Brain Tumors B&B Flashcards
what are the 5 most common tumors in adults, in order?
- Glioblastoma.
- Meningioma.
- Schwannoma.
- Oligodendroma
- Pituitary adenoma
Most adult tumors occur above tentorium (supratentorial)
what are the 5 most common tumors in children, in order?
- Astrocytoma.
- Medulloblastoma.
- ependymoma
- hemangioblastoma
- Craniopharyngioma.
Most child tumors occur below tentorium (infratentorial)
[ASTROnauts BLAST-off, ENDing HuMAN Curiosity]
where are most common metastasis to brain? (3)
lung, breast, renal
(also melanoma, colon)
usually multiple lesions on brain MRI (primary usually single)
where does the most common primary brain tumor in adults develop? what is the prognosis?
glioblastoma: occurs in cerebral cortex, often crosses corpus callosum (“butterfly glioma”), expresses GFAP
rapidly progressive and malignant, usually fatal within a year (older age = worse prognosis)
rapidly progressive and malignant brain tumor which expresses GFAP, affecting adults (50% >65yo)
What is it and where does it occur?
glioblastoma: occurs in cerebral cortex, often crosses corpus callosum (“butterfly glioma”), expresses GFAP
most common primary brain tumor in adults
describe the histology finding of glioblastoma
glioblastoma: occurs in cerebral cortex, often crosses corpus callosum (“butterfly glioma”), expresses GFAP
exhibit pseudopallisading: nuclei of tumor cells line up like stakes along edge of necrosis
highly malignant, most common primary brain tumor in adults
histology shows central area of necrosis with pseudopallisading tumor cells surrounding. cells are GFAP+
what type of tumor is this
glioblastoma: occurs in cerebral cortex, often crosses corpus callosum (“butterfly glioma”), expresses GFAP
highly malignant, most common primary brain tumor in adults
where do meningiomas occur and in whom?
2nd most common primary brain tumor in adults (F>M bc tumor expresses estrogen receptors!)
occurs in convexities of hemispheres near surface of brain, arises from arachnoid cells
“extra-axial” - external to brain, can have dural attachment (“tail”)
“extra-axial” brain tumor arising from arachnoid cells
meningioma: 2nd most common primary in adults (F>M), occurs in convexities of hemispheres near surfaces of brain - appears on outside, pushing its way in
can have dural attachment (“tail”)
how do meningiomas present?
2nd most common primary in adults (F>M), occurs in convexities of hemispheres near surfaces of brain
usually benign with no metastasis, resectable, often asymptomatic (sometimes cause seizures)
Which receptors do meningiomas express and what is the clinical relevance of this?
2nd most common primary in adults (F>M), occurs in convexities of hemispheres near surfaces of brain
express estrogen receptors —> classically affects females more than males
what is an important risk factor for meningioma?
prior radiation to head (childhood malignancy) - latency period ~20 years
2nd most common primary in adults (F>M), occurs in convexities of hemispheres near surfaces of brain, arises from arachnoid cells
how do parasagittal meningiomas present?
Compress the leg area (medial surface of lobes) similar to an ACA stroke —> lower extremity weakness
2nd most common primary in adults (F>M), occurs in convexities of hemispheres near surfaces of brain, arises from arachnoid cells
what does histology of meningioma show?
2nd most common primary in adults (F>M), occurs in convexities of hemispheres near surfaces of brain, arises from arachnoid cells
histology shows psammoma bodies
57yo F w PMH of childhood malignancy presents with lower extremity weakness. MRI reveals brain tumor. Biopsy shows psammoma bodies. What type of tumor is this?
meningioma: 2nd most common primary in adults (F>M - expresses estrogen receptor), occurs in convexities of hemispheres near surfaces of brain
parasagittal meningioma can compress leg area, similar to ACA stroke
prior head radiation is risk factor (~20 year latency)
what 3 symptoms present with schwannomas?
- hearing loss
- tinnitus
- ataxia
form at cerebellopontine angle, classically located to CN VIII
what do schwannomas stain positive for?
S-100 protein
schwannomas are closely associated with which disease? how will this present?
neurofibromatosis 2: AD mutation in NF1/NF2
aka MISME: multiple inherited schwannomas, meningiomas, and ependymomas
bilateral schwannomas + meningiomas + ependymomas =
neurofibromatosis type 2
where do oligodendrogliomas occur, and how do they present?
rare/slow growing tumor of white matter, usually in frontal lobe —> often present with seizures
describe the histological appearance of oligodendroglioimas
rare, slow-growing tumor of white matter in frontal lobe (seizures)
cells histologically resemble fried eggs
46yo pt presents with new onset seizures. brain MRI reveals tumor of the frontal lobe. biopsy reveals cells with clear cytoplasms, giving the appearance of fried eggs. what is the diagnosis?
oligodendroglioma: rare, slow-growing tumor of white matter
affects adults
what is the most common primary brain tumor in children, and where do they occur?
pilocytic astrocytoma: low grade astrocytoma, usually in posterior fossa (cerebellum)
usually benign w/ no metastasis, well-circumcised and cystic/solid, often successfully treated with surgery
GFAP positive + Rosenthal fibers
for what do pilocytic astrocytomas stain positive?
pilocytic astrocytoma: low grade astrocytoma, usually in posterior fossa (cerebellum)
usually benign w/ no metastasis, well-circumcised and cystic/solid, often successfully treated with surgery
GFAP positive + Rosenthal fibers… this makes sense because astrocytes express GFAP, and Rosenthal fibers are seen whenever there is gliosis
child presents with tumor in cerebellum which stains GFAP+, and biopsy shows Rosenthal fibers. what is the dx?
pilocytic astrocytoma: low grade astrocytoma, usually in posterior fossa (cerebellum)
usually benign w/ no metastasis, well-circumcised and cystic/solid, often successfully treated with surgery
where do medulloblastomas occur, and how do they present (and in whom)?
rare, highly malignant primary brain tumor affecting children - this makes sense because they are type of primitive neuroectodermal tumor (PNET)
usually occur in midline cerebellum —> truncal ataxia
7yo pt presents with truncal ataxia and is found to have a tumor in the midline of their cerebellum. biopsy shows primitive neural cells. what is the diagnosis and prognosis?
medulloblastoma: rare, highly malignant primary brain tumor affecting children, type of primitive neuroectodermal tumor (PNET)
however, can be treated with surgery/radiation/chemo, and 75% of children survive to adulthood - but many with complications of treatment
what are 2 complications of medulloblastomas?
rare, highly malignant primary brain tumor affecting children, usually occur in midline cerebellum —> truncal ataxia
- can compress 4th ventricle —> hydrocephalus
- can spread to CSF —> ”drop metastasis” causing nodules in dura of spinal cord
what occurs as a result of “drop metastasis” of medulloblastoma?
rare, highly malignant primary brain tumor affecting children, usually occur in midline cerebellum (truncal ataxia)
can spread to CSF, causing nodules in dura of spinal cord (“drop metastasis”) - tend to occur in lower spinal cord/cauda equina —> back pain, focal neuro lesions can occur
what is the characteristic pathology finding of medulloblastomas?
rare, highly malignant primary brain tumor affecting children, usually occur in midline cerebellum (truncal ataxia)
histology shows Homer-Wrights rosettes: tumor cells form circles with space in middle that resemble church stain glass rosettes
where do ependymomas develop, and in whom?
ependyma = epithelium-like lining of ventricles
occur in children, develop in brain and spinal cord, often found in 4th ventricle —> hydrocephalus
what is the characteristic histology finding of ependymomas?
occur in children, develop in brain/ spinal cord, often found in 4th ventricle (hydrocephalus)
histology shows pseudorosettes: tumor cells surround central clearing, but core is blood vessel (not totally clear as in rosettes of medulloblastomas)
child presents with hydrocephalus, and it is discovered they have a tumor in their 4th ventricle. biopsy shows tumor cells arranged around a blood vessel core. what is the dx?
ependymoma: occur in children, develop in brain/ spinal cord, often found in 4th ventricle
histology shows pseudorosettes: tumor cells surround central clearing, but core is blood vessel (not totally clear as in rosettes of medulloblastomas)
with what genetic disease are hemangioblastomas associated? in whom do they occur?
hemangioblastomas: very rare, slow growing CNS tumors that are well-circumscribed and highly vascular, occur in children
occur with von Hippel Lindau syndrome: AD mutation in tumor suppressor gene —> lots of tumors throughout body (hemangioblastomas, retinal angiomas, renal cell carcinomas, pheochromocytomas)
what do hemangioblastomas secrete? what is the clinical effect of this?
hemangioblastomas: very rare, slow growing CNS tumors that are well-circumscribed and highly vascular, occur with von Hippel Lindau syndrome
can produce EPO —> polycythemia
pt is 9yo F found to have tumor in their cerebellum. Imaging shows a well-circumscribed, highly vascular tumor. Serum studies reveal polycythemia. What is the dx?
hemangioblastoma: very rare, slow growing CNS tumors that are well-circumscribed and highly vascular, occur with von Hippel Lindau syndrome
can produce EPO —> polycythemia
what are the symptoms (3) of craniopharyngiomas? in whom do they occur?
rare, benign, suprasellar (above pituitary to base of 3rd ventricle) tumors, occur in ages 10-14
symptoms are from compression —> hormonal imbalance (pituitary), behavioral change (frontal lobe), bitemporal hemianopsia (optic chiasm)
where do craniopharyngiomas develop, and from what are they derived?
occur in children (10-14), derived from remnants of Rathke’s pouch: invagination of ectoderm that protrudes from roof of mouth (also forms anterior pituitary)
develop above sella turcica (suprasellar - anywhere from pituitary gland to base of 3rd ventricle)
benign, symptoms from compression
Pt is 11yo M taken to their GP by their parent due to concerns of behavioral changes and problems with vision. An MRI is performed which shows a calcified and cystic tumor in the sella turcica. A biopsy is taken, which shows epithelial cells. What is the dx?
craniopharyngioma: derived from remnants of Rathke’s pouch (invagination of ectoderm, also forms anterior pituitary)
develop above sella turcica (suprasellar - anywhere from pituitary gland to base of 3rd ventricle)
benign, symptoms from compression (optic chiasm, frontal lobe, pituitary)
what symptoms are caused by mass effect of pineal tumors?
rare germ cell or parenchymal tumors, occur in children
cause compression of pretectal area of midbrain —> Parinaud syndrome: paralysis of upward gazes + pseudo-Argyll-Robertson pupil (react to accommodation but not light)
can also compress cerebral aqueduct —> hydrocephalus, papilledema
how does Parinaud syndrome present? what type of tumor can cause it?
Parinaud syndrome: paralysis of upward gazes + pseudo-Argyll-Robertson pupil (react to accommodation but not light)
can be caused by pineal tumors: rare germ cell or parenchymal tumors, occur in children, cause compression of pretectal area of midbrain
Pt is 8yo F presenting to neurologist after GP determined they have hydrocephalus and papilledema. On neuro examination, you find paralysis of upward gaze. Further, pupils respond to accommodation, but not light. What area of the brain is compressed by this tumor? Of what cells is this tumor made?
pineal tumor: rare germ cell or parenchymal tumors, occur in children
cause compression of pretectal area of midbrain —> Parinaud syndrome: paralysis of upward gazes + pseudo-Argyll-Robertson pupil (react to accommodation but not light)
can also compress cerebral aqueduct —> hydrocephalus, papilledema
describe the classic radiologic picture of a glioblastoma. what genetics are often implicated?
radiologic picture shows cerebral rim/ring-enhancing lesion
genetics frequently involve amplification of receptor tyrosine kinases (EGFR, MET, PDGFR)
what genetics are implicated in oligodendrogliomas?
co-deletion of 1p and 19q
what features would be present in a biopsy of oligodendroglioma? (3)
- “fried egg” nuclei
- “chickenwire” vasculature
- calcifications
what does histology of schwannomas show? (2)
- hyper or hypo cellular areas (Antoni areas: appear hyper/hypo pigmented)
- Verocay bodies: nuclei lining up together
what do medulloblastomas stain positive for?
synaptophysin positive
Malignant tumor of primitive neurons that primarily affects young children, occur in cerebellar/posterior fossa
where do brain metastasis usually develop?
most commonly from lung, breast, renal, melanoma, colon
typically develop at grey-white junction as well-circumscribed masses
glandular characteristic of metastasis matches histology in primary site
what does histology of craniopharyngiomas show?
Tumor arising in sellar region (similar to Pit
adenoma)
• Derived from remnants
of Rathke pouch
• Histological features: Squamous epithelium
what is the role of the neurofibromin (FN1) gene that is mutated in neurofibromatosis 1?
NF1 negatively regulates Ras oncoprotein
mutation —> Tumors:
• Neurofibromas
• Café au lait spots
• Optic gliomas
• Pheochromocytomas
• Malignant peripheral nerve sheath tumor
what is the role of the NF2 gene which is mutated in neurofibromatosis 2?
NF2 gene (ch. 22) encodes Merlin: links pro-proliferation signals at membrane with underlying actin cytoskeleton
CNS Tumors:
• Bilateral vestibular schwannoma is hallmark of the syndrome
• Meningioma
• Ependymoma
