Mainly focused on brain tumor classification, clinical presentation and mgt

1. Approach to Management of
CNS Tumors
Presenter: Bedru M. (MD, GSR-II)
Moderator: Dr. Yetsedaw (MD, Asst. Prof. of neurosurgery)
Dec-6, 2023

2. Outline
• Introduction
• Classification of brain tumor
• Grading and common clinical presentation
• Diagnosis and Management
• Discussion on common types of brain tumors
• References
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3. Objectives
• To know the overview of different types of CNS tumors
• To have common understanding on the common clinical presentation
and Dx modalities of CNS tumors
• To have an understanding about the management options
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4. Introduction
• Primary neoplasms of CNS account 10 % of all tumors
• These tumors occur 13/100,000 adult & 2/100,000 of pediatric age group
• There are 40,000 new cases of primary brain tumor in US
• Male predominance except meningioma.
• Glioma is the commonest brain tumor overall
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• GBM commonest malignant and meningioma commonest benign brain tumor.
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• In older age groups the commonest CNS tumor is metastatic one.
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5. Age specific incidence of primary Brain Tumors
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6. Classification
A. Primary vs Metastatic
B. Intra-axial vs extra-axial
C. Benign vs Malignant
D. Supratentorial vs Infratentorial
– Supratentorial: 85 %
– Infratentorial: 15 %
– Infratentorial: 60%
– Supratentorial: 40%
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Adult
Pediatric

7. • Glioma
• Metastasis
• Meningioma
• Pituitary origin tumor
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Adult Pediatric
• Astrocytoma
• Medulloblastoma
• Ependymoma
• Craniopharyngioma

8. WHO Histologic Classification of Tumors of the CNS
1. Tumors of Neuroepithelial Tissue
2. Tumors of Cranial and Spinal Nerves
3. Tumors of the Meninges
4. Tumors of Uncertain Histogenesis
• Hemangioblastoma from primitive vascular structures
5. Lymphomas and Hematopoietic Neoplasm
6. Germ Cell Tumor
• Ex: Germinoma – common in pineal gland area
7. Cysts and Tumor-like lesions
• Usually in the third ventricle
8. Tumors of the Sellar Regions
9. Local Extension from Regional Tumors
10. Metastatic Tumors
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9. Etiology & Risk factors
• Often unknown, under investigation
• Age, sex, Race
• Genetic Changes
• Family history/Familial Syndromes
• Occupational Exposures
• Ionizing Radiation
• Formaldehyde
• Vinyl chloride
• Acrylonitrile
• ?Electromagnetic fields (Including
cellular phones)
• Trauma, ?viruses
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10. 10
Clinical Presentations
Supratentorial Tumors
• Progressive neurologic deficit [68%]
• Headache [54%]
• Classic headache only in 8%
• Worse in the morning
• Nausea & Vomiting
• Exacerbated with coughing, straining, or
bending forward
• Mechanisms
• Raised ICP
• Invasion / compression effects of pain sensitive
structures
• Difficulty of vision

11. 11
• Seizure [25%]
• Aggressively look out for tumor in a first time idiopathic seizure in > 20 yrs.
old
• Mental status changes
• Tumor TIA / Stroke
• Pituitary Tumors
• Endocrine disturbance

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• Focal neurologic deficits
• Frontal lobe
• Personality changes, apathy, dementia
• Hemiparesis, dysphasia may occur
• Temporal lobe
• Auditory or olfactory hallucinations, déjà vu,
memory impairment
• Parietal lobe
• Contralateral motor / sensory impairment
• Homonymous hemianopsia
• Occipital lobe
• Contralateral visual field defect

13. 13
Infratentorial Tumors
• SSX of raised ICP
• Headache
• Nausea / vomiting
• Papilledema [50-90%]
• Diplopia
• Ataxia
• Vertigo
• Seizures are rare

14. 14
Mass effect in the posterior fossa
• Cerebellar hemisphere
• Limb ataxia, dysmetria, intention tremor
• Cerebellar vermis
• Broad based gait, truncal ataxia, titubation
• No limb ataxia
• Brain stem
• Multiple CN involvement
• Nystagmus

15. Diagnosis
• Hx and P/E
• Basic investigations:CBC,coagulation profile
• Imaging studies (MRI, CT scan, PET scan, angiogram)
• LP
• Biopsy/surgical removal of tumor
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16. Imaging
• MRI is more detailed and preferable
• MRI Brain & Spine ,pituitary protocol
• Initial diagnosis
• Post-op assessment of the extent of resection
• Monitoring response after chemotherapy / radiotherapy

17. MRI
• T1 Weighted
• Better anatomic details
• Fluid dark
• Gray matter gray
• White matter white
• Most pathologies are dark on T1
• Bright
• Fat, hemorrhage, calcification, cyst

18. • T2 Weighted
• Better pathological details
• Fluid bright
• Gray matter relatively bright
• White matter dark
• Contrast enhancement
• Benign/LG lesion-not enhance
• Malignant/HG lesion-enhance

19. • Extra-axial
• 80% are benign
• Meningiomas, Schwannomas
• Displacement or compression of
adjacent cortex
• CSF or cortical vessels between mass
and brain
• Enhancement and thickening of dura
or meninges
• Invasion of adjacent bone, less edema

20. • Intra-axial tumors
• Containment of margin by brain
parenchyma
• Expansion of the cortex

21. • Mass effect
• Direct signs
• Expansion of brain, meninges
• Indirect signs
• Effacement of Sulci, ventricular,
or cisternal
• Displacement gyri
• Obstructive hydrocephalus
• Herniation

22. Surgery
• Biopsy
• Maximum safe
Resection(GTR/STR)
• Ventriculostomy
• Pop imaging
• Within 3 days or after 30 days
• Contrast image … residual tumor
• Non-contrast image … to
differentiate blood from
enhancement

23. Radiation Therapy
• Usually after 7-10 days post-operatively
• Dramatic response in
• Lymphoma
• GCTs
• Types
• Stereotactic radiosurgery [SRS]
• External beam radiation therapy [EBRT]

24. Chemotherapy
• In general, there’s only minimal response of most brain tumors to
chemotherapy
• Exception - Oligodendrogliomas
• Options to bypass BBB
• Lipophilic agent … Nitrosoureas
• Higher doses of medications
• Disrupt BBB with Mannitol [Iatrogenic disruption of BBB]
• Bypass BBB with intrathecal Methotrexate for primary lymphoma

25. Adjuncts
• Steroids ?
• Dexamethasone
• Mainly in metastatic tumors
• Prophylactic anticonvulsants ?
• Shouldn’t be used routinely
• Indicated in patients with brain tumors undergoing craniotomy
• Taper-off starting 1 week post-op

26. Glioma
• Most common primary brain tumor
• 50% of all symptomatic brain tumors
• Incidence increases with advancing age
• Peak in 4th and 6th decades
• Ionizing radiation: the only clear risk factor
• Originate from glial cells or their stem cell precursors
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27. • Classified as
• Astrocytoma
• Oligodendroglioma
• Ependymoma
 WHO grade on basis
a. Increased cellularity
b. Nuclear atypia
c. Endothelial proliferation
d. Necrosis
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28. 1. Astrocytoma
• Arise from astrocytes.
• The most common tumors both in children(G-l) and adults(G-IV)
• Classified in to low grade(I, II), high grade(III, IV)
• The risk is high in NF1, and Li-fraumeni syndome
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29. Low Grade Astrocytoma
• G-I (Pilocytic) and G-II(LG diffuse) astrocytoma
• More common in younger pts.
• RF; ionizing radiation, fx hx NF, Li-fraumeni
• CF- seizure, headache on awakening, personality changes,
sluggishness, memory loss
• In children due to posterior and cerebellar predilection- dizziness,
ataxia, ocular Sx are common.
• Dx- MRI
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31. Rx:
• Grade-I = surgical resection curative. Observation + serial imaging
• Grade-II = Surgical resection often performed but not curative.
ChemoRx and RadioRx often used.
• G-I can be cured, G-II has median survival of 7 yrs.
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32. • Surgical indications
• Surgical biopsy
• High risk for herniation
• Pilocytic(supratentorial, and cerebellar(young))
• Obstructive hCP
• Refractory seizure
• To delay Adjuvant Rx (XRx), in children
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35. High Grade Astrocytoma
• Anaplastic (III) and Glioblastoma (IV)
• Occur in older adults, in frontal and may have central necrosis.
• They are aggressive types of tumor, symptoms are progressive.
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36. Rx – Surgical resection which is debulking, then radiation and chemo
• The four main goals of surgery for malignant gliomas are
1) to obtain a tissue diagnosis
2) to decrease the mass effect
3) to reduce the tumor burden
4) to increase survival and quality of life.
• Outcome is dismal
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39. 2.Oligodendroglioma
• Originates from oligodendrocytes
• Seen primarily in adults (median age 35 yrs), usually in frontal lobe
• CF- some asymptomatic. Common sx is seizure, headache with
increased ICP, weakness
• Grades (2 &3)
• Dx- MRI.
• Calcification, atypical contrast enhancement present
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40. Rx:
• Surgical resection (alone is not usually sufficient )
• Grade 2- surgery then
• Follow up and if recurrence (re-resection vs XRT +Chemo)
• Grade 3- Surgery + Radiotherapy and chemo (PCV)
• Outcome- better, if it recurs - treatment resistant
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41. 3.Ependymoma
• Arise from ependymal cells that lines ventricles
• 2nd
common in children and common cause of spinal tumor
• Commonly at post. Fossa
• Sx- obstructive hydrocephalus and sx of raised ICP in pedi
• In adults Sx Depend on location (hCP, CN-palsy), Sx of cauda equina
syndrome
• In adults 75 % arise within the spinal canal
• In children, 90 percent of ependymomas are intracranial, (75 in the posterior
fossa)
• Grades(1-3)
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42. Dx:
• MRI, LP
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43. Rx:
• Surgical resection is the primary treatment modality for
ependymomas.
• GTR, NTR, STR
• Adjuvant XRT is controversial for low-grade e, but considered for G-
2& 3
• The role of adjuvant chemotherapy is not well defined
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44. 4. Meningioma
• From meningothelial cells of arachnoid
• Primarily in adults, women, benign, cranial usually, may be spinal
• 14-19% of primary intracranial neoplasms.
• Commonly at Convexity and parasagittal areas
• F:M- 1.8:1, 6-7th
decade
• Sx- seizure, morning headache, apathy and weakness from
impingement of local structures.
• Again the symptoms vary depending on the location of the tumor
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45. • Dx – MRI (dura tail)
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46. Rx
• Observation
• Surgery – For symptomatic, progressively growing, and large tumors
• G-1 – Surgery alone is enough, and follow-up
• G-2 & 3- XRx is usually given
• Radiotherapy (as primary Rx) – elderly, deep and eloquent area tumor,
& for recurrence
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47. 5. Medulloblastoma
• Likely arises from embryonal cells in medulla
• 3rd
common cause of brain tumor in children, rare in adults
• Sx- headache, ataxia/gait disturbance, and nausea/vomiting, followed
by dizziness/vertigo (vestibular symptoms) and diplopia.
• Obstructive hydrocephalus due to 4th
ventricle blockage which is
common more in children.
• Macroscopic metastases are present more often in pediatric patients
than in adult patients- that makes prognosis worse in pedi.
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48. • Dx- MRI, Biopsy
• LP- mostly not done pre-op due to raised ICP-hCP. So, it is usually
done post-op
• Rx
• Surgery is main stay of mgt
• VP- shunt for hCP – but not recommended by all (resection of tumor can
treat it)
• Radiation can be added as an adjuvant Rx
• Chemotherapy has no clear benefit- but for children till XRx , it can be
given…
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49. 6. Pituitary Adenoma
• Account 10% of intracranial tumors, common in 3rd
and 4th
decade.
• Most of them are benign. Micro-adenoma vs Macro-adenoma
• Non-functioning adenoma are the most common types.
• Risk increases in MEN-1
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50. Clinical presentation
• Endocrine symptoms- present early
• Mass effect- non-functioning adenoma (late presentation)
• Incidentaloma and Apoplexy
• Sx- Could be due to oversecretion or underproduction(pituitary
compression) of hormones produced by ant. Pituitary
• Compression of other structures
• Optic chiasm – bitemporal hemianopsia
• 3rd
ventricle – obst. Hydrocephalus
• CN-3,4,5,6 Compression – ptosis, diplopia, fascial pain
• Headache- macroadenoma-due to increased intrasellar pressure
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51. • Craniopharyngioma – benign tumor that grow near pituitary(10%),
rathkes pouch epithelium, & may have compression sx
• Pituitary Ca is a rare highly invasive, and usually secretory tumor
• Rapidly growing & Anaplastic
• diagnosis relies on presence of distant metastasis
• Pituitary apoplexy- sudden hemorrhage, necrosis- neurohormonal sx
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52. Dx
• Hormonal tests
• X-ray, CT, MRI
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53. Rx
• Hormone secreting:
• Prolactinoma – dopamine agonists-bromocriptine
• ACTH & GH secreting tumors –Surgery is main stay of Rx
• TSH secreting – TSS, XRx, medical(octreotide works)
• Non- secreting tumors:
• Asymptomtic – follow-up visual field exam and MRI
• Elderly, no progression – expectant mgt
• Central and elderly – trans sphenoidal debulking
• Parasellar / young - radical surgery
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54. • Pituitary Macro-adenoma surgical indications
• Mass effect
• Rapid neurologic deterioration(apoplexy)
• Elevation of chiasm?
• For biopsy
• Invasive macroadenoma
• Routine XRx not recommended
• Bilateral adrenalectomy
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56. 7. Vestibular Schwanoma
• It arises from schwann cells (PNS) due to loss of tumor suppressor
gene in chr. 22. It is benign and affects commonly CN-VIII
• Accounts 8-10% intracranial tumors
• Sx: depend on nerve affected, in CN-VIII- sensoryneural hearing loss,
tinnitus and dysequilibrium (loss of balance, vertigo). CN-5,7 Sx.
• Commonly located at CPA, 95% unilateral, if bilat. (NF-2)
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57. • Dx- audiometry, CT, MRI, Biopsy
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58. • Treatment:
• Expectant management – with serial follow up(audiometry, MRI)
• RadioRx +/- surgery - for hearing preservation(EBRX, Stereotactic)
• Surgery- main stay of Rx for symptomatic (retrosigmoid, trans-
labyrinthine)
• Chemotherapy- has no clear benefit, (good for NF2-associated)
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59. 8. Metastatic brain tumor
• The most common intracranial tumors in adults, accounting up to 50% of brain
tumor
• It occurs in 10-30% of adults with ca.
• Common 10
for mets are Lung, breast, melanoma, kidney, colon...
• 80% is at cerebral hemispheres
• Sx- Headache, seizure, focal neurologic deficit, cognitive change, stroke.
• Dx- Imaging(MRI), biopsy:
• Multiple lesions, Localization at the junction of the gray and white matter,
Circumscribed margins, Large amounts of vasogenic edema
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60. Rx
• Rx - of cerebral edema with steroids, seizure with AED.
• High tumor burden –WBRT
• Low tumor burden, single mets. – Surgery +/- WBRT
• SRS – for smaller and surgically inaccessible tumors
• Multiple small tumors <3cm
• Surgery:
• for equivocal diagnosis and mets. Is uncertain.
• For low tumor burden and good performance status
• Single, accessible, large masses
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