CNS Tumors
Gliomas
tumor
%
age
location & clinical pres.
histology
prognosis
tx
Astrocytomas: morphology & grade determine prognosis
diffuse (fibrillary) 20%
any usu. cerebral white matter unclear margins
grade dependent!
surgical resection
astrocytoma
of
seizures
infiltrates
good: low grade
(+ radiation?)
glioma
headaches
GFAP+ cell processes
poor: anaplastic type
(rarely complete
focal signs
progress in grade up to
(usu. in elderly)
resection)
GBM
brainstem glioma
10CN palsies
pons
poor
radiation
(subtype of
20
long tract signs
progresses thru grades up
median survival <2y
no surgery
diffuse)
gait abnormalities
to GBM (50%)
cerebellar signs
GBM
65%
adult usu. cerebral white matter supratentorial
very poor, always
surgery,
glioblastoma
peak seizures
densely hypercellular
recurs
surgery +
multiforme
50
headaches
high anaplasia: often
untreated: 3mo
radiation/chemo
(3 etiologic
focal signs
astrocytes, oliogos,
surgery: 6 mo
or none
types) 5
ependymoma tissue3
surgery + rad: 10-12 m
necrosis, hemorrhage
pseudopalisading cells4
pilocytic
kids dep. on location
circumscribed, low grade
cystic cerebellar 90%
surgical w/o
astrocytoma
cerebellar1
mixed loose and dense
5ys even w/
normal tissue
hypothalamus
microcystic areas
incomplete resection
margin
optic/nerve chiasm (esp.
Rosenthal fibers
in NF-1 pts2)
subependymal
rare
vascular, intrventricular
mixed astrocytic, neural
not progressive
?
giant cell
mass
bizarre morphology
may cause focal sx
usu. part of systemic
often hemorrhage
tuberous sclerosis
1. older children – the most common childhood astrocytoma, 60% cystic, good prognosis
2. NF-1 = von Recklinghausen’s Disease
3. must take deep, multiple biopsies for accurate dx 4. malignant cells tightly clustered around necrotic center
5. 30% progressed from diffuse (p53, Rb, others; younger pts), 30% spontaneous (EGFR amp, older pts), 30% other
CNS Tumors
Gliomas, cont.
tumor
%
oligodendroglioma 5%
ependymoma
infratentorial
supratentorial
spinal
5%
?
65
myxopapillary
subependymoma
choroid plexus
papilloma
rare
age
location & clinical pres.
histology
prognosis
oligodendrocyte tumors: molecular genetics determine prognosis
30cerebral white matter
focal calcification
LOH 1&19: 95% 5ys
50
yrs of neurologic sx
“fried egg” (perinuc. halo)
(most)
seizures
highly vascularized
CDKN2A del <2ys
or mixed astro/oligo
ependymal tumors: location, stage, tx 10 determine prognosis
th
0-10 4 ventricle mass
well-differentiated
spinal> cerebral>
inc. ICP/hydrocephalous
prominent vessels
infratentorial
rosettes & perivascular
medial survival 4y
all
cerebral/lateral ventricles
pseudorosettes
all
spinal intramedullary
rarely
malig. or infiltrative better w/more resection
high incid. in NF-2 pts.
& no CSF dissem.
inc. ICP/hydrocephalus
adult cauda equina/ filum
cuboidal cells around
usu. benign w/ tx
terminale
papillary cores
mucinous stromal
may recur or
degeneration
metastisize
adult 4th & lateral ventricles
benign, non-infiltrative
curable
usu. symptomatic
usu. very small
may cause hydrocephalus
neuroglial origin??
kids most lateral ventricles
well-differentiated
?
(4th in rare adult form)
papillary growth
rarely malignant
hydrocephalus
tx
surgery, radiation
& chemo
total resection
crucial, but
impossible in
brainstem (hence
poorer prog.
there)
total resection
necessary
surgical excision
?
CNS Tumors
Parenchymal & Meningeal
tumor
%
age
medulloblastoma
30%
kids
of
post.
fossa
meningioma
15 of
>30
head
25 of
spine
schwannoma
30%
of
spinal
neurofibroma
ganglioma
craniopharyngeoma 1-5
0-20
location & clinical pres.
cerebellum only
vermis – kids
lateral – adults
hydrocephalus
attached to dura:
50% convexity, 40% basal,
10% post fossa/spine
mass effects
histology
well-circumscribed
friable
hyerchromic nuclei
mets thru CSF to cauda eq
poorly defined cell sheets
cellular whorls
psammoma bodies
infiltrate bone w/
hyperostotic response
Nerve and nerve root tumors
usu. along CN8
benign, slow-growing
(acoustic neuroma) or
well-circumscribed
CN5
encapsulated
bilateral CN8 in NF-2
displaces nerve
also spinal, peripheral
Antoni A (dense spindle)&
Antoni B (loose stellate)
Verocay bodies
on nerves & nerve trunks Schwann cells mixed w/
usually multiple & part of
collagen, reticulin ,
NF-1 (plexiform nf is
fibroblasts
pathognomonic)
hyperplasia
3rd vent, hypothalamus, or well-circumscribed
temporal lobe
focal calcification
small cysts
Developmental tumors
bitemporal hemianopsia
from Rathke’s pouch (pit.
hypothalamus dysfnc.
epithelium)
hydrocephalus
varied but usu. cystic
whorls of keratin cells
calcification
prognosis
highly malignant BUT
very radiosensitive
up to 75% 5ys
dpds. on grade, stage,
and form
aggressive forms: clear
cell, rhabdoid,
choroid, papillary?*
tx
excision (total if
possible) +
radiation of brain
and cord
?
benign except
compression sx
resection if
necessary
chronic
resection if
necessary
most slow-growing
anaplastic quickly
deteriorate
?
ok if small
poor if >5cm diam.
resection as
complete as
possible
CNS Tumors
Miscellaneous
tumor
%
age
germinoma1
2
<20
location & clinical pres.
histology
Developmental tumors
pineal or post. 3rd ventricle identical to seminoma
prognosis
tx
dps on grade
radiation/chemo
worse w/CSF exten.
dermoid/
epi: pons, cerebellopontine cyts w/ strat. squamous epi may cause severe
epidermoid cysts
angle
keratinous debris
meningitis,
derm: midline post fossa
dermoid: sebaceous glands ependymitis
Non-neural tumors
capillary
1-3
20usu. cerebellum
endothelial-lined vascular benign: may secrete
hemangioblastoma
50
assoc. w/ systemic Lindau channels
epoietin-like substance
2
syndrome
lipid/foamy cytoplasm
primary sarcoma
1
usu. in meninges, choroid
fibroblast-derived
?
?
plexus
primary
1
in immunocompromised
B-cell type: DLCL, DLC- poor
poor response to
lymphoma
immunoblastic, or SLL
chemo
angiocentric growth
prod. reticulin
lots of necrosis
pituitary adenoma
mass effects, focal sx
uniform cells
?
?
25-30% PRL secretion
stippled,delicate chromatin
20-25% GH secretion
locally invasive
10-15% ACTH secretion
metastatic tumors
50% of all CNS tumors, esp. adults, usu. from lung, breast, melanoma, kidney, GI
usu. well-demarcated near white/gray border
1. germinomas are the most common CNS germ cell neoplasm (others: embryonal carcinoma, sinus tumor, choriocarcinoma, teratoma)
also most common tumor affecting pineal
2. incl. retinal involvement = von Hippel-Lindau