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

5. Parietal

6. Occipital

7. Temporal

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

16. Gamma Knife Radiosurgery

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

25. Gamma Knife Radiosurgery

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%

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

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

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

46. Grade II – diffuse astrocytoma (low grade)

47. Grade III – anaplasticastrocytoma

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.

54. MRI – abnormal signal on T2; usually without enhancement

56. T2 weighted T1 weighted

58. Radiation

59. Chemotherapy

60. Surgery

62. Karnofsky performance score is < 80

63. Tumor is located in elequent brain

66. 10,040 in females

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)

73. Grade IV - GBM

77. Lesions with significant bilateral involvement

78. Karnofsky score < 70

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

83. Headache, nausea, vomiting, ataxia

85. Most with hydrocephalus

86. T1 images hypo to isointense. Most enhance.

90. Chemotherapy – reserved for recurrence, poor risk patients or for children < 3 years

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

95. Lower brainstem – multiple lower cranial nerve deficits and long tract findingsPrognosis – most children with malignant BSG will die within 6 – 12 months

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

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

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

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

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

114. Surgery may be helpful to preserve ambulation or for stabilization

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

131. Diplopia

132. Dysarthria

133. Defect (visual)

135. Stenosis of both VAs or one VA when the other is hypofunctional

136. Embolism

137. Atherosclerotic occlusion of brainstem perforators

139. Antiplatelets

140. Vertebral endarterectomy

141. Bypass grafting

145. Nausea

146. Vomiting

147. Spontaneous nystagmus – not provoked

148. Loss of balance on the Romberg test

150. Vestibular suppressants (meclizine)

151. If herpes-virus suspected – acyclovir

152. Prednisone – controversialPrognosis – 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%

155. Tinnitus

157. Audiogram

159. Diuretics - diamox

160. Vestibular suppressants – valium, antivert

161. Vasodilators