CSF Tumors

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  • 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
  • Extradural = metsIntraduralextramedullary = meningioma,schwannomaIntramedullary = ependymoma, astrocytoma
  • 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
  • Compression with:Head turning = Bow hunter’s strokeOs odontoideumAnterior atlantoaxialsubluxation (ie rheumatoid arthritis)Rotatoryatlantoaxialsubluxation
  • 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
  • Vestibular suppressants – valium probably most effectiveSurgery – endolymphatic shunting, nonselective vestibular ablation, selective neurectomy

Transcript

  • 1.
  • 2. Brain And Spinal Tumors
    Keith Tucci, MD
    Connie Cerne, PA-C
  • 3. Brain Tumors
    Primary - metastatic
    Benign - malignant
    Adult - childhood
    Supratentorial - infratentorial
    Intraaxial - extraaxial
  • 4. Physical Findings:
    Depends on location of tumor
  • 9. Diagnostic Tests:
    MRI of brain with and without gadolinium
    Other special tests:
    • EEG – for suspected seizure activity
    • 10. Labs for pituitary tumors – i.e. prolactin, cortisol, TSH, T3, T4, IGF-1, LH, FSH
  • Benign Brain Tumors
    Meningiomas
    Acoustic Neuromas
    Pituitary Adenomas
    Hemangioblastomas
    PilocyticAstrocytomas
  • 11. Malignant tumors
    Gliomas
    Medulloblastomas
    Germ cell tumors
    Metastatic - lung, breast, GI, melanoma
  • 12. Intracranial Tumors
    Adult
    90% supratentorial
    10% infratentorial
    Childhood
    70% infratentorial
    30% supratentorial
  • 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
  • 15. Meningiomas:
    Treatment Options:
    • Observation with serial MRI’s for evidence of growth
    • 16. Gamma Knife Radiosurgery
    • 17. Craniotomy
  • 18.
  • 19.
  • 20.
  • 21.
  • 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)
  • 23. Acoustic Neuromas
    Symptoms: hearing loss, tinnitus and disequilibrium
    Studies: MRI with/without gadolinium; audiometric evaluation
  • 24. Acoustic Neuromas
    Treatment:
    • Expectant management: follow symptoms, hearing and tumor growth on serial imaging. Intervention performed for progression
    • 25. Gamma Knife Radiosurgery
    • 26. Craniotomy – retromastoid, translabyrinthine or middle fossa approach
  • 27.
  • 28.
  • 29.
  • 30.
  • 31. Pituitary Tumors
    Microadenomas - Macroadenomas
    Secretory - Nonsecretory
    Endocrine effect - mass effect
  • 32. Pituitary Adenomas
    Prolactinomas - 30%
    Growth hormone (Acromegaly) - 15%
    ACTH (Cushing’s Disease) - 15%
    Glycoprotein (LH, FSH, TSH, alpha subunit) - 15%
    Non-secreting - 20%
  • 33.
  • 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
  • 36.
  • 37.
  • 38.
  • 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
  • 41.
  • 42.
  • 43. Gliomas
  • 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
  • 45. Neuropathological Grading
    WHO Classification:
    • Grade I – pilocyticastrocytoma
    • 46. Grade II – diffuse astrocytoma (low grade)
    • 47. Grade III – anaplasticastrocytoma
    • 48. Grade IV – glioblastoma (GBM)
  • PilocyticAstrocytoma
    Can arise throughout the neuroaxis (cerebellum, optic nerve, hypothalamus)
    Age at presentation < 20 years in 75%
    Often cystic and half have a mural nodule
    Prognosis: 94% 10 year survival
  • 49.
  • 50.
  • 51.
  • 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.
  • 53. Low-Grade Astrocytomas
    Radiographic Appearance:
    • CT – low density
    • 54. MRI – abnormal signal on T2; usually without enhancement
    • 55. Predilection for temporal, posterior frontal and anterior parietal lobes
  • 56. T2 weighted
    T1 weighted
  • 57. Low-Grade Astrocytomas
    Treatment Options:
    • No treatment – follow serial imaging and neuro exam
    • 58. Radiation
    • 59. Chemotherapy
    • 60. Surgery
    • 61. Combination of radiation and chemotherapy
  • Low-Grade Astrocytomas
    Prognosis for survival: 7 – 10 years
    Prognosis is worse if:
    • Patient is > 50 years old at time of diagnosis
    • 62. Karnofsky performance score is < 80
    • 63. Tumor is located in elequent brain
    • 64. Maximal diameter > 4 cm
  • Malignant tumors
    Gliomas - Astrocytomas, oligodendrogliomas, ependymomas
    Medulloblastomas
    Germ cell tumors
    Metastatic - lung, breast, kidney, GI melanoma
  • 65. Incidence
    Estimated 22,020 new cases of primary malignant brain tumors in 2010
    • 11,980 in males
    • 66. 10,040 in females
    • 67. Representing 1.44 % of all cancersdiagnosed in 2010
    Central 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)
  • 71. Anaplastic Astrocytoma and GlioblastomaMultiforme
    Radiographic appearance:
    • AA – complex enhancement
    • 72. GBM – necrosis (ring enhancement)
  • 73. Grade IV - GBM
  • 74.
  • 75.
  • 76. AA and GBM
    Treatment: Cytoreductive surgery followed by external beam radiation and chemotherapy
    Poor candidates for surgical intervention:
    • Extensive dominant lobe GBM
    • 77. Lesions with significant bilateral involvement
    • 78. Karnofsky score < 70
    • 79. Multicentricgliomas
  • AA and GBM
    Prognostic factors - age, duration of symptoms, neurological status, extent of resection
    AA – median survival is 36 months
    GBM – median survival is 10 months (without treatment 95% die within 3 months)
  • 80. Medulloblastomas
  • 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
  • 82. Medulloblastoma
    Symptoms:
    • usually arise in the cerebellar vermis at the apex of the roof of the 4th ventricle predisposing to early obstructive hydrocephalus.
    • 83. Headache, nausea, vomiting, ataxia
    • 84. Drop mets may produce back pain, urinary retention or leg weakness
  • Medulloblastoma
    Evaluation:
    • MRI with gadolinium shows most lesions in the midline region of the 4th ventricle.
    • 85. Most with hydrocephalus
    • 86. T1 images hypo to isointense. Most enhance.
    • 87. All patients must be evaluated for “drop mets”
  • 88.
  • 89. Medulloblastoma
    Treatment:
    • Treatment of choice is surgical debulking (brainstem invasion usually limits complete surgical excision) followed by craniospinal XRT
    • 90. Chemotherapy – reserved for recurrence, poor risk patients or for children < 3 years
    • 91. Shunt – 30 – 40% require
  • 92. Brain stem glioma
  • 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
  • 94. Brain Stem Glioma
    Presentation:
    • Upper brainstem – cerebellar findings and rarely hydrocephalus
    • 95. Lower brainstem – multiple lower cranial nerve deficits and long tract findings
    Prognosis – most children with malignant BSG will die within 6 – 12 months
  • 96.
  • 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
  • 100.
  • 101. Metastatic tumors
  • 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
  • 103.
  • 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
  • 105. Radiosurgery and Brain Mets
    Gamma Knife Radiosurgery:
    • Uses stereotactic localization to precisely focus therapeutic radiation on a lesion
    • 106. Radiosurgery can generally be used to treat patients with multiple brain metastases in a single procedure
    • 107. Lesions must be smaller than three centimeters
    • 108. Radiosurgery does no prevent the development of distant brain metastases
  • The Spinal Cord
  • 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
  • 112.
  • 113. Metastatic Spinal Cord Tumors
    Treatment options:
    • If no neurologic compromise or bony instability, usual treatment is biopsy followed by XRT
    • 114. Surgery may be helpful to preserve ambulation or for stabilization
    • 115. Surgery not helpful for total paralysis > 8 hours, loss of ambulation > 24 hours, prognosis < 3-4 months, poor medical condition
  • Spinal Meningiomas
    Most are intraduralextramedullary
    Peak age is 40 – 70 years
    Female:male ratio = 4:1
    82% thoracic, 15% cervical, 2% lumbar
    Symptoms: local or radicular pain, motor deficits, sensory symptoms
    Surgery – recurrence rate with complete excision is 7%
  • 116. Spinal Meningioma
  • 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
  • 118. Spinal Schwannoma
  • 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
  • 120. Spinal Ependymoma
  • 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
  • 122. Spinal Astrocytoma
  • 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
  • 130. Vertebrobasilar Insufficiency
    The 5 D’s of VBI:
  • Vertebrobasilar Insufficiency
    Causes:
    • Subclavian steal
    • 135. Stenosis of both VAs or one VA when the other is hypofunctional
    • 136. Embolism
    • 137. Atherosclerotic occlusion of brainstem perforators
    • 138. Compression of the VA at the C1-2 level
  • Vertebrobasilar Insufficiency
    Evaluation – selective four vessel cerebral angiography
    Treatment:
  • Vertebrobasilar Insufficiency
    Prognosis:
    • Estimated stroke rate 4.5 – 7% per year
    • 143. Risk of CVA after first VBI-TIA has been estimated as 22% for the first year
  • Vestibular Neuronitis
    About 5% of all dizziness (and perhaps 15% of all vertigo) is due to vestibular neuritis or labyrinthitis.
    It occurs in all age groups, but cases are rare in children.
    Usually begins as a nonspecific viral illness
  • 144. Vestibular Neuronitis
    Signs and symptoms:
  • Vestibular Neuronitis
    Treatment:
    • Anti-emetics (phenergan, compazine)
    • 150. Vestibular suppressants (meclizine)
    • 151. If herpes-virus suspected – acyclovir
    • 152. Prednisone – controversial
    Prognosis – usually self-limited from 3 – 6 weeks
  • 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%
  • 154. EndolymphaticHydrops
    Symptoms:
    • Attacks of violent vertigo with N/V. Balance is normal between attacks.
    • 155. Tinnitus
    • 156. Fluctuating low frequency hearing loss
  • EndolymphaticHydrops
    Diagnostic Studies:
    • Electronystagmography (ENG)
    • 157. Audiogram
    • 158. MRI Brain – no findings
  • EndolymphaticHydrops
    Treatment:
    • Salt and caffeine restriction
    • 159. Diuretics - diamox
    • 160. Vestibular suppressants – valium, antivert
    • 161. Vasodilators
    • 162. Surgical treatment – reserved for incapacitating cases