Frontal – Broca’s aphasia (left); disinhibition; indifference and lack of initiative; deficits in concentration; recent memory impairment; abulia; contralateral hemiplegiaParietal – Wernicke’s aphasia (left); cutaneous sensory perception; decreased sense of position & orientation of limbs in space; difficulty with calculationsOccipital – contralateral homonymous hemianopia; cortical blindnessTemporal – contralateral homonymous superior quadrantanopia “pie in the sky” defect (lower fibers of the optic radiations); auditory and vestibular receptive areas produce defecits in hearing, balance and sound localization; limbic lobe affects emotion and memoryCerebellum – nystagmus; ataxia; dysarthria; ipsilateral flaccidity
Most common primary intracranial tumor
Observation - 32% of incidentally discovered meningiomas do not grow over 3 years follow upGamma Knife – tumor must be less than 3 cmSurgical indications – evidence of growth on serial imaging or symptoms referable to the lesion
Olfactory groove meningioma
Olfactory groove meningioma
Cavernous, convexity and falcinemeningioma
95% are unilateralBilateral VS iapathognomonic of neurofibromatosis type 2
From micro to macroadenoma
Pituitary apoplexy – infarction and hemorrhage into pituitary gland
VHL – genetic multisystemneoplastic disorder withhemangioblastomas of cerebellum, retina, brainstem and spinal cord, renal cell carcinoma and pheochromocytomas
Usually cystic with enhancing mural nodule
Symptoms are those of any posterior fossa mass
94% enhance with contrast; frequently have a cystic component
Controversial. No well-designed study has shown that any approach is clearly superior. These tumors are slow growing and until progression on imaging or malignant degeneration is documented, it may be no worse to not treat the patient. Consider treatment for:Extremely young patients or patients > 50 years old, large tumors that enhance, symptomatic patients, evidence of progression on imaging studes.
Ananaplastic astrocytoma will have less necrosis in the center (compared to a grade 4 or glioblastoma) but still look more abnormal (more enhancement) than a low grade (grade 1 or grade 2) .
Grade IV - GBM
Butterfly glioma (A) and infiltrative tracts (c)
Since these tumors cannot be cured with surgery, the goal is to reduce mass effect while prolonging QUALITY survival.Karnofsky score – in general with infiltrating tumors, the neurologic condition on steroids is as good as it is going to get and surgery rarely improves this.
Since these tumors cannot be cured with surgery, the goal is to reduce mass effect while prolonging QUALITY survival.
sagittal view of medulloblastoma, T1WI with gadolinium, showing multiple avidly enhancing drop metastases from the patient's posterior fourth ventricular medulloblastoma. 10 – 35% of cases have seeded the cranio-spinal axis at the time of diagnosis.
It is better to leave a small residual on the brain stem (these patients do fairly well) than to chase every last remnant into the brain stem leading to neurologic deficits.5 year survival is 50 – 85%.
MRI – lesions often occur at gray white junction, are ring-enhancing and show profound white matter edema.The cerebellum is a common site of mets (16%). It is the most common p-fossa tumor in adults, thus a solitary lesion in the p-fossa of an adult is considered a met until proven otherwise.
Solitary brain lesions in a patient with hx of cancer require biopsy since 11% will not be mets
Corticospinal tracts – skilled movementExtrapyramidal tracts – muscle toneDorsal column – joint position, fine touch and vibrationSpinocerebellar tracts – stretch receptors and whole limb position senseAnterolateral system – light touch; pain and temperature
MRI shows low signal on T1, high signal on T2 and enhancement with contrast
Sagittal and coronal contrast-enhanced T1-weighted MRIs shows intensely enhancing mass compressing the cervical cord
1. Enhanced T1-weighted sagittal magnetic resonance image shows the round shape tumor compressing the spinal cord posteriorly. 2. The homogeneously enhanced dumbbell-shaped mass located on the right side of spinal cord extending into the C1-C2 intervertebral foramen.
T1-weighted gadolinium-enhanced image shows a myxopapillaryependymoma in the lumbar region; it is homogeneously enhancing.Axial T1-weighted image confirms the central location of the tumor.
Temporal progression (4 stages): pain only, Brown-Sequard syndrome, incomplete transectional dysfunction, complete transectional dysfunction.Surgery – radical removal rarely possible (cleavage plane unusual even with microscope)
Sagittal T2-weighted magnetic resonance image of the cervicothoracic spinal cord. This image demonstrates an intramedullary lesion in the cervicothoracic spinal cord and the associated cord expansion. Histology revealed a low-grade astrocytoma.Axial T2-weighted magnetic resonance image of the spinal cord. This image demonstrates hyperintensity in the spinal cord, which is consistent with the presence of a tumor. The poorly defined margins of this tumor reflect the infiltrative nature of low-grade astrocytomas.
Diagnostic criteria (Must have 2 of):Motor or sensory symptoms occurring bilaterallyDiplopia (from ischemia of upper brainstem – midbrain)Dysarthria (for ischemia of lower brainstem)Homonymous hemianopsia – ischemia of occipital cortex; binocular c/w amaurosisfugax which is monocular
Meningiomas:<br />Account for 14 – 19% of primary brain tumors <br />Incidence peaks at 45 years of age<br />Female to Male ratio 2:1<br />Arise from arachnoid cap cells<br />Extra-axial (dural based)<br />
Meningiomas:<br />Usually benign (less than 5% are malignant) and slow growing<br />Location – most commonly located along the falx, convexity or sphenoid bone<br />Symptoms: often presents with seizure, headache <br />Studies: MRI with gadolinium shows attachment on dura often with a dural tail and typically enhances densely<br />
Meningiomas:<br />Treatment Options:<br /><ul><li>Observation with serial MRI’s for evidence of growth
Acoustic Neuromas<br />Vestibular Schwannoma is currently preferred since most arise from the superior division of vestibular nerve<br />Make up 8-10% of primary brain tumors<br />Incidence: usually after age 30<br />Pathology: AntoniA (narrow elongated bipolar cells) and Antoni B (loose reticulated)<br />
Hemangioblastomas <br />Most common primary intra-axial tumor in the adult posterior fossa<br />May also occur in spinal cord<br />Rare supratentorially<br />20% occur as part of vonHippel-Lindau disease<br />
Hemangioblastomas<br />Symptoms: those of any posterior fossa mass (HA, N/V, dizziness, ataxia, dysarthria, nystagmus) <br />Evaluation: MRI with contrast of the entire neuraxis<br />Treatment: Surgery is curative in sporadic cases. Pre-operative embolization to reduce vascularity<br />
GLIOMAS Arise from Glial Cells<br />Astrocytomas<br />Astocytomas fall on a gradient that ranges from benign to malignant<br />Benign<br />Malignant<br />Glioblastomamultiforme<br />Low Grade PilocyticAstocytomas<br />Diffuse Low Grade Astrocytomas<br />
Neuropathological Grading<br />WHO Classification:<br /><ul><li>Grade I – pilocyticastrocytoma
Grade IV – glioblastoma (GBM)</li></li></ul><li>PilocyticAstrocytoma<br /> Can arise throughout the neuroaxis (cerebellum, optic nerve, hypothalamus)<br />Age at presentation < 20 years in 75%<br />Often cystic and half have a mural nodule<br />Prognosis: 94% 10 year survival<br />
Low-Grade Astrocytomas<br />Three cell types: fibrillary, gemistocytic and protoplasmic<br />Tend to occur in children and young adult<br /> Most present with seizures<br />Ultimate behavior of these tumors is usually NOT benign. The major cause of morbidity is differentiation to a more malignant grade. <br />
Low-Grade Astrocytomas<br />Radiographic Appearance: <br /><ul><li>CT – low density
MRI – abnormal signal on T2; usually without enhancement
Predilection for temporal, posterior frontal and anterior parietal lobes </li></li></ul><li>
Combination of radiation and chemotherapy </li></li></ul><li>Low-Grade Astrocytomas<br />Prognosis for survival: 7 – 10 years<br />Prognosis is worse if:<br /><ul><li>Patient is > 50 years old at time of diagnosis
Representing 1.44 % of all cancersdiagnosed in 2010</li></ul>Central Brain Tumor Registry of the United States (CBTRUS) 2004-2007<br />
Mortality<br />An estimated 13,140 deaths in 2010will be attributable to primary malignantbrain tumors<br />Central Brain Tumor Registry of the United States (CBTRUS) 2004-2007.<br />
High-Grade Malignant Gliomas Account for 77.5% of all Gliomas<br />n=16,780.<br />Central Brain Tumor Registry of the United States (CBTRUS) 2002-2003.<br />Statistical Report 1995-1999.<br />
Anaplastic Astrocytoma and GlioblastomaMultiforme<br />Grade III and Grade IV<br />Mean age for AA is 46 years old<br />Mean age for GBM is 56 years old<br />Male:female=3:2<br />GBM accounts for 25% of all adult brain tumors (50-55% of all gliomas)<br />
Multicentricgliomas</li></li></ul><li>AA and GBM<br />Prognostic factors - age, duration of symptoms, neurological status, extent of resection<br />AA – median survival is 36 months<br />GBM – median survival is 10 months (without treatment 95% die within 3 months)<br />
Drop mets may produce back pain, urinary retention or leg weakness</li></li></ul><li>Medulloblastoma<br />Evaluation:<br /><ul><li>MRI with gadolinium shows most lesions in the midline region of the 4th ventricle.
Brain Stem Glioma<br />Not a homogeneous group – some are more malignant<br />Lower grade tumors tend to occur in the upper brain stem<br />Higher grade tumors tend to occur in the lower brainstem/medulla<br />Most are malignant, have poor prognosis and are not surgical candidates<br />
Pineal Region Tumors:<br />Tumors in this region are more common in children (3 – 8% of pediatric tumors)<br />Over 17 tumor types occur in this area<br />Most common is germinoma followed by astrocytoma, teratoma and pineoblastoma<br />Many metastasize easily through the CSF and therefore MRI of neuraxis is required<br />
Pineal Region Tumors:<br />Presentation – hydrocephalus producing headache, nausea, vomiting, lethergy, increasing head circumference, Parinaud’s syndrome, precocious puberty<br />
Pineal Region Tumors:<br />CSF tumor markers are used for following treatment response<br />Test dose XRT was previously employed but trend is toward tissue diagnosis before treatment<br />Germinomas are very sensitive to radiation and chemotherapy<br />
Metastatic Tumors:<br />Brain metastases are the most common brain tumor seen clinically (170,000 new cases per year)<br />In adults, lung and breast CA account for 50% of cerebral mets<br />At onset of neurologic symptoms, 70% will be multiple on MRI<br />
Metastatic Tumors - Treatments<br />If unknown primary or unconfirmed diagnosis = surgical excision or biopsy<br />Solitary symptomatic, large, or accessible lesion = surgical excision + WBXRT or GK<br />Solitary asymptomatic, small or inaccessible lesion = WBXRT or GKS<br />Multiple mets = < 3 GKS; > 3 WBXRT<br />Uncontrolled widespread systemic dz = WBXRT or no treatment<br />
Radiosurgery and Brain Mets<br />Gamma Knife Radiosurgery:<br /><ul><li>Uses stereotactic localization to precisely focus therapeutic radiation on a lesion
Radiosurgery can generally be used to treat patients with multiple brain metastases in a single procedure
Lesions must be smaller than three centimeters
Radiosurgery does no prevent the development of distant brain metastases </li></li></ul><li>The Spinal Cord<br />
Tumors of the Spinal Cord<br />15% of primary CNS tumors are intraspinal<br />Most primary CNS spinal tumors are benign (unlike the case with intracranial tumors)<br />Most present with symptoms of compression rather than invasion<br />
Types of Spinal Tumors<br />Extradural – arise outside cord in vertebral bodies or epidural tissues (55%)<br />Intraduralextramedullary – arise in leptomeninges or roots (40%)<br />Intramedullary – arise in spinal cord substance and invade and destroy tracts and gray matter (5%)<br />
Metastatic Spinal Cord Tumors<br />Comprise the majority of extradural tumors<br />Most are osteolytic (cause bony destruction)<br />Common ones include lymphoma, lung, breast and prostate<br />Present with back pain that persists in recumbency and often with myelopathy<br />
Metastatic Spinal Cord Tumors<br />Treatment options:<br /><ul><li>If no neurologic compromise or bony instability, usual treatment is biopsy followed by XRT
Surgery may be helpful to preserve ambulation or for stabilization
Surgery not helpful for total paralysis > 8 hours, loss of ambulation > 24 hours, prognosis < 3-4 months, poor medical condition</li></li></ul><li>Spinal Meningiomas<br />Most are intraduralextramedullary<br />Peak age is 40 – 70 years<br />Female:male ratio = 4:1<br />82% thoracic, 15% cervical, 2% lumbar<br />Symptoms: local or radicular pain, motor deficits, sensory symptoms<br />Surgery – recurrence rate with complete excision is 7%<br />
Spinal Schwannomas<br />Mostly intraduralextramedullary (8-32% may be completely extradural)<br />Slow growing benign tumor<br />Early symptoms are often radicular<br />Recurrence is rare after total excision<br />
Spinal Ependymoma<br />Accounts for 30% of intramedullary spinal cord tumors<br />The most common glioma of lower cord, conus and filum<br />Slow growing and benign<br />More common in adults<br />Evaluation requires imaging of entire neuraxis (due to seeding)<br />Treatment: surgical excision<br />
Spinal Astrocytoma<br />Intramedullary tumor that peaks in 3rd – 5th decades<br />Ratio of low grade:high grade = 3:1<br />Occurs at all levels (thoracic most common)<br />Temporal progression of symptoms dysfunction<br />Treatment – excision, biopsy, RTX (+/- chemo) for high grade only<br />50% recurrence rate in 4 – 5 years<br />
PseudotumorCerebri<br />AKA idiopathic intracranial hypertension (IIH) and benign intracranial hypertension<br />Symptomatic ICP elevation > 20 cm H20 and papilledema in the absence of intracranial mass, hydrocephalus, infection or hypertensive encephalopathy<br />There is a juvenile and adult form<br />
PseudotumorCerebri - Criteria<br />Signs and symptoms of increased ICP<br />No localizing signs other than CN VI palsy in an otherwise awake and alert patient<br />Increased CSF pressure without chemical or cytological abnormalities<br />Normal to small ventricles and no intracranial mass<br />
PseudotumorCerebri - Epidemiolgy<br />Female to male ratio 8:1<br />Obesity is reported in majority of cases<br />Peak incidence in the 3rd decade<br />Frequently self limited<br />Severe visual deficits develop in 4-12%<br />
PseudotumorCerebri - Clinical<br />Symptoms: Headache, nausea, visual loss and diplopia<br />Signs: Papilledema (almost 100%), abducens nerve palsy (20%), visual field defect (9%)<br />Associations: obesity, drugs (keprone, lindane, accutane, tmp-smo, cimetadine) and hypervitaminosis A <br />
PseudotumorCerebri - Evaluation<br />MRI with and without contrast<br />MRV to rule out dural sinus or venous thrombosis<br />Lumbar puncture to measure opening pressure and for CSF analysis<br />Neuro-opthalmologic evaluation. Will require serial evaluation.<br />
PseudotumorCerebri - Treatment<br />Spontaneous resolution is common (usually around 1 year)<br />Stop possible offending drugs<br />Weight loss<br />Fluid and salt restriction<br />Diuretics to slow CSF production (carbonic anhydrase inhibitors i.e. acetazolamide)<br />Surgery – Lumbar shunt, optic nerve fenestration<br />Neuro-opthalmologic evaluation<br />
Vertebrobasilar Insufficiency<br />The 5 D’s of VBI:<br /><ul><li>“drop attack”
C1-2 posterior arthrodesis</li></li></ul><li>Vertebrobasilar Insufficiency<br />Prognosis:<br /><ul><li>Estimated stroke rate 4.5 – 7% per year
Risk of CVA after first VBI-TIA has been estimated as 22% for the first year</li></li></ul><li>Vestibular Neuronitis<br />About 5% of all dizziness (and perhaps 15% of all vertigo) is due to vestibular neuritis or labyrinthitis. <br />It occurs in all age groups, but cases are rare in children.<br />Usually begins as a nonspecific viral illness<br />
Vestibular Neuronitis<br />Signs and symptoms:<br /><ul><li>Vertigo
Prednisone – controversial</li></ul>Prognosis – usually self-limited from 3 – 6 weeks<br />
EndolymphaticHydrops<br />aka Meniere’s Disease <br />Cause – increased endolymphatic volume and pressure with dilation of endolymph spaces and fistulization into the perilymphatic spaces<br />Incidence: 1 per 100,000<br />Most cases have onset between 30-60 years of age<br />Bilateral in 20%<br />
EndolymphaticHydrops<br />Symptoms:<br /><ul><li>Attacks of violent vertigo with N/V. Balance is normal between attacks.