C.N.S. TUMORS Flashcards
General characteristics of CNS tunors
• In childhood, tumors are likely to arise from the _______, while in adults are mostly ————-.
posterior fossa
supratentorial
The anatomic site of CNS tumors can have lethal consequences irrespective of the histological classification
T/F
T
CNS tumors do not have detectable premalignant stages
T/F
T
CNS tumors often metastasize
T/F
F
They rarely metastasize
Clinical features of CNS tumors
•_________,_________ , etc
•______,_____________ deficits
Seizures, headaches
Focal neurologic
Classification of CNS Tumors
• Coverings of the brain (_______)
• Cells intrinsic to the brain (______, ______ tumors,_______ tumors)
• Other cell populations (_______,_______ tumors)
• Metastases
• Sellar region tumors e.g. _______,______________ tumour (PitNET)
Meningioma
gliomas ; neuronal ; choroid plexus
lymphomas; germ cell tumors
craniopharyngioma, pituitary neuroendocrine tumour (PitNET)
Gliomas
3 types
List!!!!
• Astrocytoma
• Oligidendroglioma
• Ependymoma
Astrocytomas
Can either be _______ or _________
• Fibrillary
• Pilocytic
Astrocytomas: Fibrillary
•_____ to ____ decade
• Commonly found in ________
• Grades are ________ (II), _____________ (III), ________ (IV)
4th to 6th
cerebral hemisphere
Diffuse (II), Anaplastic (III), Glioblastoma (IV)
Astrocytomas: Pilocytic
• (Children or Adults?)
•location : __________
• Relatively (benign or malignant ?)
Children
Cerebellum
benign
Oligodendroglioma
• _____ and _______ decades
•location : ______ with predilection for _____ matter
Fourth and fifth
Cerebral hemispheres; white
Oligodendroglioma
• Common genetic finding is loss of ___________ for chromosomes _____ and ——-
heterozygosity
1p & 19q
Who has a better prognosis
Oligodendroglioma or Astrocytomas
Oligodendroglioma has a Better prognosis than astrocytomas
Ependymoma
•_______ decades of life
• Most often arise next to the _______________________________ including the _______ of the spinal cord.
• In adults, most common in the _____
First two
ependyma-lined ventricular system
central canal
spinal cord
Meningioma
• (Benign or Malignant?)
• Originate from _______ cells of the ______
• Usually (poorly or well?) demarcated
• Attached to the ___ with ______ of the underlying brain
Benign
meningothelial; arachnoid
Well
dura; compression
Meningioma
__________ pattern of cell growth and _______________ bodies.
Whorled
psammomatous
Meningioma
Subtypes:
•_________
• _________
• ___________
Syncytial
Fibroblastic
Transitional
Medulloblastoma
• (Children or Adults?)
• _________ location
• Largely (differentiated or undifferentiated?) tumor
•highly (benign or malignant?) .
• Exquisitely radio(sensitive or resistant?)
Children
Cerebellar
undifferentiated
malignant
Medulloblastoma
• _______ + _______ = high survival rate
Excision + Radiation
Nervous system tumors
• \___________
• _____________
• ________ tumors
• Schwannoma
• Neurofibroma
• Metastatic tumors
Schwannoma
• Benign or Malignant?
• Located often at the __________, attached to the ______ branch of the ________ nerve
Benign
cerebellopontine angle
vestibular; eigth cranial
Schwannoma
• Present with ______ loss/ ______
• Maybe associated with _____ gene mutation.
hearing; tinnitus
NF2
Schwannoma
Hypo/hypercelllular areas known as _______ and ___________
• In between are areas of nuclear _______ known as ______ bodies
Antoni A and Antoni B.
palisading
Verocay
Metastatic tumors to the CNS
• Lung, breast, skin (melanoma), kidney, GIT are least common.
• Sharply demarcated.
T/F
F( Most )
T
Sellar Region Tumors
Tumors of the pituitary gland
• _____________
• ___________________ tumor (pituitary ____________)
Craniopharyngioma
Pituitary neuroendocrine
adenoma
Craniopharyngioma
WHO grade ___
(Benign or Malignant?)
Usually _______ location but frequently extends into the ______ and neighbouring structures.
1
Benign
suprasellar
Craniopharyngioma
Two types: _______ craniopharyngioma & _________ craniopharyngioma
Papillary
Adamantinimatous
Craniopharyngioma
Clinical features: ______ disturbances, endocrine deficiencies e.g.
_________
hormone (_____ failure)
Diabetes insipidus, headachee etc
visual
Growth hormone (growth failure)
Craniopharyngioma
________ hormone is the most commonly affected hormone.
Growth
Most common intraocular malignancy in children is ??
Retinoblastoma
Retinoblastoma
Due to loss/inactivation of _____ gene (2-hit hypothesis) m
RB1
Retinoblastoma
Classification
_______ (unilateral)
__________ (bilateral)
When associated with ————— (trilateral)
Sporadic
Inherited
Pineoblastoma
Retinoblastoma
Classification
Sporadic (_____lateral)
Inherited (____lateral)
When associated with pineoblastoma (____lateral)
uni
Bi
Tri
Retinoblastoma
Loss/inactivation of ______________ gene
A ____________ gene.
________ locus
both alleles of RB1
Tumor supressor
13q14.2
Retinoblastoma
2 hit hypothesis
First mutation is ____________
2nd mutation is ________
somatic/germline.
somatic
retinoblastoma
Earlier age of onset
Usually ___lateral involvement
May have other malignancies e.g. _____________
bi
osteosarcoma
Germline mutations in RB1 lead to _________________ retinoblastoma
inherited/familial
Retinoblastoma
Clinical features: _________ (white reflex), _____,_______, ___ pain, detachment.
On histology, ______ and ———— are seen.
Leukocaria
glaucoma, strabismus
eye
rosettes and fleurettes
Retinoblastoma
Two types of rosettes:
_______________ rosettes
_____________ rosettes
Flexner-Wintersteiner
Homer Wright
Retinoblastoma
Treatment:
————
___________
Chemotherapy Enucleation
CNS tumors: By the cell type
Pineal tumors
Tumors of the ______ and ______
nerves(e.g., schwannoma, neuroma, neurofibroma)
Mesenchymal nonmeningeothelial
tumors(e.g., _____,______,_______,________)
Germ cell tumors (e.g., _______)
Pineal
hemangiomas, hemangioblastoma, lipoma, hamartoma
teratoma
Primary CNS tumors do not metastasize to organs outside the CNS.
T/F
T
Drop metastases (intra____, extra______ ,____ metastases ) and ________ metastases may occur.
Typically manifest as _____ along the _______ and ________ that can cause back pain with neurologic symptoms (e.g., limb weakness)
Can be detected by ________
dural; medullary; spinal
leptomeningeal
nodules
spine and cauda equina
lumbar puncture
Most common primary brain tumor in adults is ??
Glioblastoma
Glioblastoma
Often crosses ________, forming a ______ glioma
corpus callosum
Butterfly
Glioblastoma
Express a protein called _____ (__________________Protein)
GFAP
Glial Fibrillary Acidic
2nd most common brain tumor in adults is ??
Meningioma
Meningioma
Arise from _______ cells
arachnoid
Meningioma
“_______” - external to brain
Can have ______ attachment (“tail”)
Extra-axial
dural
Meningioma
Classically affects (male or female?) more than (males or females?)
Expresses ______ receptor
Female
Male
estrogen
3rd most common adult primary brain tumor is ??
Schwannoma
Cerebellopontine angle is where ______ never and ________ nerve emerge from
Facial nerve and vestibulocochlear nerve
Schwannoma
Stain positive for protein ———
S-100
Neurofibromatosis
Associated with ___________
Autosomal __________ disease
schwannomas
dominant
Neurofibromatosis
Mutation _____/_____ genes Neurofibromas
_____ nodules
___________ spots
NF1 /NF2
Lisch
Café-au-lait
Neurofibromatosis
Type 1:
(Most of Least?) common
————- spots, _________
Most
Café-au-lait
Neurofibromas
Neurofibromatosis
Type 2:
_______________ (almost all patients)
Meningiomas
______ tumors
MISME: —————————
Bilateral schwannomas
Multiple
Multiple inherited schwannomas, meningiomas, and ependymomas
Oligodendroglioma
Usually in _______ lobe
frontal
Pituitary adenoma
Most commonly secrete _______, leading to ______,_______,________ etc
prolactin
Amenorrhea, galactorrhea, impotence
Pituitary Adenoma
Bitemporal ________
<___mm = microadenoma
>____mm = macroadenoma
hemianopsia
10
10
1: Can produce ____ , leading to _____________
Hemangioblastoma
Two key facts to know
EPO; polycythemia
von Hippel-Lindau
Most common brain tumor children is ??
Pilocytic astrocytoma
Pilocytic astrocytoma
(Low or High?) -grade astrocytoma
Contain _______ fibers
Low
Rosenthal
Medulloblastoma
Highly (benign or malignant?) primary brain tumor
Usually occurs in (children or adults?)
Usually occurs in ________
Often in the midline ( ________)
malignant
Children
cerebellum
truncal ataxia
Medulloblastoma
Is a Type of ___________________________________________ tumor (PNET)
primitive neuroectodermal
Medulloblastoma
___% children survive to adulthood. Many with ___________________
Can compress _________ , leading yo hydrocephalus
75; complications of treatment
4th ventricle
Nodules in dura of spinal cord: ___________
Drop metastasis”
Craniopharyngioma: location
•____________: Anywhere pituitary gland → _______________
Suprasellar
base of 3rd ventricle
Craniopharyngioma
Derived from ______________
Often ———- and _____
Contain _______ cells
remnants of Rathke’s pouch
calcified and cystic
epithelial
Craniopharyngioma
Can compress the ________, causing ______________
optic chiasm
Bitemporal hemianopsia
Craniopharyngioma
Enucleation: __________________ and __________________ between the globe and the orbit.
removing the eye from the orbit
separating all tissue connections