CSF Tumors

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  • We are a Brain Tumor Treatment Center and most of our treatment depends on a number of factors including the type, location and size of the tumor as well as the patient's age and general health. The Treatment methods may vary and at Primus Hospital we provide treatment for both benign and non benign tumor. Visit - http://www.primushospital.com and Call 9953722892
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CSF Tumors

  1. 1.
  2. 2. Brain And Spinal Tumors<br />Keith Tucci, MD<br />Connie Cerne, PA-C<br />
  3. 3. Brain Tumors<br />Primary - metastatic<br />Benign - malignant<br />Adult - childhood<br />Supratentorial - infratentorial<br />Intraaxial - extraaxial<br />
  4. 4. Physical Findings:<br />Depends on location of tumor<br /><ul><li>Frontal
  5. 5. Parietal
  6. 6. Occipital
  7. 7. Temporal
  8. 8. Cerebellum </li></li></ul><li>
  9. 9. Diagnostic Tests:<br />MRI of brain with and without gadolinium<br />Other special tests:<br /><ul><li>EEG – for suspected seizure activity
  10. 10. Labs for pituitary tumors – i.e. prolactin, cortisol, TSH, T3, T4, IGF-1, LH, FSH</li></li></ul><li>Benign Brain Tumors<br />Meningiomas<br />Acoustic Neuromas<br />Pituitary Adenomas<br />Hemangioblastomas<br />PilocyticAstrocytomas<br />
  11. 11. Malignant tumors<br />Gliomas<br />Medulloblastomas<br />Germ cell tumors<br />Metastatic - lung, breast, GI, melanoma<br />
  12. 12. Intracranial Tumors<br />Adult<br />90% supratentorial<br />10% infratentorial<br />Childhood<br />70% infratentorial<br />30% supratentorial<br />
  13. 13. 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 />
  14. 14. 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 />
  15. 15. Meningiomas:<br />Treatment Options:<br /><ul><li>Observation with serial MRI’s for evidence of growth
  16. 16. Gamma Knife Radiosurgery
  17. 17. Craniotomy</li></li></ul><li>
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  22. 22. 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 />
  23. 23. Acoustic Neuromas<br />Symptoms: hearing loss, tinnitus and disequilibrium<br />Studies: MRI with/without gadolinium; audiometric evaluation<br />
  24. 24. Acoustic Neuromas<br />Treatment: <br /><ul><li>Expectant management: follow symptoms, hearing and tumor growth on serial imaging. Intervention performed for progression
  25. 25. Gamma Knife Radiosurgery
  26. 26. Craniotomy – retromastoid, translabyrinthine or middle fossa approach</li></li></ul><li>
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  31. 31. Pituitary Tumors<br />Microadenomas - Macroadenomas<br />Secretory - Nonsecretory<br />Endocrine effect - mass effect<br />
  32. 32. Pituitary Adenomas<br />Prolactinomas - 30%<br />Growth hormone (Acromegaly) - 15%<br />ACTH (Cushing’s Disease) - 15%<br />Glycoprotein (LH, FSH, TSH, alpha subunit) - 15%<br />Non-secreting - 20%<br />
  33. 33.
  34. 34. Diagnostic Tests:<br />MRI of brain with and without gadolinium<br />Labs – prolactin, cortisol, TSH, T3, T4, IGF-1, LH, FSH<br />
  35. 35. Pituitary Adenomas - treatment<br />Medical - Bromocriptine<br />Surgery - transphenoidal, craniotomy<br />Radiation <br />Conventional<br />Radiosurgery - LINAC, Gamma Knife, Cyberknife<br />
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  38. 38.
  39. 39. 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 />
  40. 40. 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 />
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  42. 42.
  43. 43. Gliomas<br />
  44. 44. 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 />
  45. 45. Neuropathological Grading<br />WHO Classification:<br /><ul><li>Grade I – pilocyticastrocytoma
  46. 46. Grade II – diffuse astrocytoma (low grade)
  47. 47. Grade III – anaplasticastrocytoma
  48. 48. 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 />
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  52. 52. 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 />
  53. 53. Low-Grade Astrocytomas<br />Radiographic Appearance: <br /><ul><li>CT – low density
  54. 54. MRI – abnormal signal on T2; usually without enhancement
  55. 55. Predilection for temporal, posterior frontal and anterior parietal lobes </li></li></ul><li>
  56. 56. T2 weighted<br />T1 weighted<br />
  57. 57. Low-Grade Astrocytomas<br />Treatment Options: <br /><ul><li>No treatment – follow serial imaging and neuro exam
  58. 58. Radiation
  59. 59. Chemotherapy
  60. 60. Surgery
  61. 61. 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
  62. 62. Karnofsky performance score is < 80
  63. 63. Tumor is located in elequent brain
  64. 64. Maximal diameter > 4 cm </li></li></ul><li>Malignant tumors<br />Gliomas - Astrocytomas, oligodendrogliomas, ependymomas<br />Medulloblastomas<br />Germ cell tumors<br />Metastatic - lung, breast, kidney, GI melanoma<br />
  65. 65. Incidence<br />Estimated 22,020 new cases of primary malignant brain tumors in 2010<br /><ul><li>11,980 in males
  66. 66. 10,040 in females
  67. 67. Representing 1.44 % of all cancersdiagnosed in 2010</li></ul>Central Brain Tumor Registry of the United States (CBTRUS) 2004-2007<br />
  68. 68. 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 />
  69. 69. 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 />
  70. 70. 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 />
  71. 71. Anaplastic Astrocytoma and GlioblastomaMultiforme<br />Radiographic appearance:<br /><ul><li>AA – complex enhancement
  72. 72. GBM – necrosis (ring enhancement)</li></li></ul><li>
  73. 73. Grade IV - GBM<br />
  74. 74.
  75. 75.
  76. 76. AA and GBM<br />Treatment: Cytoreductive surgery followed by external beam radiation and chemotherapy<br />Poor candidates for surgical intervention: <br /><ul><li>Extensive dominant lobe GBM
  77. 77. Lesions with significant bilateral involvement
  78. 78. Karnofsky score < 70
  79. 79. 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 />
  80. 80. Medulloblastomas<br />
  81. 81. Medulloblastoma<br />A small-cell embryonal tumor of the cerebellum<br />The most common pediatric brain malignancy<br />Median age at diagnosis: 5 – 7 years<br />Male:female ratio is 2:1<br />
  82. 82. Medulloblastoma<br />Symptoms:<br /><ul><li>usually arise in the cerebellar vermis at the apex of the roof of the 4th ventricle predisposing to early obstructive hydrocephalus.
  83. 83. Headache, nausea, vomiting, ataxia
  84. 84. 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.
  85. 85. Most with hydrocephalus
  86. 86. T1 images hypo to isointense. Most enhance.
  87. 87. All patients must be evaluated for “drop mets”</li></li></ul><li>
  88. 88.
  89. 89. Medulloblastoma<br />Treatment:<br /><ul><li>Treatment of choice is surgical debulking (brainstem invasion usually limits complete surgical excision) followed by craniospinal XRT
  90. 90. Chemotherapy – reserved for recurrence, poor risk patients or for children < 3 years
  91. 91. Shunt – 30 – 40% require </li></li></ul><li>
  92. 92. Brain stem glioma<br />
  93. 93. 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 />
  94. 94. Brain Stem Glioma<br />Presentation:<br /><ul><li>Upper brainstem – cerebellar findings and rarely hydrocephalus
  95. 95. Lower brainstem – multiple lower cranial nerve deficits and long tract findings</li></ul>Prognosis – most children with malignant BSG will die within 6 – 12 months<br />
  96. 96.
  97. 97. 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 />
  98. 98. Pineal Region Tumors:<br />Presentation – hydrocephalus producing headache, nausea, vomiting, lethergy, increasing head circumference, Parinaud’s syndrome, precocious puberty<br />
  99. 99. 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 />
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  101. 101. Metastatic tumors<br />
  102. 102. 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 />
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  104. 104. 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 />
  105. 105. Radiosurgery and Brain Mets<br />Gamma Knife Radiosurgery:<br /><ul><li>Uses stereotactic localization to precisely focus therapeutic radiation on a lesion
  106. 106. Radiosurgery can generally be used to treat patients with multiple brain metastases in a single procedure
  107. 107. Lesions must be smaller than three centimeters
  108. 108. Radiosurgery does no prevent the development of distant brain metastases </li></li></ul><li>The Spinal Cord<br />
  109. 109. 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 />
  110. 110. 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 />
  111. 111. 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 />
  112. 112.
  113. 113. Metastatic Spinal Cord Tumors<br />Treatment options:<br /><ul><li>If no neurologic compromise or bony instability, usual treatment is biopsy followed by XRT
  114. 114. Surgery may be helpful to preserve ambulation or for stabilization
  115. 115. 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 />
  116. 116. Spinal Meningioma<br />
  117. 117. 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 />
  118. 118. Spinal Schwannoma<br />
  119. 119. 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 />
  120. 120. Spinal Ependymoma<br />
  121. 121. 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 />
  122. 122. Spinal Astrocytoma<br />
  123. 123. 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 />
  124. 124. 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 />
  125. 125. 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 />
  126. 126. PseudotumorCerebri - Pathogenesis<br />Not fully understood<br />Mechanical theory: obesity intra-abdominal pressure central venous pressure CSF resorption ICP <br />
  127. 127. 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 />
  128. 128. 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 />
  129. 129. 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 />
  130. 130. Vertebrobasilar Insufficiency<br />The 5 D’s of VBI:<br /><ul><li>“drop attack”
  131. 131. Diplopia
  132. 132. Dysarthria
  133. 133. Defect (visual)
  134. 134. Dizziness</li></li></ul><li>Vertebrobasilar Insufficiency<br />Causes:<br /><ul><li>Subclavian steal
  135. 135. Stenosis of both VAs or one VA when the other is hypofunctional
  136. 136. Embolism
  137. 137. Atherosclerotic occlusion of brainstem perforators
  138. 138. Compression of the VA at the C1-2 level</li></li></ul><li>Vertebrobasilar Insufficiency<br />Evaluation – selective four vessel cerebral angiography<br />Treatment:<br /><ul><li>Anticoagulation
  139. 139. Antiplatelets
  140. 140. Vertebral endarterectomy
  141. 141. Bypass grafting
  142. 142. C1-2 posterior arthrodesis</li></li></ul><li>Vertebrobasilar Insufficiency<br />Prognosis:<br /><ul><li>Estimated stroke rate 4.5 – 7% per year
  143. 143. 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 />
  144. 144. Vestibular Neuronitis<br />Signs and symptoms:<br /><ul><li>Vertigo
  145. 145. Nausea
  146. 146. Vomiting
  147. 147. Spontaneous nystagmus – not provoked
  148. 148. Loss of balance on the Romberg test
  149. 149. NO deafness or tinnitus</li></li></ul><li>Vestibular Neuronitis<br />Treatment:<br /><ul><li>Anti-emetics (phenergan, compazine)
  150. 150. Vestibular suppressants (meclizine)
  151. 151. If herpes-virus suspected – acyclovir
  152. 152. Prednisone – controversial</li></ul>Prognosis – usually self-limited from 3 – 6 weeks<br />
  153. 153. 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 />
  154. 154. EndolymphaticHydrops<br />Symptoms:<br /><ul><li>Attacks of violent vertigo with N/V. Balance is normal between attacks.
  155. 155. Tinnitus
  156. 156. Fluctuating low frequency hearing loss</li></li></ul><li>EndolymphaticHydrops<br />Diagnostic Studies:<br /><ul><li>Electronystagmography (ENG)
  157. 157. Audiogram
  158. 158. MRI Brain – no findings</li></li></ul><li>EndolymphaticHydrops<br />Treatment:<br /><ul><li>Salt and caffeine restriction
  159. 159. Diuretics - diamox
  160. 160. Vestibular suppressants – valium, antivert
  161. 161. Vasodilators
  162. 162. Surgical treatment – reserved for incapacitating cases</li></li></ul><li>

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