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65 Cards in this Set

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Meningioma- hx
-Peak incidence 6-7th decades
-Female predominance
-Most common location is at falx or base of skull
-May present with seizures, headaches, and focal neurologic deficits
-Early morning headache (d/t increased ICP)
-Possible loss of taste/smell (olfactory meningioma)
Meningioma- phys
-Focal neurologic deficits
-Cranial neuropathies may arise if tumor is from base of skull
-Paraparesis if tumor is in falx cerebri – causes bilateral compression of leg areas of motor cortex
Meningioma- dx tests
-CT: smooth, lobulated, isodense tumor that is adjacent to dura and enhances uniformly w/ contrast; possible multiple small calcifications
-MRI: less characteristic; T1- isointense or hypointense; T2- isointense or hyperintense; possible edema in adjacent brain
Meningioma- tx
-Many are slow growing, small and asymptomatic and thus can be followed w/ periodic imaging
-When tumor becomes symptomatic, partial or total removal is indicated
-Gammaknife tx
-Radiotherapy and chemo show no benefit
Meningioma- prognosis
-Total tumor removal has a 10 year recurrence rate of10%
-Partial removal has a 10 yr recurrence rate of major sx in 40%
-90% are benign, 10% are aggressive
Meningioma- pathophys
-60% of sporadic meningiomas have NF2 mutation
-Pathology: whirls of cells w/ pseudonuclear inclusions; psammoma bodies
-WHO Grade I-III
-Slow-growing benign tumors attached to dura composed of neoplastic arachnoid cells
-20% recurrence in 20 yrs
-Atypical meningioma: increase in cellularity & growth, increased mitoses, prominent nucleoli
What tumor is shown here
What tumor is shown here
Meningioma
Ependymoma- dif
-CNS mass
-Meningitis
-Encephalitis
-ADEM
-Abscess
Ependymoma- hx
-Typically occurs in first 2 decades of life, 30% in children <3 yo
-Headache (60%), usually worse in am
-N/V secondary to increased ICP (80%)
-Behavioral changes: lethargy, irritability, decreased social interaction, loss of appetite (50%)
-Ataxia, dizziness (30%)
Ependymoma- phys
-Papilledema (60%)
-Ataxia (45%)
-Nystagmus with or without gaze palsy (40%)
-Apraxia or hemiparesis (20%)
-Increase in head circumf in children <2 yo (10%)
Ependymoma- dx tests
-MRI: Evidence of calcification, necrosis, cystic change; 2/3 are located in posterior fossa, 90% in 4th ventricle

-LP: contraindicated!
Ependymoma- tx
-Preoperative steroids to limit edema and alleviate sx
-Surgical resction: most effective therapy
-Postoperative radiation therapy improves survival
Ependymoma- prog
-Gross total resection: progression-free survival rates of 70-80% after 5 years, compared to 35% for incomplete resection
-The younger the pt, the worse the prognosis
-May lead to hydrocephalus  increased ICP
-Usually do not proliferate rapidly, are not invasive, and do not metastasize
Ependymoma- pathophys
-Summary: neoplasms of ependymal cells that occur throughout the entire neuraxis in association with the lining of the cerebral ventricles and central canal of the spinal cord
-Pathology: well circumscribed lesion; perivascular pseudorosette formation, cellular atypia
-WHO Grade II
-Slow-growing tumor originating from the wall of cerebral ventricles (ependyma)
What type of tumor is this
Ependymoma
Astrocytoma - pilocytic- dif
-Ependymoma
-Headache
-Hydrocephalus
-Head injury
-MS
-Oligodendro
glioma
-Epidural abscess
-Subdural empyema
-Cerebral abscess
-Hamartoma
-AVM
Astrocytoma - pilocytic- hx/phys
-Presents in 1st 2 decades
-Early morning headache (d/t increased ICP)
-Can cause sxs by perturbing cerebral function (seizures), elevating ICP by mass effect or obstructing CSF pathways ( hydrocephalus), or causing neurologic abnormalities (paralysis, sensory deficits, aberrant behavior, HA)
Astrocytoma - pilocytic- dx tests
-MRI: Contrast enhancing because there are abnormal leaky, proliferating blood vessels that allow for enhancem.
Astrocytoma - pilocytic- tx
-Surgery is 1st line
-Phenytoin for sz
Astrocytoma - pilocytic prognosis
-Cured if undergo gross total resection
-Unresected - neurologic deficit may occur over a period of years
-Median survival duration is 7.5 yrs
-Age at onset, type of astrocytoma and type of therapy influence outcomes
-May dedifferentiate into a higher grade lesion
Astrocytoma - pilocytic- pathophys
-Low grade, slow-growing
-Occurs throughout CNS, cerebellum, optic nerve, hypothalamus
-Pathology: biphasic growth pattern, bipolar cells w/ long processes; Rosenthal fibers (degenerative processes); rare mitoses
-WHO Grade I: well circumscribed, often cystic
-Most common glioma (and solid tumor) in children
What type of tumor
Astrocytoma - pilocytic
What tumor was this person treated for?
astrocytoma
Astrocytoma – diffusely infiltrating- hx
-60% occur btwn 20-45 yo
-Can occur anywhere, most commonly cerebrum
Astrocytoma – diffusely infiltrating- tx
-Can’t be surgically resected
Astrocytoma – diffusely infiltrating- pathophys
-60% occur btwn 20-45 yo
-Can occur anywhere, most commonly cerebrum
-WHO Grade II-IV: progression to malignancy from astrocyte w/ genetic mutation  astrocytoma  anaplastic astrocytoma  glioblastoma
Glioblastoma- hx
-Tends to occur in older adults (mean age 55 yrs)
-Most common primary brain tumor in adults
-Headaches (50%)
-Altered mental status (50%)
-Seizures (20%) – focal or secondarily generalized tonic-clonic
-Sx progress rapidly in the absence of tx
Glioblastoma- phys
-Hemiparesis (40%)
-Aphasia (15%)
-Visual field loss (5%)
-Papilledema
Glioblastoma- dx tests
-MRI w/ gad: central low signal intensity on T1 outlined by high intensity ring-enhancement; surrounding high intensity areas are hypointense signals that represent cerebral edema and tumor infiltration
-CT: variably hypodense or isodense lesions surrounded by hypodense cerebral edema
-EEG: focal or extensive slowing (delta waves) in tumor region
Glioblastoma- tx
-Management aims at slightly prolonging survival and controlling sx
-Corticosteroids to reduce vasogenic edema & prolong survival 1-3 mos
-Neurologic side effects of high dose steroids: psychosis, hyperactivity, irritability, insomnia, myopathy
-Surgical removal (debulking) improves length of survival and improves neurologic sx by reducing ICP
-Radiotherapy after surg slightly improves survival
-Anticonvulsants to control sz
-Palliative care
Glioblastoma- prog
-Survival less than 18 mos., always fatal
-Survival <6 mos if untreated
-Better prognosis in young pts
Glioblastoma- pathophys
-WHO Grade IV: highest grade astrocytic neoplasm

-Can cause uncal herniation

-Primary glioblastoma: de novo; older ages; genetic mutations (EGFR – epidermal growth factor)

-Secondary glioblastoma: progression over time from diffuse astrocytoma; younger ages; mutation of p55

-Pathology: microvascular proliferation, pseudopallisading necrosis, multinucleated giant cells
Type of tumor?
Glioblastoma
Oligodendroglioma- hx
-Incidence peaks in 5th-6th decades; M:F is 1:1
-Slow-growing neoplasm
-Frontal lobe 50-65% of cases
-Early morning headache (d/t increased ICP)
Oligodendroglioma- tx
-Loss of heterozygosity of 1p & 19q responds to chemo treatment and increases life expectancy

- not generally surgically resectable
- slow growing so watchful waiting.
Oligodendroglioma- pathology
-Pathology: -Well circumscribed w/ calcifications; diffusely infiltrates white matter & cortex; proliferating, leaky blood vessels, atypical, enlarged nuclei; Fried egg, halo artifact; Chicken wire vasculature
Type of tumor?
Oligodendroglioma
Medulloblastoma- epi
-Peak at 7 yrs of age
Medulloblastoma- phys
-Can grow along 4th ventricle  cause hydrocephalus
Medulloblastoma- prog
-5 yr survival 50-70% w/ poor developmental outcomes
Medulloblastoma- patho
-WHO Grade IV
-Malignant, invasive embryonal tumor in cerebellum; in posterior cranial fossa
Brain metastasis- hx
-25% of cancer pts develop brain mets
-Impaired cognition (60%)
-Headache (60%)
-Aphasia (205)
-Seizures (20%) – focal, motor
-Stupor or coma (5%)
-Clinical sx occur via displacement of brain tissue from rapidly growing tumor and surrounding vasogenic edema  vessel compression  ischemia
Brain metastasis- phy
-Hemiparesis (60%)
-Hemisensory loss (20%)
-Papilledema (20%)
-Visual field cut (10%)
Brain metastasis- dx test
-MRI w/ gad: best diagnostic test; T1 w/ gad – heterogeneous or ring-enhancing lesion usually w/ surrounding edema; shifting of brain structures d/t mass effect
-Majority of mets are supratentorial – 80% loc in cerebral hemispheres
Brain metastasis- tx
-Surgical removal of met only occasionally is helpful in markedly prolongling life
-Dexamethasone reduces edema and dramatically improves sx for 1-2 mos
-Radiation therapy adds a few more mos of survival
Brain metastasis- prognosis
-Median survival w/o tx is 1-2 mos from discovery of brain tumor
-W/ corticosteroids, survival extends 2-4 mos
-Median survival w/ steroids + radiotherapy is 3-6 mos
-Increased ICP may trigger herniation
Brain metastasis- pathophys
-Summary: Neoplasms that originate in tissues outside the brain and spread secondarily to involve the brain
-80% are supratentorial, 15% cerebellar, 5% in brainstem or spinal cord
-25% discovered before or at the time of dx of primary tumor
-Most common sources: lung (44%), breast, GI, GU, renal cell, melanoma, leukemia
Neurofibroma-tosis 1- dif
-CNS neoplasm
-Spinal cord hemorrhage, infarction, abscess
-NF2
Neurofibroma-tosis 1- hx
-First degree relative has NF1
Neurofibroma-tosis 1- phy
-Café-au-lait spots
-Axillary or inguinal freckles
-Multiple neurofibromas
-Iris hamartomas / Lisch nodules
-Optic nerve glioma
-Sphenoid dysplasia
Neurofibroma-tosis 1- dx tests
-Sequencing of NF1 gene
-Plain films to detect bony abnormalities
-Baseline CT
-Annual eye exam
Neurofibroma-tosis 1- tx
-Surgical removal of neurofibromas that press on vital structures, obstruct vision, or grow rapidly
Neurofibroma-tosis 1- progn
-Most live long and healthy lives,
-Life expectancy may be reduced by as much as 15 years
-May increase HTN, sequelae of spinal cord lesions, and malignancy
Neurofibroma-tosis 1- pathophys
-Autosomal dominant mutation in chromosome 17q12 (neurofibromin gene – tumor suppressor)
-Assoc. w/ pliocytic astrocytomas, optic nerve gliomas, malignant peripheral nerve sheath tumors (MPNST), osseous lesions, juvenile CML, pheochromocytoma, peripheral neuropathy, ADHD
-Can become malignant, can grow on spinal cord
What type of disease does this person have
Neurofibroma-tosis 1
Neurofibroma-tosis 2-dif
-NF1
-Ependymoma
-Meningioma
-Juvenile cararacts
Neurofibroma-tosis 2- hx
-Typical onset in early adulthood
-First degree relative affected
-Tinnitus
-Gradual hearing loss
-Vestibular / balance dysfunction
Neurofibroma-tosis 2- phys
-Bilateral acoustic neuromas (Schwannomas)
-Sensory motor polyneuropathy
-Optic nerve sheath meningiomas
-CN palsies d/t compression from expanding schwannoma
-Subcutaneous neurofibromas
Neurofibroma-tosis 2- dx test
-MRI: Bilateral eighth nerve masses
-Hearing evaluations
Neurofibroma-tosis 2- prognosis
-Prognosis of NF2 depends on age of onset of symptoms, degree of hearing deficit, and number and location of various tumors
-Typically decreased lifespan
Neurofibroma-tosis 2- pathophys
-Summary: AD multisystem genetic disorder associated with bilateral vestibular schwannomas, spinal cord schwannomas, meningiomas, gliomas, and juvenile cataracts with a paucity of cutaneous features
-Mutation in chromosome 22q12 (Merlin/schwannomin gene product, tumor suppressor)
-Tumor grows along the nerve
What type of tumor is seen here?
NFT2
Paraneoplastic neurological syndrome- phys
-Autonomic neuropathy: orthostatic hypotension, impaired pupillary light response, abnormal valsalva response, impotence
Paraneoplastic neurological syndrome- pathophys
- Small cell lung neoplasm  Ca2+ ion channel Lambert-Eaton
-Thymoma  ACh  MG
-Caused by or associated w/ a malignancy – antibodies against tumor & CNS
-Paraneoplastic antibody (IgG) has a neural antigen target  membrane dysfunction or focal damage to nerve or muscle