Patologi Anatomi FK UB

8/13/2019 Patologi Anatomi FK UB

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Case

A 48 year old man has noticed speech

difficulties for 2 months. On physical

examination , he has weakness on the left side.

MR Imaging of the brain shows a large

irregular 6 cm mass in the centrum of the right

cerebral hemisphere.Biopsy of the mass shows

areas of necrosis surrounded by nuclearpseudopalisading.The neoplastic cells within

the mass are hyperchromatic.


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Which of the following neoplasms

is most likely to be present in this

patient

A. Medulloblastoma.

B. Glioblastoma multiforme. C. Metastatic lung carcinoma.

D.Malignant melanoma.

E. cystic astrocytoma.


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TUMORS OF THE

NERVOUS SYSTEM

Department of Anatomic Pathology

Medical Faculty

Brawijaya University


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CENTRAL NERVOUS SYSTEM TUMORS

Glioma

Neuronal Tumors( gangliocytoma, ganglioglioma)

Poorly diff. neoplasm ( medulloblastoma )

MeningiomaMetastatic Tumors

PERIPHERAL NERVE SHEATH TUMORS

Schwannoma

Neurofibroma

MPNST


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CENTRAL NERVOUS

SYSTEM TUMORS


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INCIDENCE

# Annual incidence :

- intracranial tumors : 10-17 /100.000

- intraspinal tumors : 1-2 / 100.000

# 50%-75% : primary tumor

# 20 % of cancer in childhood

# 70% childhood CNS tumor : posterior fossa

# Adult CNS tumor : >> cerebral hemisphere


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CHARACTERISTICS

Distinction between malignant and benign is

less evident.

The ability to surgically resecting is limited.

The anatomic site of tumor can have lethal

consequences ( ex : meningioma at the

medullacardioresp. arrest )

Pattern of spread primary CNS tumors differs

( rarely outside the CNS, CSF pathways)


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Major Classification

Glioma

Neuronal Tumors ( gangliocytoma,

ganglioglioma)

Poorly diff. neoplasm ( medulloblastoma )

Meningioma

Metastatic Tumors


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GLIOMA

Derived from glial cells.

Include :

- ASTROCYTOMA- OLIGODENDROGLIOMA

- EPENDYMOMA


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ASTROCYTOMA

Derived from astrocytes :

Fibrillary astrocytoma.

Glioblastoma. Pilocytic astrocytoma.

Pleomorphic xanthoastrocytoma.


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Fibrillary Astrocytoma

& Glioblastoma

80 % of adult primary brain tu.

>> cerebral hemisphere, cerebellum, brain stem,

spinal cord.

>> 4 - 6 decades.

>> signs and symptoms : seizures, headaches,focal neurologic deficits ( depend on location and

rate of growth, anaplastic features )


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WHO GRADING

Grading predicting prognosis and treatment

options.

WHO grading :

- Diffuse Fibrillary Astrocytoma

( Well diff Astrocytoma ) : Grade II/IV

- Anaplastic Astrocytoma : Grade III/IV- Glioblastoma : Grade IV/IV


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MACROSCOPIC :

Poorly defined, gray, infiltrative tumor.

Expands and distorts the invaded brain.

Range in size : few cm to enormous lesion .

Cut surface : firm or soft and gelatinous,

cystic degeneration ( +/- )

GLIOBLASTOMA :variation from region toregion : firm whitesoft yellow ( necrosis ),

hemorrhage, cystic.


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MICROSCOPIC :

Well Diff. Fibrillary Astrocytoma

Mild to moderate increase of glial cell nuclei

Variable nuclear pleomorphism. Background fibrillary appearance

( from astrocyte cell processes).

Transition between neoplastic and normaltissue indistinct


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Glioblastoma

Histologic appearance similar to anaplastic

astrocytoma with additional features :

- Necrosis.

- Vascular / endothelial cells proliferation

- Pseudopalisading.


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Molecular Genetics

Primary Glioblastoma : without pre existing

low grade tumor, in older patient.

- Associated with amplification of the

Epidermal Growth Factor Receptor Gene .

Secondary Glioblastoma : with a previously

diagnosed lower grade astrocytoma, inyounger patient.

- Associated with p 53 mutations


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Pilocytic Astrocytoma

( Low Grade Astrocytoma )

Typically occur in children and young adults.

>> cerebellum / floor & wall of third ventricle,

optic nerves, cerebral hemisphere Macros : > cystic w mural nodule / solid,

narrow infiltrative border.

Micros : bipolar cells w long thin hairlikeprocesses.

WHO grade I/IV


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Case

A 48 year old man has noticed speech

difficulties for 2 months. On physical

examination , he has weakness on the left side.

MR Imaging of the brain shows a largeirregular 6 cm mass in the centrum of the right

cerebral hemisphere.Biopsy of the mass shows

areas of necrosis surrounded by nuclearpseudopalisading.The neoplastic cells within

the mass are hyperchromatic.


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Which of the following neoplasms

is most likely to be present in this

patient A. Medulloblastoma.

B. Glioblastoma multiforme. C. Metastatic lung carcinoma.

D.Malignant melanoma.

E. cystic astrocytoma.


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Medulloblastoma

( Poorly Differentiated Neoplasm )

>> children ( 20% of brain tumor in children )

In the cerebellum ( > midline).

Highly malignant, radiosensitive tumor. Macros : well circumscribed, gray, friable.

Micros : small cells, hyperchromatic,

Homer Wright rosettes, >> mitoses. Molecular genetic : loss of gene form the short

arm of chromosome 17.


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Case

A 45 year old woman sees the physician

because she has had unilateral headache on the

right for the past 5 months. Physical

examination yields no remarkable findings.The lesion seen on CT scan of the head is

shown in this photograph of the brain .


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MENINGIOMA

Predominantly benign tumors of adult.

Arise from meningothelial cell of the

arachnoid.

Attached to the dura.

Along any external surfaces of the brain.


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Clinical Features

Slow growing, compression of underlying

brain.

Female predominance.

Usually solitary.

WHO grade is a strong predictor of clinical

course.

The presence of brain invasion associated w

increased risk of recurrence


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Macroscopic :

Rounded masses , well defined dural base,

compress brain, easily separated .

Extension to bone ( +/-).

Surface usually encapsulated w thin fibrous

tissue.

Firm and fibrous to finely gritty, or calcified w

psammoma bodies.

En plaque varhyperostotic reaction.


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WHO GRADING

Most meningioma = grade I/IV

- Syncytial Meningioma.

- Fibroblastic Meningioma.- Transitional Meningioma.

- Psammomatous Meningioma.

Atypical Meningioma = grade II/IV Anaplastic Meningioma = grade III/IV


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Microscopic :

Syncytial M : whorled cluster.

Fibroblastic M : elongated cells, >> collagen.

Transitional M : share feat of SM and FM.

Psammomatous M : >> psammoma bodies.

Atypical M :

= / > 4 mitoses/ 10 HPF, increased cellularity,

high N/C ratio, prominent nucleoli,

patternless growth or necrosis.


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Anaplastic Meningioma :

- highly aggressive tumor, has apperance of

high grade sarcoma.- > 20 mitoses / 10 HPF.

- certain histologic subtype ( papillary

meningioma and rhabdoid meningioma )considered to be WHO gr III/IV.


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METASTATIC TUMORS

Metastatic lesions mostly carcinoma : quarter

to half of intracranial tumor.

Five most common : lung, breast, skin

( melanoma), kidney, GIT .

Macroscopic :Sharply demarcated masses ,

>> at gray matter-white matter junction,

surrounded by a zone of edema.


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Case

A 10 year old boy has had persistentheadaches for the past 3 months. On physical

examination , he is afebrile and has ataxic gait.

CT scan of the head shows a 4 cm cystic mass

in the right cerebellar hemisphere.

Neurosurgery is performed and the mass is

removed and sectioned. On gross examination

the mass is cyst filled with gelatinous material.The cyst has a thin wall and 1 cm mural

nodule. Microscopically, the mass is composed

of cells which have long, hair like processes.

Whi h f th t lik l


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Which of the most likely

diagnosis ?

A. Astrocytoma.

B.Medulloblastoma.

C. Meningioma.

D.Metastatic carcinoma.

E. Schwannoma


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Eight months later , he return s for a follow up

examination , and a mass is palpated on the

right wrist.

Histologic examination of the mass is most

likely to show which of the following

neoplasms ?


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PERIPHERAL NERVE

SHEATH TUMOR


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Peripheral Nerve Sheath Tumor

Arise from cells of the peripheral nerve :

- Schwann cells, perineural cells, fibroblast

Classification :

1. SCHWANNOMA.

2. NEUROFIBROMA.3. MPNST


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SCHWANNOMA

Arised from neural crest derived schwann cell

Assoc w Neurofibromatosis 2.

Symptoms assoc w local compression of nerve

/ brain stem / spinal cord

Location at cerebellopontine angle ( attached

to N VIII = Acoustic Neuroma / vestibular

schwannoma )tinnitus, hearing loss


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MACROSCOPIC :Well circumscribed, encapsulated masses

attached to the nerve, can be separated,

firm, gray, may also cystic .


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MICROSCOPIC :ANTONI A :

elongated cells arranged in fascicles in areas

moderate to high cellularity,

verocay bodies (+) , nuclear palisading

ANTONI B :

Less densely cellularity,loose meshwork of cells along with microcyst,

and myxoid changes.


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NEUROFIBROMA

Classif : - Cutaneous neurofibroma >>

- Solitary neurofibroma ( peripheral nerve)

Sporadically or associated w neurofibromatosis 1.

1. Cutaneous Neurofibroma

- nodules, overlying hyperpigmentation,may large & pedunculated, malignant transform


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2.Plexiform Neurofibroma :

Occur in patient w NF type 1.

Difficult to surgical removal when involvemajor nerve trunk.

Significant potential for malignant

transformation


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Morphology

CUTANEOUS NEUROFIBROMA

In the dermis and subcutaneous fat.

Well delineated but unencapsulated masses. Spindle cells in the highly collagenized

stroma.

Adnexal structures enwrapped


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PLEXIFORM NEUROFIBROMA : >> large nerve trunk, >> multiple.

The nerve is irregularly expanded, infiltrated byneopl, not possible to separate.

Micros : loose myxoid background,

low cellularity, areas of shredded carrotappearance.

Axons can be within tumor


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MORPHOLOGY

Poorly defined tumor, infiltration along nerve

axis and soft tissue.

Necrosis (+).

MICROS :

Pattern reminiscent fibrosarcoma / MFH,

areas resemble schwann cells ( elongatednuclei ), necrosis, mitoses, nuclear anaplasia,

S100 protein (+).


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Case

A 18 year old male has had decreased vision

on the left for 6 months. On physical

examination , there is papiledema on the right.

He has 14 scattered 2-5 cm flathyperpigmented skin lesions with irregular

borders on the extremities and torso. Ct scan

shows a mass in the region of the right opticnerve. Histopathology examination shows

proliferation of glial cells with moderate

atypia.


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Eight months later , she return s for a follow up

examination , and a mass is palpated on the

right wrist.

Histologic examination of the mass is mostlikely to show which of the following

neoplasms ?


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A. Lipoma.

B. Fibrosarcoma.

C. Meningioma.

D. Hemangioma.

E. Neurofibroma.


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