patologi anatomi fk ub
TRANSCRIPT
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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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