Topic: Brain and Spine Tumors

Lecturer: Dr. Gayao

Lecture: Brain and Spine Tumors
Lecturer: Dr Gayao

Outline BRAIN TUMORS

Introduction
Incidence Could be characterized/described in several ways:
Pathophysiology 1. Clinically the neoplasmic mass because of
Classification system its size or location could cause symptoms of mass
Brain Tumors effect and neurologic deficits
Type
Clinical manifestations 2. Tissue according to the group of cells and
Diagnostic tools associated extracellular matrix whose growth are
Management/Treatment Guidelines uncoordinated and exceeds that of normal tissue.
Spine Tumors
Type Grade I: circumscribed, biphasic, bipolar and
Clinical manifestations multipolar cells, fibers, microcysts, granular
Diagnostic tools bodies; no/rare mitotic figures, no/rare vascular
Management/Treatment Guidelines proliferation, no/focal necrosis
Grade II: moderately hypercellular, monotonous
cells, mild nuclear atypia, no/minimal mitotic
Brain tumors could be broadly categorized into 2
activity
groups
Primary Brain Tumors tumors that arise
Grade III: increased cellularity and diffuse
from cells of the brain parenchyma
infiltration, increased nuclear atypia, increased
Secondary Brain Tumors tumors that
mitotic activity
spread to the brain
Grade IV: vascular proliferation, necrosis,
Primary brain tumors
crowded anaplastic cells, marked nuclear atypia,
brisk mitotic activity

3. Cellular individual cells differ from normal

cells in size, shape, pigmentation, nuclear size.

As tumors become more malignant, features of

cellular anaplasia becomes more pronounced

4. Biochemical Rapidly growing tumor cells may

** intra-axial: within the brain parenchyma
extra-axial: outside the brain parenchyma
Secondary Brain Tumors
rely more heavily on the glycolytic than on
oxidative metabolism
5. Immunologic Cell surface markers, many of
which contribute to the altered growth
characteristics of tumor cells

e.g. receptors for growth factor receptors and

extracellular matrix protein

6. Chromosomal Loss or gain of entire

chromosomes, deletions, rearrangement or
duplication of parts of the chromosomes

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 7. Genetic alteration in DNA base sequence that
changes either the expression of genes or the What host factors allow these genetic alterations to
structure of the proteins the genes encode be manifested as tumors?
Tumor heterogeneity
Pathophysiology Heterogenous tumor cells constitute a
substrate for selection of progressively more
Origin of brain tumors is essentially a genetic malgnant tumor, rapidly proliferating with
question consisting of 3 parts: greater genetic lability
Invasiveness
1. What fundamental genetic alterations Angiogenesis
underlie the development of the tumor? Immune suppression
2. What causes these genetic alterations?
3. What host factors allow these genetic Brain Tumors Incidence
alterations to be manifested as tumors? CBTRUS: (Central Brain Tumor Registry of the
United States)
Genetic alterations underlie the development
Primary Brain and CNS 20.6 / 100,000
of the tumor Tumors

Malignant 7.3 / 100,000

Non-malignant 13.3 / 100,000

Females 22.3 / 100,000

Males 18.8 / 100,000

US Population

SEER (Surveillance, Epidemiology, and End

Results) program
Primary MALIGNANT 6.5 / 100,000
Brain and CNS Tumors

Males 7.7 / 100,000

Females 5.4 / 100,000

Primary NON 6.5 / 100,000

MALIGNANT Brain
and CNS Tumors

Males 10.6 / 100,000

Females 16.1 / 100,000

US Population
Age distribution of CNS Tumors

What causes these genetic alterations?

1. Radiation
2. Chemicals
3. Viral

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 Survival based on WHO grading
Brain tumor by histologic type (take note)
Grade I Curative when gross total excision is done

Grade II Survival of > 5 years

Grade III Survival of 2-3 years

Grade IV Survival ~ 1 year

WHO Classification of CNS Tumors

Tumors of Neuroepithelial Tissue
Tumors of cranial and paraspinal nerves
Gliomas by histologic type Tumors of the Meninges
Lymphomas and Hemopoietic Neoplasms
Germ Cell Tumors
Tumors of the Sellar Region
Metastatic Tumors

TUMORS OF NEUROEPITHELIAL TISSUE

Astrocytic tumors
Pilocytic astrocytoma
Subependyml giant cell tumors
Pleomorphic xanthoastrocytoma
Diffuse astrocytoma
Fibrillary
WHO grading of tumors Gemistocytic
Protoplasmic
Grade I Tumors of low proliferative potential Anaplastic astrocytoma
Possibility of cure following surgical
Glioblastoma
resection alone
Oligodendroglial tumors
Grade II Generally infiltrative in nature Oligodendroglioma
Despite low proliferative nature, may tend to Anaplastic Oligodendroglioma
recur
Some tend to progress to higher grade Oligoastrocytic tumors
Oligoastrocytoma
Grade III Generally reserved for lesions with Anaplastic Oligoastrocytoma
histological evidence of malignancy (atypia
and mitotic activity)
Tumors of Neuroepithelial Tissue
Patients receive adjuvant radiation and/or Ependymal tumors
chemotherapy Subependymoma
Myxopapillary ependymoma
Grade IV Cytologically malignant
Mitotically active, necrosis prone neoplasm Ependymoma
Rapid post operative disease evolution Cellular
Fatal outcome Papillary
Clear Cell
Tancytic
Anaplastic ependymoma
Choroid plexus tumors
Choroid plexus papilloma
Choroid plexus carcinoma
Neuronal and Mixed neuronal-glial tumors
DNET

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 Gangliocytoma Diffuse Astrocytoma
Central neurocytoma AKA Low Grade Diffuse Astrocytoma
Paraganglioma WHO grade II
Tumors of Neuroepithelial Tissue 3 cell types
Tumors of the Pineal region Fibrillary (most common)
Pineocytoma Gemistocytic (prone to progress to high
Pineal parenchymal tumor grade)
Pineoblastoma Protoplasmic
Embryonal tumors Predilection for temporal, posterior frontal and
Medulloblastoma anterior parietal lobe
PNET
Atypical teratoid / rhabdoid tumor Clinical presentation
Most commonly seizures
Pilocytic Astrocytoma Headache
WHO grade I Imaging: Tumor does not significantly enhance
subgroup of astrocytomas with better with contrast
prognosis (10 year survival: 94%) than Genetic markers
infiltrating astrocytoma Loss of heterozygosity on chromosome 10
Age < 20yo in 75% & 17
Common locations: Alteration of tumor suppressor gene at 9p,
Cerebellar hemisphere 13q, 19q and 22q
Optic nerve/chiasm Transformation of the p53 gene
hypothalamus Dedifferentiation: Major cause of morbidity
Radiologic: discrete appearing, contrast with low grade astrocytoma is dedifferentiation
enhancing lesion, often with cystic with mural to a more malignant grade
nodule Once dedifferentiation occurs, median survival
AKA: is 2-3yrs
Cystic cerebellar astrocytoma Treatment
Juvenile pilocytic astrocytoma Surgery although tumor is low grade,
Optic gliomas if it occurs on optic nerve many opt to do surgery because of risk of
Hypothalamic gliomas progression to higher grade
Treatment Radiation increases progression free
Cerebellar PCA survival but no effect on overall survival
Maximal surgical excision of the tumor Chemotherapy
without producing deficit Observation
Excision of the nodule is sufficient, cyst wall
is non neoplastic
Optic Glioma
Optic nerve sparing chiasms excision
If chiasm is involve biopsy, chemotherapy
or radiotherapy

Lesion at the optic chiasm Glioblastoma

AKA: Glioblastoma multiforme (GBM)
WHO grade IV
Most common primary brain tumor, it is also
the most malignant

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 Primary GBM arise without evidence of less
malignant precursors
Secondary GBM develop by malignant
degeneration
Imaging:
The enhancing ring is cellular tumor.
The non enhancing center may represent
necrosis or cyst.
Tumor cells are present >15mm beyond
the ring
Spread via
Tracking through white matter
Corpus callosum butterfly glioma
CSF pathways Choroid Plexus Papilloma
Treatment WHO grade I
Surgery Location:
Radiation Children: Supratentorial
Chemotherapy Adults: Infratentorial
* Even with maximal treatment median survival is Presentation:
1 yr Symptoms of increased ICP
Headache
Nausea/Vomiting
Craniomegally
Treatment
Surgery
Irregular lesion Chemotherapy and Radiation has no role

Oligodendroglioma
WHO grade II or III
Frequently present with seizures
Predilection for the frontal lobes
Histology: classic fried egg cytoplasm and
chicken wire vascularity
Imaging: Calcifications are common Medulloblastoma
Treatment WHO grade IV
Surgery A small cell embryonal tumor of the cerebellum
Chemotherapy for all found predominantly in children
Radiotherapy for anaplastic trasformation Arises in the cerebellar vermis in the region of
Prognosis the apex of the roof 4th ventricle
10 yr survival 10-30% Compromises 30-55% of posterior fossa tumors.
Most common malignant pediatric brain tumor
Presentation:
Headache
Nausea/vomiting

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 Ataxia Most frequently in middle aged and elderly
Irritability, lethargy and macrocrania in adults with a predilection for females (F:M 1.5 -
infants 2 : 1)
Imaging: Clinical presentation depends on the size of the
Cranial CT or MRI tumor, the nerve of origin and the exact location
Spinal MRI to check for drop metastasis Intracranial Schwannoma
Treatment Trigeminal schwannoma
Surgery debulk as much as possible Facial Nerve
without causing neurologic injury Vestibular schwannoma (most common)
Radiotherapy Craniospinal. MB are Vestibular schwannoma
highly radiosensitive Arises from the superior vestiular nerve at
Chemotherapy MB are moderately the Obersteiner-Redlich zone
chemosensitive Arise as a result of loss of a tumor
Prognosis suppressor gene at the long arm of
Poor prognosticators chromosome 22
Age < 3yo If present bilaterally it is associated with
Drop metastasis Neurofibromatosis type 2
Tumor residual > 1.5cm2 Presenting symptoms
Hearing loss
Ependymoma Tinnitus
WHO grade II Dysequilibrium
Arise from ependymal cells lining the cerebral Evaluation
ventricles and central canal of the spinal cord Cranial MRI
Most often occur in the floor of the 4th ventricle Pure tone audiogram
Ependymoma Speech discrimination score
Presentation: Treatment
Increase ICP Headache, N/V, ataxia Expectant management
CN VI and VII palsies Radiation therapy
Imaging Surgery
Cranio-spinal MRI Chemotherapy
Treatment
Gross total removal TUMORS OF THE MENINGES
Radiotherapy on tumor bed, cranio-spinal if Lymphomas and Hemopoietic Neoplasms
with drop mets Germ Cell Tumors
Chemotherapy limited role Tumors of the Sellar Region
Metastatic Tumors
TUMORS OF CRANIAL AND PARASPINAL Tumors of the Meninges
NERVES Tumors of meningothelial cells
Schwannoma Meningioma
Cellular Mesencymal tumors
Plexiform Lipoma
Melanotic Angiolipoma
Neurofibroma Leiomyoma
Plexiform Osteoma
Perineurioma Osteosarcoma
Malignant peripheral nerve sheath tumor Hemangioma
Hemangiopericytoma
Schwannoma Primary Melanotic lesions
WHO grade I Diffuse melanocytosis
Melanocytoma
Malignant melanoma

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 Meningioma
Grade Degree of Removal
WHO grade I, II, or III depending on subtype
Slow growing extra axial tumor arising from the I Gross total excision of tumor and dural
arachnoid cap cells attachment
2nd most common primary intracranial tumor II Gross total excision of tumor and
Location: coagulation of the dural attachment

III Gross total excision, without coagulation of

attachment

IV Partial excision of tumor

V Biopsy

LYMPHOMAS AND HEMOPOIETIC

NEOPLASMS
Malignant lymphomas
Plasmacytoma
Granulocytic sarcoma
Parasagittal CNS Lymphoma
Convexity May be primary or secondary
Tubercullum sella Ssuspected with homogenously enhancing
Sphenoid ridge lesions in the central grey matter, corpus
Olfactory groove callosum, periventricular
Falx Presenting symptoms
Lateral ventricle Mental status changes
Tentorial Symptoms of inc ICP
Spinal Generalized seizures
Presenting symptom would depend on the Diagnostics
location of the tumor MRI no pathognomonic feature. Difficult to
Imaging: determine if tumor is subependymal
Cranial CT scan with contrast Ghost tumor disappears when steroids
Cranial MRI with contrast are given
Cerebral angiogram CSF studies elevated protein and cell
Spinal MRI for spinal meningioma count, positive cytology in 10%
Treatment: Treatment
Surgery Surgery for biopsy purposes only
Radiotherapy for atypical and anaplastic Radiotherapy Whole brain but lower dose
subtypes compared to other primary CNS tumors
Recurrence in 10 years Chemotherapy
Simpson I excision 8% Methotrexate
Simpson II excision 18% Prognosis
Simpson III excision 28% No treatment 1.8-3.3 months
RT median survival 10 mos
Simpson Grading of Meningioma Excision Chemo median survival of 41 mos

GERM CELL TUMORS

Germinoma
Embryonal carcinoma
Yolk sac tumor
Choriocarcinoma

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 Teratoma
Mixed germ cell tumor TUMORS OF SELLAR REGION
Craniopharyngioma
Pineal Region Neoplasm - Adamantinomatous
Tumors in this region are more common in - Papillary
children Granular cell tumour
Pineal Tumors Pituicytoma
Pineal cell tumors Spindle cell oncocytoma of the adenophypophysis
Pineocytoma
Pineoblastoma Pituitary Tumors
Germ cell tumors Most are benign adenomas arising from the
Germinoma anterior pituitary (adenophypophysis)
Non Germinomatous germ cell Neurohypophyseal tumors are rare
tumor (NGGCT Microadenoma if tumor is < 1cm
Embryonal carcinoma Macroadenoma if tumor > 1cm
Choriocarcinoma May be classified by endocrine function
Endodermal sinus tumor Prolactin secreting
teratoma Thyroid hormone secreting
Diagnostic Growth hormone secreting
Tumor markers Cortisol secreting
B-HCG choriocarcinoma Presentation
Alpha fetoprotein (AFP) endodermal Endocrine disturbance
sinus tumors Mass effect
Placental alkaline phospahatase (PLAP) Optic chiasm bitemporal
germinoma hemianopsia
Germinoma based on Japanese (43-70%) and 3rd ventricle Obstructive
European (21-44%) Studies is the most hydrocepahlus
common tumor in this region Caverous sinus Cranial nerve
Presentation: palsy
hydrocephalus Pituitary Apoplexy
Nausea/vomiting Due to sudden expansion of
Parinauds syndrome adenoma due to hemorrhage or
Precocious puberty necrosis
Management: Surgical emergency
- Surgery Give glucocorticoid
- Biopsy immediately
- CSF diversion Diagnostics:
- Trial Radiotherapy??? Formal visual fields
Endocrine screening
Cortisol
TSH, T4
Prolactin
FSH, LH
IGF-1
Imaging Cranial MRI

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 Fewer number of Mets
Prolactin Interpretation Situation Female gender
(ng/ml)
Sources of Brain Mets
3-30 Normal Normal physiologic
Primary %
25-150 Mod elevated - Prolactinoma
- Stalk effect
Lung 44
> 150 Markedly Prolactinoma
elevated
Breast 10

Management Kidney 7
Prolactinoma - may opt medical treatment
using bromocriptine GI 6
Growth hormone secreting may opt
medical treatment using octreotide
Melanoma 3
Surgery
Radiotherapy
Undetermined 10

SPINE TUMORS
Could be classified into 3 groups
Group % Description

Extradural (ED) 55 Outside the cord, in

% vertebral bodies and
epidural tissues

Brain Metastasis Intradural 40 Arise in

Most common brain tumor seen clinically Extramedullary % leptomeninges or
(IDEM) roots
They compromise more than half of brain
tumors Intradural 5 Arise in spinal cord
Route of metastatic spread to the brain is Intramedullary (ID- % substance. Invade and
usually hematogenous, although local extension IM) destroys tracts and
grey matter
do occur
Imaging Solitary Mets
CT: at time of diagnosis 50% are solitary EXTRADURAL
on CT A. Metastatic compromises majority of ED
MRI: same patient have an MRI, <30% tumors
will be solitary B. Primary spinal cord tumors very rare
Treatment 1. Chordomas
Surgery 2. Osteoma
Stereotactic radiosurgery 3. Osteoblastoma
Whole brain RT 4. Aneurysmal bone cyst
Chemotherapy 5. Vertebral hemangioma
Brain Metastasis
Factors associated with better prognosis Spinal Epidural Metastases
Karnofsky score >70 Suspected in a cancer patient with back pain that
Age < 60 yo persist in recumbency
Metastasis to brain only ( no systemic 80% of primary site: Lung, breast, prostate, GI,
mets) lymphoma
Controlled primary Routes of Metastasis
Arterial

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 Venous via Batsons plexus 15% cervical
perinervous 2% lumbar
Presentation Recurrence rate with complete excision 7%
Pain
local pain at level of involvement, Spinal Schwannoma
increased with recumbency Slow growing benign tumors
Radicular shooting pain at Most (75%) arise from the dorsal (sensory)
dermatome of involved nerve rootlets
Mechanical exacerbated by Early symptoms are often radicular
movement Recurrence is rare after total excision
Motor dysfunction
Sensory dysfunction INTRADURAL INTRAMEDULLARY
Spinal epidural metastases A. Astrocytoma 30%
Treatment B. Ependymoma 30%
Surgery to reduce pain, stabilize and preserve C. Misc 30%
ambulation
Surgery not recommended for prognosis <3-4 Spinal Ependymoma
months survival, poor medical condition Slight male predominance
Radiotherapy Peak in 3rd to 6th decade
50% occurs in filum, next is cervical
INTRADURAL EXTRAMEDULLARY It is the most common glioma of the lower
A. Meningomas cord, conus and filum
B. Neurofibromas/Schwanomas Usually Myxopapillary subtype (WHO gr I)
C. Lipomas Treatment excision (most are encapsulated)
D. Misc.

Spinal Meningioma
Peak age: 40-70yo
Female:male ratio 4:1 NOTE: memorize the pie graph, simpson grading
90% completely intradural and the tumors of the pineal tumor. Know the
Location grading of the tumors and read on parinaud's
82% thoracic syndrome.

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