Ch28, Tumors Flashcards
Location of primary tumors in adults
cerebral cortex
above tentorium
Lcoation of priamry tumors on children
posterior fossa
below tentorium
single lesion;
also associated with what other clinical finding?
primary tumor
striking edema
multiple lesions
metastasis; not a primary tumor
___% of adult primary tumors are infiltrating astrocytomas
80%
Age of onset of infiltrating astrocytoma
40-60yo
Presenting signs of infiltrating astrocytoma
seizure, HA, focal neuro deficity
Types of infiltrating astrocytoma
diffuse astrocytoma (2/4) anaplastic astrocytoma (3/4) glioblastoma (4/4)
Glioblastioma (4/4) morphology
necrotic edges»_space; pseudopalisading
GFAP
intermediate filament present in glial cells in Glioblastioma (4/4)
Glioblastioma (4/4) prognosis
15 months and 25% alive at 2 years
Pilocytic astrocytoma location
floor/wall of 3rd vent
optic nerve
cerebral hemishpheres
Pilocytic astrocytoma age group
children, YA
Pilocytic astrocytoma gross morphology
mural nodile in wall of cyst, posterior fossa
Rosenthal fibers and microcysts present in ____ astrocytoma
Pilocytic astrocytoma
Location of pleomorphic xanthoastrocytoma (2/4)
temporal lobe of young pt
Pleomorphic xanthoastrocytoma
Infiltrating astrocytoma
Oligodendroglioma
Dysembryoplastic neuroepothelial tumor
seizures
Age by which most BS Gliomas occur
20yo
- Pontine glioma
- Tectal gliomas
- Cortocomedullary junciton tumors
(Location) of most to least aggressive BS gliomas
Morphology - Calcification tumor in white matter
Location - Frontal lobe
Seizure presenteation
Mid life; 5-15% of gliomas
Oligodendroglioma
Age of oligodendroglioma onset
midlife (5-15%)
Oligodendroglioma is d/t loss of heterozygosity in what chromosomes?
1p
19q
Ependymoma:
Age + Most common location (for adults and children
0-20yo + 4th ventricle
Adults + SC central canal
Ependymoma:
if not radiated correctly, growth down thru 4th vent»_space; ventricular space blockage = ??
hydrocephalus
adult ependymoma associated with which NF?
NF2
Myxopapillary ependymoma location
distal SC, philum terminale
Paraventriculat mass lesions:
solid, may be calcified, attached to ventricular slinign
subependymoma
Ependymoma Subependymoma Choroid plexus papilloma Colloid cysts of 3rd vent Medulloblastoma
hydrocephalus
colloid cysts of 3rd vent = noncomminicating
most common CNS tumor of mature neurons
Ganglioma
rare, low grade childhood neoplasm often presenting as a seizure disorder;
Dysembryoplastic neuroepothelial tumor
prognosis of Dysembryoplastic neuroepothelial tumor
prognosis after resection is good
“floating neurons” indicative of this
Dysembryoplastic neuroepothelial tumor
neoplasm exclusively in cerebellum and children (20%)
medulloblastoma
Homer Wright rosettes and small round blue cells
medulloblastoma
indicative of what neoplasm?
WNT type mutatations (ch6)
SHH type
medulloblastoma
i17q: neoplasm and prognosis
medulloblastoma; poor prognosis
What type of neoplasm is extremely radiosensitive?
medulloblastoma
Chromo22 deletion; hSNF1/INI1 gene (chromatin remodeling)
atypical teratoid/rhabdoid tumor
age + location of atypical teratoid/rhabdoid tumor
young children + posterior fossa and supratentorium
survival of atypical teratoid/rhabdoid tumor
less than 1 year`
virus infecting almost all pts with primary CNS lymphoma
EBV
tumor that grows within small vessels; leads to microscopic infarcts
intravascular lymphoma
What presents with vasculitis and dementia?
intravascular lymphoma
Germ cell tumors:
Race
Japanese > Europeans
does primary CNS lymphooma respond well to chemo?
no
Common location of primary brain germ cell tumors
along midline:
pineal (esp males) and suprasellar rections
what do you always need to rule out if you see something that looks liek a pineal tumor?
testicular germ cell tumor
Type of pineal parenchymal tumor that tends to affect adults + is it well differentiated or aggressive?
pineocytoma + well differentiated
Type of pineal parenchymal tumor that tends to affect children + is it well differentiated or aggressive?
pineoblastoma + aggressive
spread throoughout CSF
are meningiomas benign or malignant?
what type of cells
gender
ALWAYS benign
arachnoid cells
women > men
Presentation of meningiomas
seizure
round mass attached to dura
progesterone
What are psammoma bodies + assocaited with what type of tumor
calcification of syncytial nests of meningothelial cells
meningiomas
meningiomas associated with what chromosome, what NF, and loss of what gene?
Chromo22, LOH of long arm
NF2
loss of merlin
What makes meningiomas dangerous?
slow growth that manifests d/t CNS compression
most common primary sites that metasticize to brain
lung breast kidney GI skin
Name of syndrome with PTCH mutation resulting in medulloblastoms
gorline syndrome
Name of syndrome with APC or mismatch repair gene mutation resulting in medulloblastoms/glioblastoma
Turcot syndrome
Name of syndrome with p53 mutation resulting in medulloblastoms
Li-Fraumeni Syndrome
Name of syndrome with PTEN mutaiton, resulting in dysplastic cerebellar gangliocytoma
Cowden syndrome
Characterized by angioma, seizure, metnal retardation
Tuberous Sclerosis Complex
Tuberouis SClerosis Complex:
TSC1 encodes…
TSC2 encodes…
hamartin
tuberin
Assocated with:
- Polycythemia and posterior fossa cystic mass
- EPO/high RBC
- Dysregulated HIF-1»_space; increased vascualr endothelial GF
VHL
NF-type?
Plexiform, optic nerve glioma, Lisch noduels, cafe au lait
NF1
NF-type?
schwannoma (B/L CN8), meningioma (multiple), ependymoma
NF-2
NF-2 chromo and gene
chr22, merlin
Schwanomma - beinign or malignant?
BENIGN peripheral nerve sheath tumor
location commonly associated with CN8 schannoma
cerebellopontine angle
acoustic neuroma (tinnitus and hearing loss)
shchwannoma
carney complex
schwannoma
antoni __ = (more cellular) elongated cells with cytoplasmic processes arranged in fascicles
A
antoni __ = (less cellular) less densly cellular tissue with microcysts and myxoid changes
B
s100 positive
Schwannoma
plesiform neurfibroma found at what locatoin and associated with whath NF
NF-1
deep or superficial locations assocaited with nerve roots or large nerves
loss of both of what genes = NF1
FN1 genes
Antoni A and B associated iwth what tumor type?
Schwannoma
Diffuse neurofibroma infiltrates what part of CT
dermis and subcutaneous
arises de novo (not from schwannoma) from plexiform neurbibroma transformaiton
MPNST
MPNST associated with what NF?
NF1
