Neuro Path - Eye and tumors

Question 1

New-onset adult seizure?

Answer 1

think brain tumor

- glioblastoma is mc in adults

Question 2

How do you astrocytomas progress with age?

Answer 2

Worsen as you get older grade wise

Question 3

Grade I/IV
Most commonly in post fossa enter or cerebral hemispheres or cerebellum
First two decades of life
associated with an NF1 functional loss of neurofibromin
cystic with a mural nodule
low cellularity

Contain a biphasic pattern with a loose glial with cystic changes and dense pilots tissue

Hair like cells with a long bipolar processes, Rosenthal fibers, eosinophilic granular bodies

Answer 3

Pilocytic astrocytoma

Question 4

mc primary brain neoplasm

contrast ring-enhancing, hypodense central necrosis

Answer 4

glioblastoma, IV/IV

Question 5

types of glioblastoma

Answer 5

primary - later in life, no precursor lesions
EGFR and PTEN

secondary - have low grade lesion - TP53
IDH1 (R132H mut has better prognosis)
IDH2 (younger)

Question 6

histo hallmarks of glioblastoma

Answer 6

necrosis - serpentine pattern
pseudo-palisading cells around necrosis
vascular/endothelial proliferation - VEGF thickens malig astrocytes

Question 7

adults, II/IV
IDH1 and IDH2 - favorable prognosis, 1p19Q, favorable, anaplastic (III/IV)
cerebral hemispheres
calcification restricted to cortex, curvy or gyriform distribution
perinuc halows and anastomosing capillaries

Answer 7

“fried eggs and chicken wire appearance”

oligodendroglioma

Question 8

first 2 decades of life
4th ventricle - discrete, exophytic, enhancing, mc site, spinal cord for adults
suprattentorial
grade III
true rosettes are diagnostic

Answer 8

ependymoma

ependymal rosettes are true with an opening

Question 9

mc in children in lateral ventricles

hydrocephalus and increased CSF

Answer 9

choroid plexus papilloma

Question 10

ya
obstruction of foramen monro bc of its attachment to roof of 3rd ventricle -> noncommunication hydrocephalus
fatal and positional

Answer 10

colloid cyst of 3rd ventricle

Question 11

IV/IV children, malignant embryonal tumor
cerebellum, midline and occludes csf flow
sheets of anaplastic cells, abundant mitoses, homer-wright rosettes
drop mets (cauda equine)
radiosensitive
supratentorial PNET

Answer 11

medulloblastoma

Question 12

medulloblastoma molecular grps

Answer 12

WNT - older kid, chr 6, B catenin, good prog
SHH - infants-ya, MYCN, intermediate prog
group 3 - MYC and I17Q, infant and childern, WORST prognosis
gropu 4 - I17Q, maybel MYCN, poor prog

Question 13

<2yo, large cell w paranuc filamentous inclusions, prob w large cell medulloblastoma
posterior fossa or supratentorial 
EMA and VImentin
Rhabdoid cells - eosinophilic cytoplasm
ch22, HSNF5/INI1
aggressive, <1yr survival

Answer 13

atypical teratoid, rhabdoid tumor

Question 14

immunosuppressed, increased in AIDS

multifocal, periventricular
Bcell, CD20, aggressive, assoc w EBV
accum around vessels - Hooping (cells sep by reticulin)

Answer 14

primary cns lymphoma

Question 15

primary brain germ cell tumors that are midline
see in Japanese, in first two decards
mets of gonadal germ cell to cns is common
mc males? supraselluar

Answer 15

germ cell tumors
pineal

germinoma - responds to XRT and chemo, track w AFP and b-hCG

Question 16

mc pineal tumor

Answer 16

germinoma

Question 17

mc pineal tumor in adults

Answer 17

LG, pinecytoma

Question 18

mc in kids

Answer 18

pineblastoma, high grade, necrosis, mitosis, RB gene

Question 19

after 3rd decade
slow, compresses brain but doesn’t hardly infiltrate, penetrates bone
radiation induced
calcified psammoma bodies,=- en plaque (sheet like spread along dura)
wholred clusters of monotonous cells
secretory - PAS+ droplets, TRAF7, KLF4
atypical II/IV - more aggressive, clear and chordoid
anaplastic II/IV is malig (pap and rhaboid subtypes)

Answer 19

meningioma, who I

Question 20

molecular types of meningioma

Answer 20

NF2 on 22Q, higher grade and multiple/sporadic
TRAF7, mc next, lower histo grade

dura based- meningothelial cell origin of arachnoid EMA+

Question 21

meningiomas are mc in

Answer 21

women, spinal, express PR, after preg and during

Question 22

mc metastatic brain tumors

meninges mc site of metc, gray and white jxn, spc compression

Answer 22

lung, breast, melanoma, kidney, gi

choriocarcinoma mc spreads to

Question 23

mc hemorrhage of metastatic brain tumors

Answer 23

melanoma, choriocarcinoma, RCC, lung

Question 24

carcinomatosis mc in

Answer 24

lung and breast, tumor spread along surface of brain and s

Question 25

PCA-1 antibody destroys purkinje cells, gliosis and mild chronic inflammatory infiltrate women w ovarian, uterine and breast carcinoma

Answer 25

subaute cerebullar degeneration

Question 26

limbic encephalitis types

Answer 26

anna-1 antibody, scc of lung NMDA receptor - overain teratomas VGKC complex antibody, peripheral neuropathy,

Question 27

AD, mc of types, 17q, neg regulates RAS signaling pheochromocytoma Neurofibromatosis, cafe au lait spots, optic n glioma, lisch nodules-pigment nod of iris

Answer 27

NF1 | neurofibromatosis type1

Question 28

RCC, hemangioblastoma, panc endocrine neoplasm pheochromocytoma, paraganglioma AD, downreg HIF-1, which reg VEGF causes polycythemia and increases erythropoietin

Answer 28

von hippel lindau | VHL

Question 29

AD ch 1pq23 lipid phosphatase, benign follicular appendage tumors (trichilemmomas), internal adenoca (breast, endometrial)

Answer 29

PTEN -> IK3/AKT signal pathway | cowden syndrome

Question 30

``` AD 22q merlin integrates cytoskeletal sig and neurofibromas and acoustic neuromas bilateral schwannomas - cn VIII increased meningiomas and ependymomas ```

Answer 30

NF2 | neurofibromatosis II

Question 31

AD 9q, 16p TSC1 hamartin, TSC2 tuberin work together in a complex that neg regulates mTOR/angiofibromas/MR hamartomas, cortical tubers, subependymal nodules, SEGA, renal angiomyolipomas, cardiac rhabomyomas, cut lesion slike shagreen and ash leak patches

Answer 31

tuberous sclerosis

Question 32

medulloblastomas, mut in TP53

Answer 32

li-fraumeni syndrome

Question 33

mut in apc, medullo or glioblastoma, mismatch repair genes

Answer 33

turcot syndrome

Question 34

mut in PTCH gene, meduloblastoma, upreg of SHH

Answer 34

gorlin syndrome

Question 35

s100 cerebellar pontine angle acoustic neuroma - cn viiii, tinnitus and hearing loss NF2, loss of merlin, antoni A (verocay bodies palisading nuclei around nuc free zoens), antoni B

Answer 35

schwannoma

Question 36

bag of worms, sporadic nf-1 related superficial cutaneous, diffuse, and plexiform

Answer 36

neurofibroma

Question 37

HG tumors, arrise in NF1 pt

Answer 37

mpnst

Question 38

risk factor - DM lenticular opacities - acquired or congenital can result from opacification of the lens nucleus (nuclear sclerosis)

Answer 38

cataracts

Question 39

assoc w increased intraocular pressure from resitance to aqueous outlfow or peripheral zone of iris adheres to trabecular meshwork and physically impedes outflow RF - aa/hispanic, DM

Answer 39

glaucoma

Question 40

corneal transplant lacks what two things

Answer 40

blood vessels and lymphatics this contributes to its opacity and lack of rejection

Question 41

mc intraocular malignancy of childhood ch 13 long arm, RB leukocoria (white pupillary reflex), pseudohypopyon, can cause metz to distant places via optic nerve (poor prog) histo - round shape hyperchromatic nuclei w scant cytoplasm, flexner wintersteiner (eosinophilic columnar cells w peripherally oriented nuclei around a lumen), homer wright, fleurette

Answer 41

retinoblastoma