brain tumors Flashcards
intra-axial tumor
w/in brain parenchyma
extra-axial tumor
not in brain parenchyma
NF1
aka von recklinghausen NF1 on 17q plexiform neurofibroma MPNST optic and other gliomas phenochromocytoma GIST cafe au lait spots
NF2
NF2 on 22q
vesticular and PN schwannoma
menigiomas
TS
TSC1 on 19q TSC2 on 16p SEGA renal AMLs lung LAM cardiac rhabdomyomas
VHL
VHL on 3p
hemangioblastoma
renal cell carcinoma
pheochromocytomas
Li-fraumeni
TP53 on 17p
astrocytomas
breast carcinoma
bone and soft tissue sarcomas
turcot syndrome
FAP on 5p medulloblastoma GBM GI polyps colorectal CA
gorlin syndrome
PTCH on9q
desmoplastic medulloblastoma
nevoid BCC
odontogenic keratocysts
what are the most common CNS tumors
meningiomas 35%
glioblastomas 17%
pituitary 13%
astrocytomas 7%
meningiomas
arise for arachnoid but anchor to dura F>M loss of chr 22 -> loss of merlin multiple in NF2 and post radiation good prognosis locally invades into bone, but most are benign can be fatal dependent upon location
histo characteristics of meningiomas
calcifications
whorls
psammoma bodies
gliomas
most common grp of priamry brain tumors include: astrocytomas oligodendrogliomas ependymomas thought to derive from stem cells in periventricular area
pilocytic astrocytoma
grade I astrocytoma most frequent glial tumor of kids almost alwasys in cerebellum well circumscribed cystic w/solid tumor inside cyst increased ICP TP53 mutations very rare good prognosis
diffuse astrocytoma
ill defined, infiltrating any site in CNS seizures, HA, focal signs progress no matter what 5-7yr survival
astrocytoma grade IV
usually called glioblastoma
grade II astrocytoma mutations
TP53
grade III astrocytomas
mutations
aka anaplastic astrocytoma
TP53
secondary IV glioblastomas mutations
IDH1 and IDH2 point mutations LOH EGFR p16 deletion TP53 PTEN
primary glioblastoma
do not go thru other phases, originate as grade IV LOH EGFR (more then progressive p16 deletion TP53 (fewer then progressive) PTEN
oligodendroglioma
ill defined, infiltrating supratentorial, mostly white matter frontal most common 4th-5th decades long history of HAs, seizures frequently calcified prognosis is 6yrs fried egg appearance on histo
Oligodendroglioma mutations
IDH1/2 mutaiton in 90% and portend better prognosis
deletions of chrom 1p with 19p can be used for Tx
medulloblastoma
grade IV malignant tumor of kids most in vermis ataxia and signs of ICP tendency to disseminate via subarachnoid space and CSF can live to 5 yrs old
medulloblastoma CSF
increased pressure
high protein
low glucose
cerebral lymphoma
rare as primary tumor
typically a diffuse large B cell lymphoma
almost all are immunocompromised pts who are EBV +
meningneal spread common
corticosteroid Tx so successful tumor usually disappears
ependymoma
slow growing tumors
hydrocephalus, increased ICP, ataxia
well circumscribed, but can disseminate
schwannoma
much much more common in PNS does occur in vestibular system 4-6th decades solitary b/l in NF2 benign
