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TUMORS of the CNS - Coggle Diagram
TUMORS of the CNS
primary tumors
astrocytoma
pilocytic astrocytoma
(grade I)
children / young adults
often subtentorial
frequent regressive changes
low cellularity
no atypia and mitoses
bipolar cells
fibrillary cores
NF1 association
favorable outcome
Rosenthal's fibers in the form of eosinophilic granular bodies
regressive changes:
cystic degeneration
ischemic necrosis
calcifications
grades II, III, IV
morphologic criteria for grading
cellularity
mitotic index
nuclear atypia (pleomorphism)
microvascular proliferations
necrosis
molecular criteria
diffuse astrocytoma
(grade II)
30-40 y
4-8 y survival
infiltrative
few mitoses
Ki-67 <4%
monomorphic
low cellularity
forms
fibrillary
protoplasmic
gemistocytic
anaplastic astrocytoma
(grade III)
41 y
2-5 y mean survival
increased cellularity
increased atypia
increased mitotic index
Ki-67 10-15%
glioblastoma
(grade IV)
53 y
hemispheric white matter
multiforme
from giant cells to pseudo-glandular to sarcomatous (DD with metastases)
GLIOSARCOMA
diagnostic features
marked atypia
microvascular proliferations
ischemic palisading necrosis
newly formed vessels due to the overexpression of VEGF and VEGFr
GLIOMAS
glioblastomas
de novo: early appearance of molecular alteration
secondary: following the progression from astrocytomas of grades II-IV
IDH mutations
associated with a better prognosis
it can be present or not
for DD also look for co-deletion 1p/19q
positive in oligodendrogliomas
negative in astrocytomas
on the basis of this:
glioblastoma IDH wild-type
high-grade
predominant astrocytic differentiation
marked nuclear atypia
pleomorphism
high mitotic activity
microvascular proliferation
necrosis
glioblastoma IDH mutated
less frequent necrosis
more frequent oligodendroglioma-like features
median survival 31 months
glioblastoma NOS
IDH test not performed
variants
giant cell
gliosarcoma
epithelioid glioblastoma (50% with BRAF V600E)
TERT mutation variant
EGFr mutations
new 2022 classification
based on METHYLATION status
treatment based on TEMOZOLOMIDE
MGMT enzyme makes the cells less sensitive to this alkylating agent
oligodendroglioma
no grade I
grade II and III differ in cellularity and atypia
necrosis and microvascular proliferations only in grade III
grade II
supratentorial
frontal lobes
recurrent seizures
infiltrative
chemosensitive (alchylating agents: temozolomide)
5 y survival (40-60%)
appearance
small cells
homogenoeus nuclei
honeycomb cytoplasmic appearance
branched vascular network
micro / macrocalcifications
in the presence of increased cellularity, atypia and microvascular proliferations
anaplastic oligodendroglioma (WHO III)
from 2016: new classification based on molecular markers
better prognostic stratification
better reproducibility
better selection of therapeutic strategies
co-deletion 1p/19q in 100% of cases
ependymoma
classified on the basis of the sublocation
in children (6 y):
intracranial (posterior fossa)
in adults (30-40 y):
spine (10% survival in 10y)
morphological architecture
pseudorosettes
perivascular localization
fibrillary
rare necrosis
vascular hyalinosis
variants:
sub-ependymoma
cellular
papillary
clear cells
myxopapillary
anaplastic
GFAP+
(in particular in the less mature forms)
MYCN amplification is associated with a worse prognosis
(aggressive form)
medulloblastoma
EMBRYONAL TUMOR
7-10 y; 20-40 y
SUBTENTORIAL (cerebellum)
origin from neuroblasts of external germinal layer
highly malignant
good response to CT/RT/surgery
intra-CSF dissemination
GFAP +/- (not the actual marker)
NF is the key marker
morphological types
classic:
75%
completely undifferentiated
coexpression of neuronal and glial markers
desmoplastic / nodular:
20%
dense stroma
high apoptotic index
reticulin ++
large cell:
5%
predominant neuronal differentiation
melanotic:
rare
melanoma metastasis-like
look for NF+
for DD with melanoma:
melanin
HNB45
SOX5
medullo-myosarcoma:
rhabdomyosarcoma metastasis-like
also in children
familial forms
syndromes:
Li-Fraumeni
Turcot
other embryonal tumors:
Wilms
adverse prognostic parameters
metastatic disease at diagnosis
age <3 y
non-radical surgery
intermediate risk if all neg
other embryonal tumors
supratentorial PNET
same as medulloblastoma
different site
more frequent in sellar region
age 1-10 y
complete loss of differentiation / neuronal differentiation
pinealoblastoma
pineal gland localization
atypical rhabdoid teratoid tumor
children
sub-tentorial, supra-tentorial or spine
highly malignant
similar to a tumor of the kidney
epithelioid and sarcomatous forms
rosettes formation
molecular groups
TYR
SHH
MYC
meningioma
usually low-grade
(also malignant forms exist)
origin from cells in the arachnoid
age: 50-70 y
M:F = 1:2
single or multiple
location: central or spinal
nodular or plaque
not benign, simply low-grade malignancy
low mitotic index and low proliferation index
(<4x10 HPF / Ki-67< 4%)
TERT mutations are associated with aggressive phenotypes
mandatory evaluation in the presence of a meningioma!
grading
grade I
most frequent
grade II
atypical morphology
classified as choroid or clear cell
grade III
anaplastic, papillary or rhabdoid
increased mitotic index up to 20
molecular alterations
general information
association between age and localization
adults: supratentorial
children: subtentorial
signs and symptoms
edema (vasogenic / herniation)
compression / infiltration
functional deficits / irritative stimuli
lethal infiltration in low grade forms too
Intraparenchymal and intraliquoral dissemination
even at the advanced stage, lack of systemic symptoms
primary vs metastatic
IHC markers
GFAP
primary tumor of the glia when present alone
metastases