Neuro Path - Eye and tumors Flashcards
New-onset adult seizure?
think brain tumor
- glioblastoma is mc in adults
How do you astrocytomas progress with age?
Worsen as you get older grade wise
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
Pilocytic astrocytoma
mc primary brain neoplasm
contrast ring-enhancing, hypodense central necrosis
glioblastoma, IV/IV
types of glioblastoma
primary - later in life, no precursor lesions
EGFR and PTEN
secondary - have low grade lesion - TP53
IDH1 (R132H mut has better prognosis)
IDH2 (younger)
histo hallmarks of glioblastoma
necrosis - serpentine pattern
pseudo-palisading cells around necrosis
vascular/endothelial proliferation - VEGF thickens malig astrocytes
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
“fried eggs and chicken wire appearance”
oligodendroglioma
first 2 decades of life 4th ventricle - discrete, exophytic, enhancing, mc site, spinal cord for adults suprattentorial grade III true rosettes are diagnostic
ependymoma
ependymal rosettes are true with an opening
mc in children in lateral ventricles
hydrocephalus and increased CSF
choroid plexus papilloma
ya
obstruction of foramen monro bc of its attachment to roof of 3rd ventricle -> noncommunication hydrocephalus
fatal and positional
colloid cyst of 3rd ventricle
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
medulloblastoma
medulloblastoma molecular grps
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
<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
atypical teratoid, rhabdoid tumor
immunosuppressed, increased in AIDS
multifocal, periventricular
Bcell, CD20, aggressive, assoc w EBV
accum around vessels - Hooping (cells sep by reticulin)
primary cns lymphoma
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
germ cell tumors
pineal
germinoma - responds to XRT and chemo, track w AFP and b-hCG
mc pineal tumor
germinoma
mc pineal tumor in adults
LG, pinecytoma
mc in kids
pineblastoma, high grade, necrosis, mitosis, RB gene
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)
meningioma, who I
molecular types of meningioma
NF2 on 22Q, higher grade and multiple/sporadic
TRAF7, mc next, lower histo grade
dura based- meningothelial cell origin of arachnoid EMA+
meningiomas are mc in
women, spinal, express PR, after preg and during
mc metastatic brain tumors
meninges mc site of metc, gray and white jxn, spc compression
lung, breast, melanoma, kidney, gi
choriocarcinoma mc spreads to
mc hemorrhage of metastatic brain tumors
melanoma, choriocarcinoma, RCC, lung
carcinomatosis mc in
lung and breast, tumor spread along surface of brain and s
PCA-1 antibody
destroys purkinje cells, gliosis and mild chronic inflammatory infiltrate
women w ovarian, uterine and breast carcinoma
subaute cerebullar degeneration
limbic encephalitis types
anna-1 antibody, scc of lung
NMDA receptor - overain teratomas
VGKC complex antibody, peripheral neuropathy,
AD, mc of types, 17q, neg regulates RAS signaling
pheochromocytoma
Neurofibromatosis, cafe au lait spots, optic n glioma, lisch nodules-pigment nod of iris
NF1
neurofibromatosis type1
RCC, hemangioblastoma, panc endocrine neoplasm
pheochromocytoma, paraganglioma
AD, downreg HIF-1, which reg VEGF causes polycythemia and increases erythropoietin
von hippel lindau
VHL
AD
ch 1pq23
lipid phosphatase, benign follicular appendage tumors (trichilemmomas), internal adenoca (breast, endometrial)
PTEN -> IK3/AKT signal pathway
cowden syndrome
AD 22q merlin integrates cytoskeletal sig and neurofibromas and acoustic neuromas bilateral schwannomas - cn VIII increased meningiomas and ependymomas
NF2
neurofibromatosis II
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
tuberous sclerosis
medulloblastomas, mut in TP53
li-fraumeni syndrome
mut in apc, medullo or glioblastoma, mismatch repair genes
turcot syndrome
mut in PTCH gene, meduloblastoma, upreg of SHH
gorlin syndrome
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
schwannoma
bag of worms, sporadic
nf-1 related
superficial cutaneous, diffuse, and plexiform
neurofibroma
HG tumors, arrise in NF1 pt
mpnst
risk factor - DM
lenticular opacities - acquired or congenital
can result from opacification of the lens nucleus (nuclear sclerosis)
cataracts
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
glaucoma
corneal transplant lacks what two things
blood vessels and lymphatics
this contributes to its opacity and lack of rejection
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
retinoblastoma
