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Neuro Neoplasms
Neurology
Question | Answer |
---|---|
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 |