13. Meningiomas: Account for 14 – 19% of primary brain tumors Incidence peaks at 45 years of age Female to Male ratio 2:1 Arise from arachnoid cap cells Extra-axial (dural based)
14. Meningiomas: Usually benign (less than 5% are malignant) and slow growing Location – most commonly located along the falx, convexity or sphenoid bone Symptoms: often presents with seizure, headache Studies: MRI with gadolinium shows attachment on dura often with a dural tail and typically enhances densely
22. Acoustic Neuromas Vestibular Schwannoma is currently preferred since most arise from the superior division of vestibular nerve Make up 8-10% of primary brain tumors Incidence: usually after age 30 Pathology: AntoniA (narrow elongated bipolar cells) and Antoni B (loose reticulated)
34. Diagnostic Tests: MRI of brain with and without gadolinium Labs – prolactin, cortisol, TSH, T3, T4, IGF-1, LH, FSH
35. Pituitary Adenomas - treatment Medical - Bromocriptine Surgery - transphenoidal, craniotomy Radiation Conventional Radiosurgery - LINAC, Gamma Knife, Cyberknife
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39. Hemangioblastomas Most common primary intra-axial tumor in the adult posterior fossa May also occur in spinal cord Rare supratentorially 20% occur as part of vonHippel-Lindau disease
40. Hemangioblastomas Symptoms: those of any posterior fossa mass (HA, N/V, dizziness, ataxia, dysarthria, nystagmus) Evaluation: MRI with contrast of the entire neuraxis Treatment: Surgery is curative in sporadic cases. Pre-operative embolization to reduce vascularity
44. GLIOMAS Arise from Glial Cells Astrocytomas Astocytomas fall on a gradient that ranges from benign to malignant Benign Malignant Glioblastomamultiforme Low Grade PilocyticAstocytomas Diffuse Low Grade Astrocytomas
52. Low-Grade Astrocytomas Three cell types: fibrillary, gemistocytic and protoplasmic Tend to occur in children and young adult Most present with seizures Ultimate behavior of these tumors is usually NOT benign. The major cause of morbidity is differentiation to a more malignant grade.
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54. MRI – abnormal signal on T2; usually without enhancement
67. Representing 1.44 % of all cancersdiagnosed in 2010Central Brain Tumor Registry of the United States (CBTRUS) 2004-2007
68. Mortality An estimated 13,140 deaths in 2010will be attributable to primary malignantbrain tumors Central Brain Tumor Registry of the United States (CBTRUS) 2004-2007.
69. High-Grade Malignant Gliomas Account for 77.5% of all Gliomas n=16,780. Central Brain Tumor Registry of the United States (CBTRUS) 2002-2003. Statistical Report 1995-1999.
70. Anaplastic Astrocytoma and GlioblastomaMultiforme Grade III and Grade IV Mean age for AA is 46 years old Mean age for GBM is 56 years old Male:female=3:2 GBM accounts for 25% of all adult brain tumors (50-55% of all gliomas)
81. Medulloblastoma A small-cell embryonal tumor of the cerebellum The most common pediatric brain malignancy Median age at diagnosis: 5 – 7 years Male:female ratio is 2:1
93. Brain Stem Glioma Not a homogeneous group – some are more malignant Lower grade tumors tend to occur in the upper brain stem Higher grade tumors tend to occur in the lower brainstem/medulla Most are malignant, have poor prognosis and are not surgical candidates
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95. Lower brainstem – multiple lower cranial nerve deficits and long tract findingsPrognosis – most children with malignant BSG will die within 6 – 12 months
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97. Pineal Region Tumors: Tumors in this region are more common in children (3 – 8% of pediatric tumors) Over 17 tumor types occur in this area Most common is germinoma followed by astrocytoma, teratoma and pineoblastoma Many metastasize easily through the CSF and therefore MRI of neuraxis is required
98. Pineal Region Tumors: Presentation – hydrocephalus producing headache, nausea, vomiting, lethergy, increasing head circumference, Parinaud’s syndrome, precocious puberty
99. Pineal Region Tumors: CSF tumor markers are used for following treatment response Test dose XRT was previously employed but trend is toward tissue diagnosis before treatment Germinomas are very sensitive to radiation and chemotherapy
102. Metastatic Tumors: Brain metastases are the most common brain tumor seen clinically (170,000 new cases per year) In adults, lung and breast CA account for 50% of cerebral mets At onset of neurologic symptoms, 70% will be multiple on MRI
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104. Metastatic Tumors - Treatments If unknown primary or unconfirmed diagnosis = surgical excision or biopsy Solitary symptomatic, large, or accessible lesion = surgical excision + WBXRT or GK Solitary asymptomatic, small or inaccessible lesion = WBXRT or GKS Multiple mets = < 3 GKS; > 3 WBXRT Uncontrolled widespread systemic dz = WBXRT or no treatment
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106. Radiosurgery can generally be used to treat patients with multiple brain metastases in a single procedure
109. Tumors of the Spinal Cord 15% of primary CNS tumors are intraspinal Most primary CNS spinal tumors are benign (unlike the case with intracranial tumors) Most present with symptoms of compression rather than invasion
110. Types of Spinal Tumors Extradural – arise outside cord in vertebral bodies or epidural tissues (55%) Intraduralextramedullary – arise in leptomeninges or roots (40%) Intramedullary – arise in spinal cord substance and invade and destroy tracts and gray matter (5%)
111. Metastatic Spinal Cord Tumors Comprise the majority of extradural tumors Most are osteolytic (cause bony destruction) Common ones include lymphoma, lung, breast and prostate Present with back pain that persists in recumbency and often with myelopathy
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114. Surgery may be helpful to preserve ambulation or for stabilization
117. Spinal Schwannomas Mostly intraduralextramedullary (8-32% may be completely extradural) Slow growing benign tumor Early symptoms are often radicular Recurrence is rare after total excision
119. Spinal Ependymoma Accounts for 30% of intramedullary spinal cord tumors The most common glioma of lower cord, conus and filum Slow growing and benign More common in adults Evaluation requires imaging of entire neuraxis (due to seeding) Treatment: surgical excision
121. Spinal Astrocytoma Intramedullary tumor that peaks in 3rd – 5th decades Ratio of low grade:high grade = 3:1 Occurs at all levels (thoracic most common) Temporal progression of symptoms dysfunction Treatment – excision, biopsy, RTX (+/- chemo) for high grade only 50% recurrence rate in 4 – 5 years
123. PseudotumorCerebri AKA idiopathic intracranial hypertension (IIH) and benign intracranial hypertension Symptomatic ICP elevation > 20 cm H20 and papilledema in the absence of intracranial mass, hydrocephalus, infection or hypertensive encephalopathy There is a juvenile and adult form
124. PseudotumorCerebri - Criteria Signs and symptoms of increased ICP No localizing signs other than CN VI palsy in an otherwise awake and alert patient Increased CSF pressure without chemical or cytological abnormalities Normal to small ventricles and no intracranial mass
125. PseudotumorCerebri - Epidemiolgy Female to male ratio 8:1 Obesity is reported in majority of cases Peak incidence in the 3rd decade Frequently self limited Severe visual deficits develop in 4-12%
126. PseudotumorCerebri - Pathogenesis Not fully understood Mechanical theory: obesity intra-abdominal pressure central venous pressure CSF resorption ICP
127. PseudotumorCerebri - Clinical Symptoms: Headache, nausea, visual loss and diplopia Signs: Papilledema (almost 100%), abducens nerve palsy (20%), visual field defect (9%) Associations: obesity, drugs (keprone, lindane, accutane, tmp-smo, cimetadine) and hypervitaminosis A
128. PseudotumorCerebri - Evaluation MRI with and without contrast MRV to rule out dural sinus or venous thrombosis Lumbar puncture to measure opening pressure and for CSF analysis Neuro-opthalmologic evaluation. Will require serial evaluation.
129. PseudotumorCerebri - Treatment Spontaneous resolution is common (usually around 1 year) Stop possible offending drugs Weight loss Fluid and salt restriction Diuretics to slow CSF production (carbonic anhydrase inhibitors i.e. acetazolamide) Surgery – Lumbar shunt, optic nerve fenestration Neuro-opthalmologic evaluation
153. EndolymphaticHydrops aka Meniere’s Disease Cause – increased endolymphatic volume and pressure with dilation of endolymph spaces and fistulization into the perilymphatic spaces Incidence: 1 per 100,000 Most cases have onset between 30-60 years of age Bilateral in 20%
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
Cavernous meningioma
Parasagitalmeningioma
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
It is thought to result from a reactivation of herpes simplex virus that affects the vestibular ganglion, vestibular nerve, labyrinth, or a combination of these.
Vertigo - when one of the two vestibular nerves is infected, there is an imbalance between the two sides, and vertigo appears.Nystagmus is away from the affected ear.
Attack duration – usually 5-30 minutes but may last as long as 6 hoursTinnitus – often described as sound of escaping steam