CNS tumors- Norton Flashcards
where do most brain tumors occur in children
posterior fossa
where do most brain tumors occur in adults
supratentorial
what can be found on the PE for a primary brain tumor
fundoscopic exam: papilledema CN3 and 6 nerve palsy increase ICP headache hemiparesis aphasia seizures
tumors in the ventricles present with what clinical features
hydrocephalus
what tool is used to diagnose brain tumor
MRI with contrast
BBB does not allow contrast but the new vessels in tumor do
what is the only risk factor for brain tumors
ionizing radiation
what are the glioma tumors impact what cells and create their own tumors
astrocytes
oligodendrocytes
Ependymal cells
what are the two types of Astocytoma
infiltrating
noninfiltrating
what is the most common genetic mutation for astrocytoma infiltrating
p53
over expression of platelet derived growth factor
what is the histologic grading for astrocytomas I -IV
I: well-differentiated- pilocytic astrocytoma
II: diffuse growth of well-differentiated astrocytes-diffuse or fibrillary astrocytoma
III: anaplastic features (pleomorphism, increased mitosis) - anaplastic astrocytoma
IV: undifferentiated w/necrosis - gliobastoma
name a type of astrocytoma infiltrating that occurs in younger adults
diffuse astrocytoma
what is the histology for diffuse astrocytoma
- increased number of glial cells
- variable nuclear pleomorphism
- GFAP-positive astrocytic processes give a fibrillary background
how long do patients survive with diffuse astrocytoma
5 years
Name a astrocytoma infiltrating tumor that has regions of more densely cellular with greater nuclear pleomorphism, mitotic figures
anaplastic astrocytoma
what tool is used to diagnose diffuse and anaplastic astrocytoma infiltrating
MRI
brain biopsy
treatment options for diffuse and anaplastic astrocytoma infiltrating
surgical resection
radiation
survival rate for diffuse and anaplastic astrocytoma infiltrating
2 years
name the astrocytoma infiltrating tumor that is high grade end of spectrum of astrocytomas, most atypical and mitotically active
gliobastoma
what is the treatment for gliobastoma
surgery
radiation
chemotherapy
glioblastoma usually occurs in who and what is the prongosis
5th - 7th decade (40s-60s)
always fatal
what does the histology for glioblastoma look like
densely cellular, vascular proliferation, necrosis and pseudopalisading (ring shape)
Name a non-infiltrating astrocytoma
pilocytic
who usually gets pilocytic astrocytomas and what is the prognosis
children and young adults
lived for 40 years
where does pilocytic astrocytoma usually occur in the brain? what grows
cerebellum
third ventricle
cystic with mural nodule in wall of cyst
what does pilocytic astrocytoma look like under the microscope
cells with hair-like processes
Rosenthal eosinophilic fibers
Name a type of tumore that occurs in the 30s - 40s that is slow growing, may be diagnostic after years of neurologic complaints, and often seen in cerebral hemisphere
Oligodendroglioma
what does oligodendroglioma look like under the microscope
sheets of regular cells with spherical nuclei
surrounded by clear halo of cytoplasm
what is the genetics of oligodendroglioma
deletions of chromosome 1p and 19q
what is the treatment and survival of oligodendroglioma
surgery radiation and chemotherapy
5-10 years
Ependymoma usually occurs in who and where in the brain for adults and young people
children and young adults
ependymal-lined ventricles
children/young adults: 4th ventricle
adults: spinal cour
ependymoma presents with what?
hydrocephalus
what is seen under the microscope for ependymoma cells
small dark cells with perivascular pseudorosettes
benign appearing
what is the molecular genetics for ependymoma tumors with spinal cord lesions and supratentoral ependymomas
spinal cord: mutation in NF2 gene on chromosome 22
supratentorial: alterations in chromosome 9
prognosis for ependymoma
5 years after surgery
better for supratentorial and spinal then posterior fossa
what is myxopapillary pendymoma
in filum terminale
may extend into subarachnoid space
surround roots fo cauda equina
Choroid plexus papillomas is most common in who and location of tumur in adults and childres
common children: lateral ventricle
adults: 4th ventricle
what does choroid plexus papillomas look like under microscope
normal choroid plexus
how do people with choroid plexus papillomas present
hydrocephalus
who usually gets colloid cyst of 3rd ventricle? location? what can this obstruct? what can it cause? prognosis ? clinical complaint
children roof of ventricle foramina of monro hydrocephalus fatal complain of positional headache
What is the histology of colloid cyst of 3rd ventricle
spherical mass filled with mucinous material
-simple epithelial lining may contain ciliated or goblet cells
Name two embryonal tumors
medulloblastoma
CNS PNET
where does medulloblastoma occur in the brain and who usually gets it
cerebellum only, midline
children
what does medulloblastoma look like under the microscope
small round blue cell tumor
rosettes or perivascular pseudorosettes
what is the molecular genetics for medulloblastoma
loss from 17p
medulloblastoma is sensitive to what and prognosis
radiosenstive
5 yr
what brain tumor occurs in children in the cerebral hemisphere. resembles medulloblastoma in histology and genetically distinct from medulloblastoma
CNS supratentroial primitive neuroectodermal tumors CNS PNET
What is the most common CNS neoplasm in immunosuppressed patients
primary brain lymphoma
do primary brain lymphomas spread
not to lymph nodes or outside of brain
most common sites for metastic tumors
Lung breast skin kidney GI lots of bad stuff kills gila
this is actually not a true brain tumor, arises from meningothelial cells from external membranes covering the brain
meningioma
how can the patient present in the clinical with Meningioma
headache or neurological symptoms
not usually found until autopsy
multiple meningiomas are found in patients with what
neurofibromatosis type 2
what is seen on an MRI for meningiomas
“dural tail”
what does meningioma look like under the microscope
whorls of bland cells
psammoma bodies
where does Craniopharyngioma occur
suprasellar asrising in pituitary stalk
arise from remnants of Rathke’s pouch
sold or mixed solid and cystic
what is the age distribution for carniopharyngioma
5-14 yrs
50-75 yrs
what is the histologic feature of craniopharyngioma in children
adamantinomatous
- stratified squamous epithelium in nests or chords
spongy reticulum
calcifications
lamellar keratin formation
cysts with thick brownish-yellow fluid content
what is the histologic feature of craniopharyngioma in adults
squamous epithelium lined solid sheets and papillae
no keratin, calcifications or cysts
what are clinical symptoms for craniopharyngioma
visual from pressure on optic chiasm
endocrine symptoms from compressed pituitary
headache
what is the treatment for craniopharyngioma
surgery
radiation therapy
what is commonly seen at cerebellopontine angle where attached vestibular branch of 8th nerve - also called acoustic neuroma
schwannoma
how do patients with schwannoma present in the clinic
tinnitus and hearing loss
Schwannoma is associated with what
neurofibromastosis type 2
Schwannoma shows a mixture of what 2 growth patterns
Antoni A: moderate to high cellularity with nuclear palisading ; “ nuclear-free zones” called Verocay bodies
Antoni B: less cellular area, more myxoid
Neurofibromas are benign spindle cell lesions which occur in what 2 forms
cutaneous or solitary
plexiform
cutaneous or solitary neurofibroma occurs where
skin or peripheral nerve
never turns malignant
plexiform neurofibromas occurs in what type of patients
with NF
potential malignant transformation
what are clinical features of neurofibromastosis 1
gliomas of optic nerve
pigmented nodules of iris ( Lisch nodules)
Cafe au lait spots: brown spots on skin
neurofibromastosis 1 is associated with what gene
NF1 gene tumor suppressor gene
chromosome 17
which neurofibromastosis develop a range of tumors
neurofibromastosis 2
neurofibromastosis 2 what chromosome association
chromosome 22
what tumor has CNS hamartomas- occur as haphazardly arranged neurons which lack normal neural oragnziation “cortical tubers”
Tuberous sclerosis
where do extracranial lesions occur for tuberous sclerosis
renal angiomyolipomas
pulmonary and cardiac myomas
skin rash
Von-Hippel-Lindau develop what
capillary hemangioblastomas in cerebellum, retina, and spinal cord
- highly vascular tumor
von-Hippel_lindau can also have cysts where else
pancreas liver and kidney
