SURGICAL PATHOLOGY: DISEASES OF THE CENTRAL NERVOUS SYSTEM III

Dr. Baby Lynne Asuncion | October 20-28, 2014

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DEMYELINATING/DEGENERATIVE DISORDERS
CASE

Free Printing by Jolo Belenski (Peace!)

A 72-year-old was complaining of difficulty of breathing. On examination, an apparent

tremor of his fingers was seen with expressionless face and monotonous voice. He died
after a day. On autopsy, a portion of the brain had a characteristic finding.
o Absence of substantia nigra: Parkinson's disease

Part of normal aging process: loss of dopaminergic responses subsequent

decrease in dopamine levels

Piso (di ko sure, baka dos) Printing ni Almond

DEMYELINATING DISEASES

Sawasdee Car and Motor accessory Shop with Free

installation
At Bonifacio St., Baguio City (Near Baguio Central University)

Floydie, natakot ka kay Ellen no? :P

May libreng kape pala sa Dragons Den.

Di po pala kami ung nagpapaabolish ng notes :P
Tsaka di ko din po sinabing magquiquit ako, joke lang un :P
NEVER din pong namin sinabing ABUSED kami >.<
Basahin nyo daw ung mga cases! Tsaka ung mga pictures na may yellow borders ung di exact sa
pinakita nya. Pero Robbins daw ireview nyo sa pictures, pero wala naman pictures masyado dun kaya
oh well.
Di man pala kami nakakareply sa mga comments nyo, you are most welcome
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MULTIPLE SCLEROSIS
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Oh Ella, eto na! Humane, eto na din! Ang aadik nyo :P

Hahaha, baka magalit ka kasi nasasayang ung space, pero rest ka muna! Chill lang.
Review ka din muna ng vulva, vagina, breast, menopause at cervix.
Ayaw ko itranslate to para sau Pri.

Acquired conditions: myelin damage and axonal preservations

Multiple sclerosis: MC

Others:

Neuromyelitis optica/Devic disease

Acute disseminated encephalomyelitis (ADEM)

Acute necrotizing hemorrhagic encephalomyelitis (ANHE)

Central pontine myelinolysis

Females more affected than males

No age predilection
Pathophysiology: Immune mediated response: targets the myelin sheath
demyelination

Optic nerve, brainstem and spinal cord

MC: SC

Common manifestation: paralysis of lower extremities

NEUROMYELITIS OPTICA/DEVIC DISEASE

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SALAMAT ATE JAIDI

Immune mediated: antibodies against aquaporin

Seen in optic nerve and spinal cord

ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM)

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Often follow a viral infection

One complication associated with postviral vaccination

ACUTE NECROTIZING HEMORRHAGIC ENCEPHALOMYELITIS (ANHE)

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Demyelinating symptomatology
Affect younger adults and children
Sequelae of viral infection particularly upper respiratory infection
More fatal outcome than ADEM

CENTRAL PONTINE MYELINOLYSIS (CPM)

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affect pons (MC): Loss of myelin

demyelination associated with severe electrolyte imbalance

SURGICAL PATHOLOGY: DISEASES OF THE CENTRAL NERVOUS SYSTEM III

Dr. Baby Lynne Asuncion | October 20-28, 2014

presents as quadriplegia

OTHER FINDINGS

MS PLAQUES
Multiple specks of gray to tan color
Irregular lesions: seen in demyelination

Luxol Fast Blue PAS Stain

Normal: blue color
Unmyelinated: patches of unstained areas

Neuritic plaque: spherical collections of neuritic

processes surrounding amyloid beta

Neurofibrillary tangle

Filaments extending (cone-shaped):

Accumulation of tau protein

Severe cognitive dysfunction

DEGENERATIVE DISEASE
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Diseases of grey matter

Progressive loss of neurons, secondary changes in white matter tracts
Resistance of degradation of protein aggregates

Tau protein: tauopathy

- Related to Alzheimers disease
- Protein noted in microtubule and associated with hyperphosphorylation
- Abnormal accumulation presents with cortical dementias
Groups

Symptomatic/anatomic: Cerebral, Basal ganglia, Brainstem, Spinocerebellum,

Motor Neurons
- Cerebral: Alzheimer
- Basal Ganglia & Brainstem: Parkinson & Huntingtons disease (AD; abnormal
protein: huntingtin)
- Spinocerebellum: Friedreich ataxia (MC inherited form of ataxia, early
onset, wheel-bound at age 20)
- Motor neurons: ALS

Pathologic
- Degenerative diseases that have inclusions/abnormal structures observed in
affected cells

ALZHEIMER DISEASE
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MC cause of dementia in elderly patients

65 years: incidence of 2% and doubles every 5 years

Insidious impairment of higher intellectual function with
mood and behavior alterations
5-10% hereditary: autosomal
dominant
R: Normal brain (healthy brain) vs Alzheimer
brain

L: Cortical atrophy: widened cerebral sulci in the

frontal, temporal and parietal lobes

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Granulovacuolar degeneration

Black staining granule within vacuole:

argyrophilic granule - silver stain

Hirano bodies

Eosinophilic bodies, elongated not

spherical

Glassy eosinophilic color

Found in pyramidal cells in hippocampus

Ronald Reagan died of severe pneumonia, a complication of

Alzheimer's.

SURGICAL PATHOLOGY: DISEASES OF THE CENTRAL NERVOUS SYSTEM III

Dr. Baby Lynne Asuncion | October 20-28, 2014

AMYOTROPHIC LATERAL SCLEROSIS (ALS)

PARKINSON DISEASE AND SYNDROME

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Loss of pigmented dopaminergic neurons

Manifestations:

Diminished facial expression

Rigidity and trembling of head

Forward tilt of trunk (stooping posture)

Slow voluntary movement

Reduced arm swinging

Rigidity and trembling of extremities

Shuffling gait with short steps (festinating)

Very characteristic: pill rolling

Disease: idiopathic
Syndrome: with causative factors like infection,
vascular condition, toxic insults particularly in the
substantia nigra

Amyotrophic: no muscle nourishment

Lateral: affected nerves (lateral area of SC or brain)
Sclerosis: diffuse fibrosis
Loss of LMN in SC and BS
Loss of UMN in CST
Missense mutation: gain of function
Males more than females
5th decade of life or even later
Progressive degeneration early onset of death
Motor neurons die inability of brain to initiate and
control muscle movements

Respiratory arrest

Paralysis
Asymmetric hand weakness, fasciculations, progressive
muscle atrophy
Very thinned out anterior nerve
roots (very characteristic of ALS)

Midbrain
L: Normal substantia nigra
R: Depigmented (pallor of
substantia nigra) Parkinsons
disease
Top: Lewy body
Round, pink inclusion: 2 parts
Inner area (dense core)
Outer area (pale halo)
Accumulation of alpha-synuclein

L: Spinal cord with unstained areas

Loss of myelinated fibers
associated with degeneration
R: Bunina bodies noted within the
neurons (Make use of PAS stain)

Why Ice bucket challenge?

o

Numbness of entire body (main manifestation of ALS): NUMB

ALL OVER

TREATMENT: Give DOPAMINE (LEVODOPA) FOR LIFE

o

Common in patients with multiple injury to head

Lou Gehrigs disease
Lou Gehrig, a famous baseball player for the New York
Yankees had this disease

Muhammad Ali diagnosed at age 42

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Michael J. Fox diagnosed at 30

SURGICAL PATHOLOGY: DISEASES OF THE CENTRAL NERVOUS SYSTEM III

Dr. Baby Lynne Asuncion | October 20-28, 2014

HUNTINGTON'S DISEASE (13!)

READ ON: one case daw dito
MULTIPLE SYSTEM ATROPHY (MSA)
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Presence of glial cytoplasmic inclusions, typically within the cytoplasm of

oligodendrocytes
Different patterns of clinical presentation
Dominant symptoms:

Parkinsonism (MSA-P: striatonigral degeneration)

Cerebellar dysfunction (MSA-C: olivopontocerebellar atrophy)

- Least frequently observed

Autonomic dysfunction (MSA-A: Shy-Drager Syndrome)

Gross Morphology:

Cerebellar forms: atrophy of cerebellum, including the cerebellar peduncles,

pons (basis pontis), medulla (inferior olive)

Parkinsonian: atrophy of substantia nigra and striatum (putamen)

Autonomic: usually no gross findings

Atrophic brain regions show evidence of neuronal loss and variable numbers of
neuronal cytoplasmic and nuclear inclusions
Microscopic:

Diagnostic glial cytoplasmic inclusions in oligodendrocytes with silver

impregnation

Contain alpha-synuclein, ubiquitin, alpha-beta-crystallin

Inclusions are ultrastructurally distinct from other neurodegenerative disease

Similar inclusions may also be found in the cytoplasm of neurons, neuronal and
glial nuclei, and in axons
Pathogenesis:

The relationship between glial cytoplasmic inclusions and disease is supported

by the observation that the inclusions are present in low numbers at earliest
stages of MSA and increase in abundance as the disease progresses, although
they eventually disappear as cells die in the final stages

Glial cytoplasmic inclusions can occur in the absence of neuronal loss, suggesting
that they may represent a primary pathologic event

Several studies: no up-regulation of alpha-synuclein expression

- Suggest protein is acquired secondarily by oligodendrocytes from injured or
dying neurons

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AD
Progressive movement disorders and dementia
Degeneration of striatal neurons
Jerky, hyperkinetic, sometimes dystonic movements involving all parts of the body
(chorea) are characteristic
May later develop parkinsonism with bradykinesia and rigidity
Relentlessly progressive, ave. course of about 15 years to death
Microscopic:

Small brain and shows striking atrophy of the caudate nucleus, and less
markedly at early stages, the putamen

Globus pallidus may be atrophied secondarily, and the lateral and 3rd ventricles
are dilated

Atrophy is frequently seen in the frontal lobe, less in parietal lobe and occasional
in the entire cortex

Severe loss of striatal neurons

- Most marked changes in caudate nucleus, especially in tail and portions
nearer the ventricle
- Putamen: later stages of disease
- Nucleus accumbens: best preserved portion of the striatum

Two populations of neurons spared:

- Diaphorase positive neurons that contain nitric oxide synthase
- Large cholinesterase positive neurons

Fibrillary gliosis that is more extensive than in the usual neuronal loss

Protein aggregates containing HUNTINGTIN can be found in the neurons in the

striatum and cerebral cortex
Direct relationship: degree of degeneration in the striatum and severity of symptoms
Pathogenesis:

Loss of medium spiny striatal neurons lead to dysregulation of the basal ganglia
circuitry that modulates motor input
- Normal function: dampen motor activity
- Degeneration results in increased motor output (choreoathetosis)

Cognitive changes probably related to neuronal loss from cerebral cortex

Huntingtin:
- Little evidence to suggest that the disease is caused by haploinsufficiency
related to a mutated allele
Clinical features:

Onset: MC in 4th and 5th decade

Related to length of CAG repeat in the HD gene

Motor symptoms often precede cognitive impairment

Movement disorder: choreiform, with increased and involuntary jerky

movements of all parts of the body
- Writhing movements of extremities are typical

Early symptoms of higher cortical dysfunction:

- Forgetfulness, thought and affective disorders, progression to a severe
dementia

Increased risk of suicide

Intercurrent infection: MC natural cause of death

SURGICAL PATHOLOGY: DISEASES OF THE CENTRAL NERVOUS SYSTEM III

Dr. Baby Lynne Asuncion | October 20-28, 2014

FREIDRICH ATAXIA

ATAXIA TELANGIECTASIA

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Spinocerebellar degeneration, AR progressive illness

Beginning in the 1st decade of life with gait ataxia, followed by hand clumsiness and
dysarthria
DTR are absent or depressed,

Extensor plantar reflex is typically present

Joint position and vibratory sense are impaired
Sometimes, loss of pain and temperature sensation and light touch
Most develop pes cavus and kyphoscoliosis
High incidence of cardiac arrhythmia and CHF
Concomitant diabetes found in 10% of patients
Most: wheel-bound within about 5 years of onset
Cause of death: intercurrent pulmonary infections and cardiac disease
Extremely low levels of FRATAXIN

Normally in inner mitochondrial membrane

Role in iron regulation

- Mutation: generalized mitochondrial dysfunction
Morphology:

SC shows loss of axons and gliosis in posterior columns, distal portions of CST,
and spinocerebellar tracts

Degeneration of neurons in SC (Clarke column), BS (CN nuclei VIII, X, XII),

cerebellum (dentate nucleus & Purkinje of superior vermis), Betz cells of motor
cortex

Decreased large dorsal root ganglion neurons

- Secondary degeneration of myelinated axons
Heart is enlarged and may have pericardial adhesions

Multifocal destruction of myocardial fibers with inflammation and fibrosis is

detectable in half of individuals in autopsy

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AR
Characterized by ataxic-dyskinetic syndrome beginning in childhood, with
subsequent development of telangiectasia in the conjunctiva and skin; and
immunodeficiency
ATM gene encodes a kinase with a critical role in orchestrating the cellular response
to double-stranded DNA breaks
Increased sensitivity to X-ray induced chromosome abnormalities

Cells continue to replicate the damaged DNA

Carrier: 1%

Mutated ATM allele may underlie an increased risk of cancer (breast Ca)
Mutation in ATM results in failure to remove cells with DNA damage from developing
nervous system, predisposing to degeneration
Morphology:

Predominantly in the cerebellum, with loss of Purkinje and granule cells

Degeneration of dorsal columns, spinocerebellar tracts, and anterior horn cells

Peripheral neuropathy

Telangiectatic lesions:
- In CNS, conjunctiva, skin of face, neck, arms

Cells in many organs show a bizarre enlargement of nucleus to 2-5x normal size
- Schwann cells on dorsal root ganglia and peripheral nerves, endothelial
cells, pituicytes
- Referred to as amphicytes

LN, thymus, gonads are hypoplastic

Clinical Features

Relentlessly progressive, with death early in second decade

Recurrent sinopulmonary infections and unsteadiness in walking

Speech becomes dysarthric

Eye movement abnormalities

Many develop lymphoid neoplasms, often T-cell leukemias, gliomas and

carcinomas

SURGICAL PATHOLOGY: DISEASES OF THE CENTRAL NERVOUS SYSTEM III

Dr. Baby Lynne Asuncion | October 20-28, 2014

METABOLIC DISEASES
CASE
A 62-year-old chronic alcoholic was seen
confused and wandering. He had peripheral
neuropathy, diplopia, nystagmus, and difficulty
in walking. He expired after few days to lobar
pneumonia. On autopsy, the brain showed this
finding
DIAGNOSIS: Wernicke's encephalopathy
o Thiamine deficiency, rapid

Small area with mamillary bodies punctate hemorrhages

Very characteristic finding

GENETIC METABOLIC DISEASES

o

Neuronal storage disease

Autosomal recessive: enzyme deficiency

- Tay-Sachs' disease
Leukodystrophies

Myelin abnormalities either in synthesis or turnover

- Progressive degeneration of myelinated white matter of the brain
- Metachromatic leukodystrophy: Krabbe's disease
Mitochondrial encephalomyopathies

Problem in the mitochondria

Disorders of oxidative phosphorylation

- MELAS: Mitochondrial Encephalomyopathy and lactic acidosis and stroke
like episodes

Manifest like stroke + progressive muscle weakness because of lactic

acidosis (encephalo: brain & myopathy: muscles)
- Ragged red fibers: associated with myoclonic epilepsy

Combined methotrexate and radiation-induced injury

Common in patients in chemotherapy

Manifests with drowsiness, confusion, ataxia

Necrosis of white matter: axonal degeneration and surrounding reactive gliosis

Radiation

High doses coagulative + liquefactive necrosis with subsequent edema

Neuronal loss, reactive gliosis altered mental status

Methanol

Occupational exposure (factories); usually analytic grade

Target retinal ganglion cells blindness

Focal necrosis of white matter

Left: Cerebellar degeneration in ethanol toxicity
Acute onset: reversible symptoms
Chronic: Wernicke Korsakoff encephalopathy

Atrophic: widening of cerebellar vermis

Common manifestation: ataxia, unsteady

gait, nystagmus
Right: Bergmann gliosis
Loss of Purkinje cells

No layering

Predominating small blue dots: glial cells

replacing Purkinje cells

Cyanocobalamin (B12) deficiency

Subacute combined degeneration of the

spinal cord

Degeneration: patches of unmyelinated areas

(Luxol Fast Blue PAS)

TOXIC AND ACQUIRED METABOLIC DISEASES

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Hypoglycemia or hyperglycemia

Same manifestation: altered consciousness

Hypoglycemia: target pyramidal neurons of cerebral cortex, Sommer section of

Hippocampus and Purkinje cells of Cerebellum
- Cells also commonly affected by hypoxia

Hyperglycemia: DKA or HONK

- MC manifestation: Severe cerebral edema
Hepatic encephalopathy

Accumulation of ammonia reactive gliosis and neuronal loss

Carbon Monoxide

Poor delivery of oxygen to the brain

Neuronal layer 3, 5 of cerebral cortex, Sommer section of hippocampus and

Purkinje cells of cerebellum (same cells affected by hypoxia)
- Carbon monoxide competes with oxygen in the blood

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Thiamine (B1) deficiency

Pinpoint hemorrhages and areas of necrosis in

mamillary bodies (near the hypothalamus)

Wernicke-Korsakoff encephalopathy

Memory disturbances, psychosis, eye problems

(diplopia or nystagmus)

Chronic alcoholism

SURGICAL PATHOLOGY: DISEASES OF THE CENTRAL NERVOUS SYSTEM III

Dr. Baby Lynne Asuncion | October 20-28, 2014

CNS TUMORS

MC NEOPLASMS
o

CASE
A 3-year-old boy was brought by his parents to a physician due
to loss of balance. His speech is more slurred now. He had a
wide-based gait. He was also seen bobbing his head. A midline
tumor was noted
Problem in balance: affectation of cerebellum
Microscopy:
o Small round blue cells (blastic), highly cellular neoplasm,
proliferation of small hyperchromatic cells
o See Homer-Wright rosettes
DIAGNOSIS: Medulloblastoma
o Commonly presents as cerebellar dysfunction
o Primitive cells tend to have the same features irregardless of location
o Associated with high incidence of hydrocephalus (push the pathways of CSF)

GENERALITIES
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Found intracranial, slow and subtle onset

Malignant > benign
15% SC and 85% brain
Primary malignancy > metastasis

Gliomas: MC primary malignancy

Adults

Astrocytoma

Glioblastoma [multiforme]
- MC primary intracranial malignancy in adults

Meningioma: 2nd MC
- 3rd MC: Schwannoma

Metastasis
Children

Astrocytoma

Medulloblastoma: MC

Both glioblastoma and medulloblastoma are aggressive tumors: diagnosed in

their late stages

STAGING OF CNS TUMORS

TUMOR SIZE STAGE
Most Important
T1
T2
T3
T4

Headache, seizures, mental changes, increased ICP symptoms, focal neurologic

symptoms
Depend on size: benign tumors can be fatal due to brain compression

LOCATION OF CNS TUMORS

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Tentorium cerebelli: extension of dura matter dividing cerebellum and inferior

portion of occipital lobe

Supratentorial: above the tentorium

- Commonly seen in tumors of adults
- Cerebrum, basal ganglia,
thalamus and hypothalamus

Infratentorial: below the tentorium

- Tumors of children
- Cerebellum, midbrain and
brainstem

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INFRATENTORIAL

</= 5 cm, unilateral

<= 3 cm, unilateral
>5 cm, unilateral
>3 c, unilateral
Invades the ventricular system
Crosses the midline/invades supra- or infratentorially

GRADING OF CNS TUMORS

Most important basis for prognosis and treatment
WHO Grading

MANIFESTATIONS
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SUPRATENTORIAL

GRADING
I
II
III
IV
M1

T1
IA
IIA
IIIA

FOUR BASES
o Nuclear atypia (pleomorphism)
o Mitotic activity
o Necrosis
o Vascular endothelial hyperplasia
o **If see 4 features: high-grade tumor

T2

T3
IB
IIB
IIIB

IV

T4

IV

SURGICAL PATHOLOGY: DISEASES OF THE CENTRAL NERVOUS SYSTEM III

Dr. Baby Lynne Asuncion | October 20-28, 2014

PROGNOSIS
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Correlates well with grading of tumor

Associated with response of patient with treatment protocol, particularly radiation
Consider age when tumor was diagnosed

GRADE
OVERALL PICTURE
<50 Y/O
>50 Y/O
I
50% survive
II
4 years
5.5 years
0.5 years
III
1.6 years
2.5 years
0.7 years
IV
0.7 years
1 year
0.5 years
***Grade III in > 50y/o: already symptomatic easy to diagnose immediate treatment
Grade I: Don't usually show symptoms
Grade II: Headache
Grade III: mental dysfunction
Grade IV: severe

HISTOLOGIC CLASSIFICATION
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Gliomas

Infiltrating fibrillary astrocytoma: largest group of gliomas

- Main presentation: based on size, location
- Prognosis: depends on WHO grading

Other glial tumors: oligodendroglioma, ependymoma, choroid plexus papilloma

Others

Neuronal tumors: ganglioma

Primitive tumors: medulloblastoma

Other tumors: meningioma, peripheral nerve tumors, metastatic tumors,

paraneoplastic syndrome
Treatment protocol: increase survival rate by 25%

Gliomas: 5 YSR

GBM: less than 5 YSR

DIFFUSE ASTROCYTOMA
o WHO Grade II
o Capacity to grow exponentially
o Subtle onset but could have progressive manifestation aggressive
o Grossly: poorly defined tumor

Similar to pilocytic astrocytoma: gelatinous surface

o Microscopically

Higher cellularity

Differentiation in sizes of fibrillary structures

Positive for GFAP: brown color

GEMISTOCYTIC ASTROCYTOMA
o WHO Grade III
o Gemistocytes: Astrocytes that have pleomorphic appearance
o Abundance of eosinophilic cytoplasm push nuclei towards one side
ANAPLASTIC ASTROCYTOMA
o WHO Grade III
o Difficult to differentiate with other astrocytoma
o More dense cellular pattern
o Variability of sizes and shapes of astrocytes
o Mitotic figures present

GLIOBLASTOMA [MULTIFORME]
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PILOCYTIC ASTROCYTOMA
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WHO Grade 1
Good prognosis
Benign behavior
Affects growing children and young adults
Picture

Cystic space, well-circumscribed

Cerebellum (MC), floor and walls of third ventricles, optic nerves and cerebrum

Like gelatin in consistency

Glial fibrillary acidic protein + (MC stain used to identify astrocytomas)

Eosinophilic granular bodies: characteristic of tumor

Pinkish Hair-like extensions: Rosenthal fibers

Treatment: Resection

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WHO Grade IV
High-grade tumor due to presence of necrosis

Hemorrhagic ill-defined area, necrotic center paler than periphery

Microscopically

Pseudopalisading pattern of tumor cells

Surrounds pinkish areas: necrotic areas

Increased in vascular endothelial proliferation (normal: single lined)

- See tufting of vascular supply: glomeruloid-like body

Presence of mitotic figures

Vascular proliferation

OLIGODENDROGLIOMA
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WHO Grade II/III

4th to 5th decade
Common location: white matter of cerebrum
Gross: Gelatinous surface, well circumscribed, often with cystic space

With associated hemorrhage, calcifications

Microscopically

Grade II: sheets of similar fried egg looking cells

- Very cellular: closely packed proliferating oligodendrocytes

Grade III: nuclear pleomorphism

- Denser, anaplastic, pleomorphic appearance,
- Prominence of vascular supply, proliferation of endothelial lining

SURGICAL PATHOLOGY: DISEASES OF THE CENTRAL NERVOUS SYSTEM III

Dr. Baby Lynne Asuncion | October 20-28, 2014

EPENDYMOMA

MEDULLOBLASTOMA

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WHO Grade II/III

MC intramedullary tumor (within the spinal cord)
Often noted at 1st decade of life (ventricles)
Adult onset (spinal cord)
Grossly

Warty projections: finger like papillary masses extended into ventricular surface
Microscopically

GRADE II
- Regular round cells surrounding a vascular channel: ependymal rosettes
- Pinkish area devoid of nucleus: fibrillary extension of ependymal cells
- Perivascular pseudorosettes

GRADE III
- Glandular structures
- Pseudorosettes
- Tubular acinar structures
- Increased cellularity
- Predominance of necrosis and mitosis
- Poorer prognosis

MYXOPAPILLARY EPENDYMOMA
o WHO Grade II/III
o Filum terminale of spinal cord
o Myoid background: see more of vacuolation
o Myxomatous change: mucinous background or slimy surface
o Cuboidal cells lining vascular channels interspersed vacuolated areas
o Form around the vascular channels
o Prognosis depends on grading

Young patient with hydrocephalus: 5YSR is 50%

CHOROID PLEXUS PAPILLOMA

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WHO Grade I
Children: lateral ventricle
Adult: fourth ventricle
Common manifestation: hydrocephalus due to obstruction of flow
Transformation to malignant tumor seen more in children than in adults
Papillary growth simulating normal choroid plexus
Microscopically: finger like projections

Papillae supported by very thin fibroconnective tissue

Lined by ependymal cells (cuboidal to columnar)

GANGLIOGLIOMA
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WHO Grade I
MC location: temporal lobe
Composed of irregular clusters of ganglion cells
Similar to astrocytoma

Difference: ganglioglioma has no mitosis, necrosis, positive reaction to

chromogranin or synaptophysin

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WHO Grade IV
MC CNS tumor arising from a primitive neuroepithelium
Mass seen near cerebellum

Children: midline location

Adult: lateral location

MC manifestation: hydrocephalus
Grade IV but radiosensitive
Microscopic:

Proliferation of small, round tumor cells: from primitive neuroepithelium

Very characteristic: tendency to form pseudorosettes called Homer-Wright

- Lumen: with glial processes

Flexner-Wintersteiner: in retinoblastoma
- Empty lumen

MENINGIOMA
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WHO Grade 1
MC benign tumor of adult
MC extramedullary tumor: outside the spinal cord but within the dura mater
Gross

Fixed in meninges

Well-circumscribed round mass

Compress adjacent brain tissue

Morphologic patterns

Whorling: Syncytial meningothelial

Calcified: Psammomatous/Psammoma bodies

- Starts syncytial then become calcified

No prognostic significance

ATYPICAL MENINGIOMA
o WHO Grade II
o Higher recurrence rate, more aggressive than the usual meningioma
o Radiation therapy
o No usual syncytial or psammomatous arrangement
o Increase cellularity, pleomorphism and mitosis noted
ANAPLASTIC MENINGIOMA
o WHO Grade III
o Highly aggressive tumor
o Course similar to sarcomatous lesion
o See multilayer of cells surrounding a vascular core
o Aka Papillary meningioma

SURGICAL PATHOLOGY: DISEASES OF THE CENTRAL NERVOUS SYSTEM III

Dr. Baby Lynne Asuncion | October 20-28, 2014

SCHWANNOMA
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WHO Grade 1
Neurilemmoma
Arising from cells derived from neural crest: Schwann cells
Located in cerebellopontine angle
Attached to CN VIII: Acoustic neuroma
Tendency to compress adjacent structure: brain stem and SC
Benign lesion with fatal outcome
Well-circumscribed lesion, encapsulated (very thin)

On cut section: cystic structure

Antoni A: cellular

Verocay bodies: absence in central area; palisading processes surrounding

central area
Antoni B: hypocellular

Gross
o Presence of multifocal/multiple lesions
Microscopic
o See vacuolated larger tumor cells: more of epithelial origin due to vacuolation

PRIMARY

METASTATIC

Poorly circumscribed
Usually single
Location varies and depend on specific
type

Well-circumscribed
Often multiple
Usually located at the junction between the
white and gray matter

Pictures to follow

NEUROFIBROMA
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WHO Grade 1
Discretely delineated mass
Seen on the skin or attached to a nerve
One component of Neurofibromatosis
Microscopic:

Snake-like/S-like cells that are wavy or eosinophilic

More compact appearance of spindle cells

Treatment: resection

Pagdating sa Patho:

Pagdating padin sa patho:

OTHER TUMORS
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o

Paraneoplastic syndromes

Immune response against tumor antigens that cross react with antigens in
CNS/PNS
Familial tumor syndromes

Usually AD
Metastatic tumors

NEUROFIBROMATOSIS TYPE I
o
o

Lisch nodule: marked melanin deposition in affected eye (iris)

Caf-au-lait spots: marked melanin deposition on skin

TUBEROUS SCLEROSIS
o
o

Mushroom like masses within the ventricular system

Manifests as hydrocephalus

METASTATIC TUMORS
o
o
o
o

25-50% of intracranial tumors

80%: Lungs, Breast (Lung: MC)
Others: melanoma, kidney, GIT, choriocarcinoma
50% extracranial: meninges (dura)

Extradural: site of mets

Page 10 of 10 | ad | j9 | kdrs | med 2016

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