

What are symptoms of upward herniations through the tentorium?

Answer 34

ipsilateral occulmotor nerve palsey mixed with horner's syndrome (so midposition unreactive pupil), contralateral hemiparesis

Question 35

What type of herniation could cause BP and respiratory changes, weakness and horner's syndrome?

Answer 35

cerebellar tonsilar herniation

Question 36

What types of neoplasms are considered gliomas?

Answer 36

astrocytomas, oligodendroglioma, ependymoma | GBM

Question 37

What are good prognostic factors for gliomas like astrocytomas or oligodendrogliomas?

Answer 37

``` MGMT methylation (good band=good prognosis), IDH mutation, Ch. 1/19 deletion in tumor. Obvious ones like low age, low grade/stage. ```

Question 38

Where are gliomas most commonly found?

Answer 38

cerebral hemispheres

Question 39

Butterfly lesion on MRI = ?

Answer 39

butterfly glioma. they like to cross the corpus callosum.

Question 40

GFAP+ astrocytes with pseudopalisading tumor cells around the area of necrosis on biopsy should make you think what?

Answer 40

astrocytoma

Question 41

What type of MRI is best for GBM?

Answer 41

T2 it will pop up as hyperintense (white). | Will be negative on T1 because it is slow growing, with few vessels and not a lot of leakiness

Question 42

Treatment for gliomas such as astrocytomas, or ependymomas?

Answer 42

chemo: temozolomide and bevacizumab. radiation steroids to restore BBB (fast and temporary) anticonvulsants (only after seizures occur)

Question 43

Which are secreting pituitary adenomas: macro or micro?

Answer 43

``` micro secrete (< 1cm). macro >1 cm tend to be nonsecreting so they get large and start causing compression symptoms that clue you in. ```

Question 44

What type of receptor does GH use?

Answer 44

JAK/STAT

Question 45

What are symptoms of pituitary microadenoma?

Answer 45

galactorrhea from prolactin, acromegally from GH.

Question 46

What are symptoms of pituitary macroadenoma?

Answer 46

bitemproal hemaniopsia. headache diplopia and facial numbing.

Question 47

What nerves travel in cavernous sinus?

Answer 47

5 nerves in the carvernous: 3,4,V1,V2, and 6

Question 48

If lower quadrants of vision are lost, from which side is the optic tract/chiasm being compressed?

Answer 48

vision loss on bottom = compression from top = Rahtke pouch craniopharyngioma

Question 49

What is drug treatment for GH secreting tumor?

Answer 49

somatostatin analogue octreotide

Question 50

What is drug treatment for prolactin secreting tumor?

Answer 50

Dopamine agonist bromocriptine. DA inhibits prolactin secretion

Question 51

EBV infection in an AIDs patient is a risk for what brain neoplasm?

Answer 51

CNS lymphoma. Most common CNS tumor in AIDS.

Question 52

Multiple lesions in leptomeninges and deeper periventricular brain parenchyma =

Answer 52

CNS lymphoma

Question 53

What is used for diagnosis of CNS lymphoma

Answer 53

B cells found in CSF

Question 54

Why should you not give steroids prior to doing an LP?

Answer 54

it will mess with results. In CNS lymphoma it will necrose B cells.

Question 55

What are meningiomas derived from?

Answer 55

arachnoid cap cells outside the brain (extra-axial)

Question 56

What are the different types of meningiomas?

Answer 56

low grade (most common), atypical, and malignant

Question 57

Where are meningiomas found?

Answer 57

convexity, parasagittal or optic sheath. | Well defined masses easily separated from neuropil

Question 58

What is microscopic pathology of meningioma?

Answer 58

psammoma bodies

Question 59

How will a meningioma appear on MRI?

Answer 59

dark on T1, bright on T2. | Calcifications from psammoma bodies or dural tail.

Question 60

Where are acoustic neuroma's derived from?

Answer 60

scwhann cells around vestibular branch of CN 8

Question 61

What does Antoni A and Antoni B mean?

Answer 61

for acoustic neuromas. on microscopic path, Antoni A = densely packed cells Antoni B = loose cystic areas

Question 62

What is clinical presentation of acoustic neuroma?

Answer 62

hearing loss, tinnitus, dizziness, ataxia

Question 63

What is most common source of brain mets?

Answer 63

lung cancer (50%), then breast cancer in females. Melanoma high very high probability of met to brain. also colon and renal

Question 64

Where are extradural spinal cord tumors from?

Answer 64

mets from breast, lung, prostate, | less importantly from renal, lymphoma, sarcoma

Question 65

What are the intradural extramedullary tumors?

Answer 65

schwannomas, neruofibriomas and meningiomas

Question 66

What are the intradural intramedullary tumors?

Answer 66

astrocytoma in PEDs, ependymoma in ADULTS, hemangioblastomas