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Neuro Neoplasms

Neurology

QuestionAnswer
Majority of malignant gliomas are: grade IV tumors (GBM or gliosarcomas)
Gliomas include: astro; oligo; ependymoma; glial cells: support
Grade 1 Glioma = Pilocytic Astrocytomas
Grade 2 Glioma = Astrocytomas, Oligodendrogliomas
Grade 3 Glioma = Anaplastic Astrocytomas, Anaplastic Oligodendrogliomas
Grade 4 Glioma = GBM; Gliosarcoma
Diffuse astrocytomas include: Low-grade infiltrative Astro (WHO II); anaplastic astro (WHO III); Glioblastoma (WHO IV)
Circumscribed astrocytomas include: Pilocytic Astro (WHO grade I); Pleomorphic xanthoastrocytoma (PXA); Subependymal giant cell Astro (SEGA)
Well-differentiated Astro: 10-15% of Astros; 35 yo; 5-10 yr surviv; cerebral hemi / cortex; slow growing; often undergo malig progression to AA or GBM
High-grade gliomas (III/IV): invade via white matter tracts, cross via corpus callosum;
Primary characteristic of a grade IV glioma = necrosis with vascular proliferation
Anaplastic astro High-Grade; 1/3 of Astros; 45 yo; 3 yr surviv; usu in cerebral white matter; relatively fast-growing; Frequent progression to GBM
GBM 50% of Astros; >60 yo; rare <30; 1 yr surviv; usu in cerebral hemi white matter; spreads rapidly & diffusely; Ring of tissue around necrotic core; Highly vascular
Oligodendroglioma Usu presents at low-grade (II) stage; young / middle-aged; grade II >10 yr surviv; Fried-egg cells
Most common primary brain neoplasm = GBM
Untreated GBMs: growth double in size in 14 days
Brain tumor: genl clin presentation HA; seizures; Cognitive dysfn; Focal neuro deficits; N/V; Sx endocrine dysfn; Visual sx; Sx from plateau waves (ICP changes)
Headache in brain tumor 20% of pts present w/HA (more w/ post fossa tumor); usu d/t inc ICP; Progressive increase in HA freq & severity; Classic HA on waking or HA that wakens one from sleep
Seizure in brain tumor 35% of pts present with this; often the sx that precedes dx; focal or secondarily generalized
Probably most common problem in pts w/brain tumors = cognitive dysfn
Cognitive dysfn in brain tumor: Frontal personality; Memory problem; Depression
Cognitive dysfn: Left hemispheric tumors: language dysfunction
Cognitive dysfn: Right hemispheric tumors: problems with visual perception & scanning
Brain tumor: Focal neurologic deficits Hemiplegia; Hemiparesis; Ataxia; Nystagmus; Can mimic stroke
N/V more common in: post fossa tumors
Endocrine sx: hypothyroid; dec libido
Visual symptoms include: Contralateral flashing lights; Visual field loss; Diplopia
Symptoms from plateau waves Transitory episodes of altered consciousness & visual disturbances
Brain tumor: eval & dx H&P; CT +/- MRI; EEG; LP; PET
Factors of better prognosis: Young; High KPS; Lower pathological grade
Less significant prognostic predictors: Long duration of sx; Absence of mental status changes; Cerebellar location; Small preop tumor size; Completeness of surgical resection
BCNU (chemo) adverse effects fatigue, low blood counts, pulmo fibrosis
Tx for high grade gliomas Primary tx = surg resection, RTx, CTx; most sig prognostic factors: extent of surg resection, age, & performance status
First treatment modality for high grade glioma Surgery
Goals of surgery: Confirm patho dx; improve sx rapidly; reduce number of ca cells requiring tx (removes hypoxic core of the tumor; relatively resistant to radiation & inaccessible to CTx)
Std tx for WHO III &IV gliomas: Radiation tx (role of RT in WHO II gliomas is controversial)
RTx Modalities: Whole brain (ltd use now in gliomas); Focal RTx: Conventional high-dose (59.4 Gy x 42 days; 2 cm border around tumor area) or IMRT; Stereotactic (gamma-knife)
RTx injury Acute (fever, neuro def) or delayed encephalopathy (edema, MRI enhance); tx w/c’steroids; focal cerebral necrosis (mos-yrs): tx w/steroids & hyperbaric
Most frequent delayed effect of RTx Diffuse cerebral injury; cortical dysfunction, progressive dementia, gait disturbance; WM destruction; MRI: lg ventricles, wide sulci, basal ganglia calcifn, hyperintense T2
Std of care: high grade gliomas Resection; RTx & 42 days temozolomide; then (if stable dz), adjuvant CTx
Stupp study result: temozolomide added to RTx improves survival
CTx MOA & AE targets DNA replication; AE: Myelosuppression; N/V; Fatigue; Constipation or diarrhea; DI
CTx: passage across BB depends on: Molecule Size; Lipid solubility; Ionization state
CTx DI: Dex (closing BBB); phenobarbital (dec nitrosourea efficacy); anti-epileptics (affect CTx metabm)
Low grad glioma: Tx Bx (confirm dx & r/o high grade); seizures only: defer tx til progresses; if progressive sx: resect +/- CTx (+ RTx only if refractory)
VEGF higher the VEGF, worse the prognosis; anti-VEGF Ab’s effective in xenografts
Tumor blood vessels dept on growth factor; leaky & fragile; less supported by pericytes; disorganized, twisted, variable pressure; inconsistent nutrient delivery to tumor tissue
Second leading COD in brain tumor pts: thromboembolic complications
Meningioma slow growing, benign; attached to dura mater; 13-26% of primary IC tumors; RF: prior radiation; often Asx; visual complications; DDx: neurofibromatosis; Firstline tx: Surgery
Ependymoma: worse prognosis: <3 yo
Ependymomas within brain: locations Infratentorial > supratentorial (2x)
Ependymoma Usu slow growing; kids & YA; young adults; 4% all BT; from walls of ventricles or spinal canal (obstructive hydrocephalus); 90% are in brain, 10% in spinal cord (often adults)
Medulloblastoma Malig, invasive embryonal cerebellar tumor; kids & YA; 70-80% 5-yr survival; Most arise in vermis (4th ventricle involvement)
Medulloblastoma: metastases Metastasize via CSF pathways (Drop Mets); Rare mets to bone, lymph nodes, other extracranial sites
Medulloblastoma: Sig LT toxicities of tx: Cognitive decline; Psychomotor / growth retardation; Hormonal deficits
Primary CNS lymphomas Usu present deep in brain parenchyma; usu multifocal (diffuse lg cell B-cell lymphoma); Survival w/o tx <1 yr; Steroid tx & Methotrexate CTx
Primary CNS lymphomas: incidence increased in: Immunodef pts; AIDS; Organ TP; older pt
Std of care: Grade I Surgery +/- RT
Std of care: Grade II Surgery; Observe; If progression: CTx
Std of care: Grade III Surgery; RT with temozolomide; 12 cycles of temozolomide
Std of care: Grade IV Surgery; RT with temozolomide; 52 weeks rotational CTx
HA worse in AM w/ focal neuro deficits Brain Tumor (MC is glioma)
Majority of malignant gliomas are: grade IV tumors (GBM or gliosarcomas)
Gliomas include: Astro (Grade 1); oligo (2); ependymoma; glial cells
Grade 3 Glioma = Anaplastic Astrocytomas, Anaplastic Oligodendrogliomas
Grade 4 Glioma = GBM; Gliosarcoma
Diffuse astrocytomas include: Low-grade infiltrative Astro (WHO II); anaplastic astro (WHO III); Glioblastoma (WHO IV)
Circumscribed astrocytomas include: Pilocytic Astro (WHO grade I); Pleomorphic xanthoastrocytoma (PXA); Subependymal giant cell Astro (SEGA)
Well-differentiated Astro: 10-15% of Astros; 35 yo; 5-10 yr surviv; cerebral hemi / cortex; slow growing; often undergo malig progression to AA or GBM
characteristic of high-grade gliomas (III/IV): Invasion via white matter tracts, cross via corpus callosum
Primary characteristic of a grade IV glioma = necrosis with vascular proliferation
Anaplastic astro High-Grade; 1/3 of Astros; 45 yo; 3 yr surviv; usu in cerebral white matter; relatively fast-growing; Frequent progression to GBM
GBM usu in cerebral hemi white matter; spreads rapidly & diffusely; Ring of tissue around necrotic core; Highly vascular
Oligodendroglioma Usu presents at low-grade (II) stage; young / middle-aged; grade II >10 yr surviv; Fried-egg cells
Most common primary brain neoplasm = GBM
Untreated GBMs: growth double in size in 14 days
Brain tumor: genl clinical presentation HA; seizures; Cognitive dysfn; Focal neuro deficits; N/V; Sx endocrine dysfn; Visual sx; Sx from plateau waves (ICP changes)
Headache in brain tumor 20% of pts present w/HA (more w/ post fossa tumor); usu d/t inc ICP; Progressive increase in HA freq & severity; Classic HA on waking or HA that wakens one from sleep
Seizure in brain tumor 35% of pts present with this; often the sx that precedes dx; focal or secondarily generalized
Probably most common problem in pts w/brain tumors = cognitive dysfn (Frontal personality; Memory problem; Depression)
Cognitive dysfn: Left hemispheric tumors: language dysfunction
Cognitive dysfn: Right hemispheric tumors: problems with visual perception & scanning
Brain tumor: Focal neurologic deficits Hemiplegia; Hemiparesis; Ataxia; Nystagmus; Can mimic stroke
Brain tumor: N/V more common in: posterior fossa tumors
Endocrine sxs in brain tumor: hypothyroid; dec libido
Visual symptoms in brain tumor include: Contralateral flashing lights; Visual field loss; Diplopia
Low grade glioma: Tx Bx (confirm dx & r/o high grade); seizures only: defer tx til progresses; if progressive sx: resect +/- CTx (+ RTx only if refractory)
Second leading COD in brain tumor pts: thromboembolic complications
Ependymoma: worse prognosis: <3 yo
Ependymomas within brain: locations Infratentorial > supratentorial (2:1)
Ependymoma Usu slow growing; kids & YA; young adults; 4% all BT; from walls of ventricles or spinal canal (obstructive hydrocephalus); 90% are in brain, 10% in spinal cord (often adults)
Characteristics of Medulloblastoma Malig, invasive embryonal cerebellar tumor; kids & YA; 70-80% 5-yr survival; Most arise in vermis (4th ventricle involvement)
Medulloblastoma: metastases Metastasize via CSF pathways (Drop Mets); Rare mets to bone, lymph nodes, other extracranial sites
Characteristics of Primary CNS lymphomas Usu present deep in brain parenchyma; usu multifocal (diffuse lg cell B-cell lymphoma); Survival w/o tx <1 yr; Steroid tx & Methotrexate CTx
most common spinal tumor: ependymomas; 10% of spinal tumors are intramedullary
Brain tumor: frontal lobe: S/S: cog decline; contralat grasp reflex; expressive aphasia
Brain tumor: temporal lobe: S/S: sz, olfactory hallucination, depersonalization, vis field def, auditory illusions
Brain tumor: parietal lobe: S/S: contralat sensory def; cortical sens loss (stereognosis); inattention
Brain tumor: occipital lobe: S/S: crossed homonymous hemianopia / partial field defect; visual agnosia
Brain tumor: brain stem/cerebellar: S/S: CN palsies, ataxia, incoordination, nystagmus, pyramidal & sensory deficit
Astrocytoma Glial tumors: 40-50% of CNS Neoplasms
Astrocytoma: Grade IV GBM (45-55 yo); necrosis/ hemorrhage, edema, ring enhancement
Meningioma 50-60 yo; may increase in PG; various grades (90% benign)
Brain neoplasm: mets 1/3 of all intracranial neoplasms; lung, breast, melanoma, colon, lymphoma, prostate
Brain neoplasm: most common site for kids (unlike adults) posterior fossa (medulloblastoma)
brain tumors seldom__ metastasize outside the CNS
__ spread the soonest with metastatic brain tumors lung and renal cancer cells
mets to the brain in men generally come from __ lung, colon, and renal cancers
mets to the brain in women generally come from __ breast, lung, and melanoma
a tumor in the supratentorial region may result in which pathologic disorder epilepsy
which cancers metastasize to the brain the fastest? lung and renal
in which area of the brain do most mets tumors arise? cerebrum 80%
tethered cord abnormally low conus medullaris
Central cord syndrome S/S motor deficit in UE > LE; Varying degrees of sensory loss (pain/temp); most common of the incomplete spinal cord lesions (better prognosis)
Ligamentum flavum buckles into spinal cord => contusion to central regions of spinal cord = Central cord syndrome
Anterior cord syndrome S/S Paraplegia and dissociated sensory loss with loss of pain and temperature sensation; 2/2 infarction of the cord in the region supplied by the anterior spinal artery
Anterior cord syndrome: what fn is preserved: Posterior column (position, vibration, deep pressure) preserved
Brown-Sequard syndrome: cause Hemisection of the cord; From penetrating injuries; Rare
Brown-Sequard syndrome S/S Ipsilateral motor loss w/loss of vibration, pressure, proprioception; contralateral loss of pinprick, pain, temperature sensations
Benign nonencapsulated tumor of nerve fibers = Neurofibroma
Benign tumor of nerve sheath = Schwannoma
Created by: Abarnard