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Pathology > Neuropathology > Flashcards

Neuropathology Flashcards

1
Q

Acute Neuronal Injury (red neuron)

A

Acute CNS hypoxia or ischemia or acute insults reflect cell death, necrosis or apoptosis
- shrink cell body, pyknosis of nucleus, disappearances of nucleolus, loss of Nissl, eosinophilia of cytoplasm

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2
Q

Subacute and chronic neuronal injury

A

Cell loss with reactive gliosis

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3
Q

Axonal reaction

A

Increased protein synthesis associated with axonal sprouting

- enlargement and rounding of cell body, peripheral placement of nucleus, nucleolus enlargement, dispersion of Nissl

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4
Q

Rosenthal fibers

A

Thick, elongated, brightly eosinophilic, irregular structures in astrocytes
- found in gliosis and glial tumor –> pilocytic astrocytoma

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5
Q

Corpora amylacea

A

Basophilic, PAS lamellated structures with astrocytic end processes in subpial and perivascular zones
- degenerative change in astrocyte

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6
Q

Alzheimer Type II astrocytes

A

gray matter cell with large nucleus, pale chromatin, intranuclear glycogen droplet, nuclear membrane and nucleolus
- hyperammonemia from chronic liver disease, wilson disease, metabolic disorders hereditary of urea cycle

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7
Q

Ependymal granulations

A

Inflammation or dilation of ventricular system leads to small irregularities in ventricular surfaces
- ex: CMV

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8
Q

Microglial response to injury

A

Steps:

  1. proliferative
  2. develop elongated rod cells
  3. form aggregates about small foci of tissue necrosis (microglial nodules)
  4. congregating around cell bodies of dying neurons (neuronophagia)
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9
Q

Vasogenic edema

A

blood brain barrier disruption with increased vascular permeability
- fluid to shift from intravascular to intercellular spaces of brain

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10
Q

Cytotoxic edema

A

increase in intracellular fluid secondary to neuronal, glial, endothelial cell membrane injury

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11
Q

Interstitial edema

A

increase in intravascular pressure causing abnormal flow of fluid from intraventircular CSF

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12
Q

Noncommunicating hydrocephalus

A

portion of ventricular system enlarged from excess CSF i.e. mass in third ventricle

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13
Q

Communicating hydrocephalus

A

Enlargement of entire ventricular system

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14
Q

Hydrocephalus ex vacuo

A

Dilation of ventricular system with compensatory increase in CSF volume secondary to loss of brain parenchyma

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15
Q

Hydrocephalus in infants and children

A

enlargement of head and increased head circumference

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16
Q

Subfalcine herniation

A

unilateral or asymmetric expansion of cerebral hemisphere displaces cingulate gyrus under falx cerebri –> can compress ACA

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17
Q

.Transtentorial herniation

A

Medial aspect of temporal lobe compressed against tentorium –> can compromise cranial nerve three or PCA

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18
Q

Tonsillar herniation

A

Displacement of cerebellar tonsils through foramen magnum –> brainstem compression and compromises viral respiratory and cardiac centers in medulla oblongata

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19
Q

Spina Bifida Occulta

A

Failure of closure or reopening of caudal neural tube –> asymptomatic bony defect

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20
Q

Meningocele

A

Extension of meningeal extrusion

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21
Q

Myelomeningocele

A

Extension of CNS tissue through a defect in vertebral column in lumbosacral region
- motor and sensory disturbances of bowel and bladder control

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22
Q

Encephalocele

A

Diverticulum of malformed CNS tissue through a defect in cranium –> occipital region or posterior fossa

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23
Q

Anencephaly

A

Malformation of anterior end of neural tube with absence of brain and calvarium

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24
Q

Lissencephaly

A

Absence of gyri, leads to a smooth surfaced brain

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25
Q

Polymicrogyria

A

Small unusually numerous and irregular cerebral convolutions

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26
Q

Neuronal heterotopias

A

Migrational disorders associated with epilepsy –> collections of neurons in inappropriate locations along migrational pathways

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27
Q

Holoprosencephaly

A

Spectrum of malformations from incomplete separation of cerebral hemispheres across midline

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28
Q

Agenesis of corpus callosum

A

Absence of white matter bundles with misshapen lateral ventricles
- associated with mental retardation or normal

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29
Q

Dandy-walker Malformation

A

Enlarged posterior fossa, cerebellar vermis absent, instead midline cyst is there

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30
Q

Arnold Chiari Type II Malformation

A

Small posterior fossa, misshapen cerebellum with downward vermis through foramen magnum, hydrocephalus, lumbar myelomeningocele
- caudal displacement of medulla, malformation of tectum, aqueductal stenosis, cerebral heterotopias, hydromyelia

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31
Q

Syringomyelia

A

Formation of fluid-filled cleftlike cavity in inner portion of cord which may extend into brainstem
- isolated loss of pain and temperature in upper extremities

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32
Q

Hydromyelia

A

Expansion of ependyma lined central canal of cord

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33
Q

Cerebral Palsy

A

Nonprogressive neurologic motor deficit from spasticity, dystonia, ataxia/athetosis, paresis

  • increased risk of intraparenchymal hemorrhage in germinal matrix
  • infarcts in supratentorial periventricular white matter (periventricular leukomalacia)
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34
Q

Concussion

A

Clinical syndrome of altered consciousness secondary to head injury from change in momentum of head

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35
Q

Epidural hematoma

A

Middle meningeal artery vulnerable to injury with temporal skull fractures with extravasation of blood under arterial pressure causing dura to separate from inner surface of skull

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36
Q

Subdural hematoma

A

Inner surface of dura and outer arachnoid layer from bridging veins

  • acute –> fresh clotted blood on brain surface, underlying brain flattened
  • chronic –> episodes of repeat bleeding
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37
Q

Border zone (watershed) infarct

A

Occur at regions of brain distal of arterial blood supply

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38
Q

Majority of Thrombotic Occlusions

A

Due to atherosclerosis

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39
Q

Embolism

A

Paradoxical emboli from tumor, fat, or air –> cardiac surgery/ cardiac anomalies

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40
Q

Infectious vasculitis

A

small and large vessels associated with syphilis and tuberculosis –> immunosuppression

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41
Q

Hemorrhagic (red) infarction

A

mutliple, sometimes confluent, petechial hemorrhages associated with embolic events

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42
Q

Nonhemorrhagic infarction

A

Pale, bland, anemic associated with thrombosis

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43
Q

Lacunar infarct

A

CNS arterial lesion from HTN arteriolar sclerosis as single or multiple, small, cavitary infarcts
- occurs in lenticular nucleus and pons

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44
Q

Acute hypertensive hemorrhage

A

malignant hypertension can lead to diffuse cerebral dysfunction
- sx: headache, confusion, vomiting, convulsions, coma

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45
Q

Charcot-Bouchard microaneurysms

A

Arteriolar walls affected by hyaline change in chronic hypertension

46
Q

Saccular aneurysm

A

Can cause SAH, most common aneurysm

  • found near major arterial branch points in anterior circulation
  • increased incidence in polycystic kidney disease, Ehlers-Danlos type IV, neurofibromatosis type I, Marfan syndrome, coarctation of aorta
  • cigarette and HTN predisposing
47
Q

Arteriovenous malformations

A

Tangled network of wormlike vascular channels with prominent pulsatile arteriovenous shunting

  • involve vessels in subarachnoid space
  • increased risk of hemorrhage, most common
  • males 2 x greater than females
48
Q

Cavernous hemangioma

A

loosely organized vascular channels with thin collagen walls without nervous tissues

  • occur in cerebellum, pons, subcortical regions
  • increased risk of hemorrhage
49
Q

Capillary telangiectasias

A

Microscopic foci of dilated, thin-walled vascular channels separated by normal brain parenchyma
- occur most frequently in pons

50
Q

Foix-Alajouanine disease

A

venous angiomatous malformation of spinal cord and overlying meninges

  • most often in lumbosacral region
  • ischemic myelomalacia and slowly progressive neurologic symptoms
51
Q

Bacterial meningitis

A

Neonates: E. coli and Group B strep
Adolescents: Neisseria meningitidis, Haemophilus influenzae
Old age: Strep pneumonia, Listeria monocytogenes
Sx: headache, photophobia, irritability, clouding of consciousness, neck stiffness
Spinal tap: purulent CSF, increased pressure, increased neutrophils, increased protein, decreased glucose

52
Q

Viral meningitis

A

Sx: headache, photophobia, irritability, clouding of consciousness, neck stiffness
Spinal tap: moderate elevated protein, normal glucose, sterile, pleocytosis with neutrophils

53
Q

Brain abscess

A

Direct implantation (mastoiditis, paranasal sinusitis), hematogenous spread

  • causes: acute bacterial endocarditis, congenital heart disease, chronic pulmonary sepsis, immunosuppression
  • organism: strep and staph
  • morph: liquefactive necrosis surrounded by fibrosis and swelling, capsule around produced by fibroblasts from walls of blood vessels with a zone of reactive gliosis on the outside
  • sx: progressive focal deficits, raised intracranial pressure, raised WBC, increased protein, glucose normal
54
Q

Subdural empyema

A

Bacterial or fungal of skull bones or air sinuses spreading to subdural space
- sx: mass effect, thrombophlebitis may develop in bridging veins with venous occlusion and infarct of brain

55
Q

Tuberculosis

A

Chronic bacterial meningoencephalitis

  • morph: gelatinous or fibrinous exudate most often at the base of the brain, obliterating cisterns, and encasing CN
  • -> mixtures of lymph, plasma cells, macrophages with florid cases of well formed granulomas
  • -> hydrocephalus may result
  • tuberculoma: single well circumscribed intraparenchymal mass associated with meningitis
  • sx: headache, malaise, mental confusion, vomiting
56
Q

Neurosyphilis (3 types)

A

Chronic bacterial meningoencephalitis
- tertiary stage of syphilis
Meningeal neurosyphilis
- chronic meningitis involving base of brain
- associated with obliterative endarteritis –> perivascular inflammatory reaction in plasma cells and lymphocytes
- cerebral gummas
Paretic neurosyphilis
- insidious but progressive mental deficits associated with mood alterations and dementia
- loss of neurons, proliferation of microglia, gliosis, iron deposits
- granular ependymitis
Tabes dorsalis
- sensory nerves in dorsal roots, impaired joint position sense and resultant ataxia, loss of pain, absence of DTR

57
Q

Arthropod borne viral encephalitis

A

Sx: seizures, confusion, delirium, stupor/coma, reflex asymmetry, ocular palsies
CSF: colorless, elevated pressure, neutrophilic pleocytosis convernts to lymph, protein elevated, glucose normal
Morph: microglial around foci of necrosis, nectrotizing vasculitis with associated focal hemorrhages

58
Q

Herpes Simplex Virus Type I Encephalitis

A

Age: children and young adults
Sx: alterations in mood, memory, behavior, weakness, lethargy, ataxia, seizures
Morph: necrotizing hemorrhagic with perivascular inflammatory infiltrates with viral inclusion bodies in neuron and glia

59
Q

Cytomegalovirus

A

Age: fetus and immunosuppressed individuals
- neonates: microcephaly and periventricular calcification
Morph: periventricular necrosis, inclusion bearing cells
Sx: severe hemorrhagic necrotizing ventriculoencephalitis and choroid plexitis or radiculoneuritis

60
Q

Poliomyelitis

A

Acute cases: mononuclear and neuronophagia of anterior horn motor neurons of the spinal cord
Sx: meningeal irritation and CSF aseptic meningitis
–> if progresses to spinal cord, flaccid paralysis with muscle wasting and hyporeflexia

61
Q

Rabies

A

Morph: intense edema and vascular congestion
- widespread neuronal degeneration and infl in brain stem
- Negri bodies are pathognomonic –> cytoplasmic, round to oval eosinophilic inclusions in pyramidal neurons of hippocampus and Purkinje cells of cerebellum
Sx: malaise, headache, fever, local paresthesias, meningismus, flaccid paralysis, mania, stupor, coma, death from respiratory center failure

62
Q

HIV encecphalitis

A

Morph: Chronic inflammatory reaction with widespread infiltrates of microglial nodules with foci of tissue necrosis and reactive gliosis
- endo cells and perivascular foamy or pigment macro
- multinucleated giant cell
Sx: cognitive changes of dementia

63
Q

Progressive multifocal leukoencephalopathy

A

Sx: demyelination principle effect, focal and relentlessly progressive neurologic symptoms and signs
Morph: destruction of white matter with scattered lipid laden macro and reduced number of axons
- glassy viral inclusion, bizarre giant astrocytes with irregular hyperchromatic nuclei mixed with astrocytes

64
Q

Creutzfeldt-Jakob disease

A

Most common prion disease
Sx: rapidly progressive dementia, involuntary jerking muscle contractions on subtle stimulation, ataxia minorly
Path: mutation in PRNP, iatrogenic transmission
Morph: spongiform cerebral cortex, deep gray matter structures
- cystic spaces (status spongiosus)
- Kuru plaques –> extracellular deposits of aggregated abnormal protein, usually occur in cerebellum

65
Q

Variant Creutzfeldt- Jakob disease

A

Age: young people
Sx: slower progressive neurologic symptoms
Morph: spongiform cerebral cortex, deep gray matter structures
- cystic spaces (status spongiosus)
- Kuru plaques –> extracellular deposits of aggregated abnormal protein, usually occur in cerebellum

66
Q

Multiple sclerosis

A

Autoimmune demyelinating disorder with distinct neuro deficits
Gender: women more than men
Path: DR2 related as autoimmune disorder, initiated by CD4 TH1 and TH17 cells that react against self myelin antigens and secrete cytokines
Morph: depressed irregular shaped plaques, found throughout white matter and into gray matter
- active plaque has myelin breakdown with macro, infl cells, astrocytes reactive changes
- shadow plaques on border between normal and affected white matter not circumscribed
Sx: unilateral visual impairment, involved in optic nerve
- CN signs, ataxia, nystagmus, internuclear ophthalmoplegia from interruption of MLF
- motor and sensory impairment of trunk and limbs, spasticity, difficulties with bladder function
CSF: elevated protein level, moderate pleocytosis, increased IgG

67
Q

Acute Disseminated Encephalomyelitis

A

Diffuse monophasic demyelinating disease following a viral infection
Sx: headache, lethargy, coma
Morph: myelin loss with preservation of axons
- polymorphonuclear leukocytes found within lesions with mononuclear infiltrates
- accumulation of lipid laden macro
- monophasic vs MS which is not

68
Q

Central pontine myelinolysis

A

Loss of myelin with preservation of axons and neuronal cell bodies in a symmetric pattern of pons and ponteine tegmentum
- associated with rapid correction of hyponatremia
Sx: evolving quadriplegia

69
Q

Alzheimer disease

A

Most common dementia in elderly (>50 yo)
- autosomal dominant
Path: beta secretase activity leads to Abeta plaques
- plaques aggregate and be neurotoxic
- apoE4 increases risk
Sx: insidious impairment of higher intellectual function with alteration in mood and behavior
- progressive disorientation, memory loss, aphasia, severe cortical dysfunction
Morph: cortical atrophy with widening of sulci in frontal, temporal, parietal lobes
- neuritic plaques –> spherical tortuous processes around a central amyloid core in hippocampus, amygdala, neocortex, Abeta peptide
- neurofibrillary tangles –> bundles of filaments in cytoplasm of neuron that displace or encircle nucleus, tau protein
- cerebral amyloid angiopathy, granulovacuolar degeneration, hirano bodies

70
Q

Pick Disease

A

Progressive dementia characterized by early onset of behavioral changes with alterations in personality and language disturbances
Morph: asymmetric atrophy of frontal and temporal lobes
- lobar atrophy
- pick bodies are cytoplasmic round to oval filamentous inclusions that are weakly basophilic
- composed of straight filaments

71
Q

Parkinsonism

A

Causes:

  • Parkinson’s disease
  • multiple system atrophy
  • postencephalitic parkinsonism
  • progressive supranuclear palsy and corticobasal degeneration
72
Q

Parkinson disease

A

Inheritance: AD or AR, deficiency of LDOPA
Morph: pallor of substantia nigra and locus ceruleus
- Lewy body in remaining neuron which are cytoplasmic, eosinophilic round inclusions
–> composed of alpha-synuclein
Sx: tremor, rigidity, bradykinesia, dementia

73
Q

Huntington Disease

A

Inheritance: Auto Dominant
- progressive movement disorders and dementia with degeneration of striatal neurons (GABA)
Sx: jerky, hyperkinetic, dystonic movements, chorea –> can progress to parkinsonism
Path: CAG repeats of huntingtin disease, earlier sx depending on number of repeats
- dysregulation of basal ganglia from loss of medium spiny striatal neurons
Morph: atrophy of caudate nucleus with putamen; lateral and third ventricles dilated

74
Q

Friedreich Ataxia

A

Inheritance: autosomal recessive progressive illness
Clinical: first decade - gait ataxia with clumsiness and dysarthria
- DTR depressed or absent, joint position and vibratory sense are impaired
- pes cavus and kyphoscoliosis
- associated with cardiac arrhythmias and CHF
Morph: expansion of GAA trinucleotide repeat encoding frataxin
- spinal cord shows loss of axons and gliosis in posterior columns, spinocerebellar tracts, corticospinal tracts
- brainstem, spinal cord, cerebellum and Purkinje cells of superior vermis and Betx cells
- heart enlarged

75
Q

Amyotrophic Lateral Sclerosis

A

Loss of lower motor neurons in spinal cord, brainstem, upper motor neurons
- affects men more than women
Age: fifth decade or later
Genetics: Mutations in SOD1
Path: Reduced capacity to detoxify free radicals leading to neuronal death
Morph: anterior roots of spinal cord are thin, precentral gyrus atrophic
- reactive gliosis and loss of anterior root myelinated fibers –> hypoglossal, ambiguus, motor trigeminal CN nuclei
Sx: asymmetric weakness of hands –> difficulty in fine motor tasks and cramping spasticity of arms and legs
- fasciculations occur
- progressive muscular atrophy or bulbar palsy when it affects CN nuclei

76
Q

Tay Sachs

A

Disease begins in early infancy with developmental delay, paralysis, loss of neuro function and death

77
Q

Krabbe Disease

A

Inheritance: Autosomal recessive leukodystrophy
- deficiency of galactocerebroside beta-galactosidase –> leads to accumulation of galactocerebroside
Sx: rapidly progressive, death by age 2
- motor signs, stiffness and weakness with worsening difficulties in feeding
Morph: loss of myelin and oligodendroctyes in CNS and similarly in peripheral nerves
- engorged macrophages in parencyhuma and BV

78
Q

Metachromatic leukodystrophy

A

Inheritance: autosomal recessive
- deficiency of lysosomal enzyme arylsulfatase A
- accumulation of sulfatides leading to myelin breakdown
Age: late infantile form (most common), juvenile form, adult form
Sx: childhood - motor sx which progress gradually
- adult –> psychiatric or cognitive sx with motor sx later
Morph: demyelination with gliosis, macrophages with vacuolated vytoplasm scattered throughout white matter
- contain crystalloid structures composed of sulfatides

79
Q

Leigh Syndrome

A

Age: early childhood
- lactic acidemia with arrest of psychomotor development, feeding problems, seizures, extraocular palsies, weakness with hypotonia
- death occurs 1-2 years
Morph: symmetric regions of brain tissue with spongiform appearance and proliferation of blood vessels
- periventricular gray matter of midbrain, tegmentum of pons, thalamus and hypothalamus
Sx: neuropathy, ataxia, retinitis pigmentosa (NARP)

80
Q

Thiamine (vit B1) deficiency

A

Causes beriberi –> associated with cardiac failure
Sx: psychotic symptoms or ophthalmoplegia begins abruptly as Wernicke encephalopathy
- can progress to Korsakoff syndrome –> memory disturbances and confabulation
Causes: chronic alcoholism, gastric disorders, persistant vomiting
Morph: hemorrhage and necrosis in mamillary bodies and third and fourth wall of ventricle
- infiltration of macro and cystic space with hemosiderin laden macro

81
Q

Vitamin B12 deficiency

A

Anemia with severe effects on nervous system
Sx: numbness, tingling, ataxia in lower extremities, spastic weakness in lower extremities
- complete paraplegia
Morph: swelling of myelin layers producing vacuoles
- begins at midthoracis level of spinal cord
Clinical: subacute combined degeneration of the spinal cord

82
Q

Hypoglycemia

A

Selective injury of large pyramidal neurons of cerebral cortex

  • can lead to pseudolaminar necrosis of cortex
  • hippocampus vulnerable may show in Sommer sector
83
Q

Hyperglycemia

A

Inadequately controlled DM associated with ketoacidosis or hyperosmolar coma
Sx: dehydration, confusion, stupor, coma

84
Q

Hepatic encephalopathy

A

Glial cells, Alzheimer type II evident in cortex and basal ganglia with subcortical gray matter regions

85
Q

Carbon monoxide

A

Neurons in layer III and V of cerebral cortex, Sommer sector of hippocampus, purkinje cells

  • bilateral necrosis of globus pallidus
  • demyelination may be a later event
86
Q

Methanol

A

Affects retina –> causes blindness

- selective bilateral putamenal necrosis and focal white matter necrosis possible

87
Q

Ethanol

A

Chronic alcohol abuse can lead to Wernicke-Korsakoff syndrome
- cerebellar dysfunction occurs in 1% of chronic alcoholics –> cerebellar ataxia, unsteady gait, nystagmus
Morph: atrophy and loss of granule cells in anterior vermis
- loss of purkinje cells and proliferation of adjacent astrocytes between granular cell layer and molecular layer of cerebellum

88
Q

Radiation

A

Intractable nausea, confusion, convulsions, rapid coma and death
- delay: HA, nausea, vomiting, papilledema
Morph: large areas of coagulative necrosis and adjacent edema

89
Q

Astrocytoma

A

Two categories: infiltrating and non-infiltrating neoplasm

  • infiltrating accounts for 80% of adult brain tumors
  • -> usually in cerebral hemispheres
  • -> sx: seizures, headache, focal neurologic deficits
  • -> morph: p53 and PDGF, higher grade is RB and p16/CDKNaA
90
Q

Diffuse astrocytoma

A

Grade II

  • poorly defined, gray, infiltrative tumor that invaders and distorts brain
  • tumor is firm or soft and gelatinous with cystic degeneration
  • glial cellularity and variable nuclear pleomorphism with GFAP astrocytic processes
91
Q

Anaplastic astrocytoma

A

Grade III

- densely cellular with nuclear pleomorphism with mitotic figures

92
Q

Glioblastoma

A

Grade IV

  • Some firm areas with white others soft and yellow from necrosis
  • cystic degeneration and hemorrhage
  • necrosis and vascular or endothelial cell proliferation
  • pseudopalisading pattern
93
Q

Pilocytic astrocytoma

A

Grade I
Age: children and young adults
Location: cerebellum or in floor and walls of third ventricle, optic nerves, cerebral hemispheres
Morph: cystic, if solid well circumscribed
- bipolar thin hairlike processes GFAP positive with fibrillary meshworks
- Rosenthal fibers and eosinophilic granular bodies

94
Q

Oligodendroglioma

A

Stats: 5-15% of gliomas
Age: fourth and fifth decades
Sx: seizures
Morph: lesions in white matter
- well circumscribed, gelatinous, gray masses with cysts, focal hemorrhage, calcification
- sheets of regular cells with spherical nuclei containing finely granular chromatin surrounded by clear halo of cytoplasm

95
Q

Ependymoma

A

Central canal of spinal cord
Age: first two decades of life occur near fourth ventricle
- adults: spinal cord, related to NF2
Morph: solid or papillary masses extending from floor of ventricle
- perivascular pseudorosettes –> tumor cells arranged around vessels with thin ependymal processes directed toward wall of vessel
- GFAP
Sx: hydrocephalus secondary to progressive obstruction of fourth ventricle
- can be disseminated by CSF

96
Q

Choroid plexus papillomas

A

Age: children more common in lateral ventricle
- adult involve fourth ventricle
Sx: hydrocephalus from obstruction of ventricular system by tumor or overproduction of CSF

97
Q

Colloid cyst of third ventricle

A

Non-neoplastic lesion occurring in young adults
- cyst attached to roof of third ventricle obstruct foramina of monro causing noncommunicating hydrocephalus
Sx: headache, positional; rapidly fatal

98
Q

Ganglioglioma

A

Most common CNS tumor with mature appearing neurons –> admixed glial neoplasm
Morph: Commonly found in temporal lobe with cystic component
- irregularly clustered and has random neurites with binucleate forms

99
Q

Medulloblastoma

A

Age: children mostly in midline, adults in lateral
Other: only in cerebellum
Sx: hydrocephalus, highly malignant, confusion with peripheral lesion related to ewing sarcoma, PNET
Morph: well circumscribed, gray, friable extending into cerebellar folia and leptomeninges
- extremely cellular with anaplastic cells, mitoses
- neuronal and glial phenotypes
- loss of 17p
- blue small cells

100
Q

Germ cell tumors

A

Primary brain germ cell tumors along midline, pineal and suprasellar regions
- more common in Japanese
Age: young
Gender: males in pineal region
Sx: similar to counterparts in gonads, not usually metastasis –> alpha-fetoprotein and beta-HCG

101
Q

Meningiomas

A

Age: adults
- benign tumors attached to dura from arachnoid
Morph: rounded masses with well defined bases that compress brain –> can be separated
- syncytial –> whorled clusters of cells in groups
- fibrobastic –> elongated cells and abundant collagen
- transitional –> syncytial and fibroblastic
- psammomatous –> psammomabodies
- secretory –> PAS positive intracytoplasmic droplets and intracellular lumens by EM
- microcystic –> loose spongy appearance
- Atypical (grade II), anaplastic (grade III)
Molecular: loss of chromosome 22, loss of NF2
Sx: vague nonlocalizing sx or focal findings

102
Q

Most Common Metastasis

A

Lung, breast, skin, kidney, GI tract

Morph: junction of gray and white matter with zone of edema surrounding

103
Q

Limbic encephalitis

A

Subacute dementia with perivascular inflammatory cuffs, microglial nodules, neuronal loss, gliosis

104
Q

Schwannoma

A

Benign tumors from neural crest derived cell –> part of NF2
Sx: locally compressed nerve or adjacent structures
Molecular: loss of NF2 merlin
Morph: well circumscribed masses attached to nerve but can be separated
- firm gray masses with cystic and xanthomatous change
- Antoni A showing high cellularity zones and nuclear free zones termed verocay bodies
- Antoni B less dense cells with microcyts and myxoid stroma

105
Q

Neurofibroma

A

Discrete localized masses, nodules with hyperpigmentation
Morph: spindle cells with highly collagenized tumor
Molecular: NF1 inhibits RAS activity, loses NFI

106
Q

Plexiform neurofibroma

A

Anywhere along nerve tumors, multiple
- cannot be separated from nerves
Morph: myxoid stroma with collagen bundles
Molecular: NF1 inhibits RAS activity, loses NFI

107
Q

Malignant peripheral nerve sheath tumor

A

Locally invasive, frequent recurrences, metastases
- medium to large nerves
Morph: poorly defined tumor masses infiltrate along axis of parent nerve and adjacent soft tissues
- mitosis, necrosis, nuclear anaplasia common

108
Q

Neurofibromatosis Type I

A

Inherited: Autosomal dominant disorder
- neurofibromas, gliomas of optic nerve, pigmented nodules of iris (lisch nodules), cutaneous hyperpigmented macules (cafe au lait spots)
Molecular: lack of NF1 activity, loses inhibition of RAS

109
Q

Neurofibromatosis Type II

A

Inherited: autosomal dominant disorder
- range of tumors most commonly CN VIII schwannomas and multiple meningiomas with gliomas
Molecular: NF2 encodes merlin

110
Q

Tuberous Sclerosis Complex

A

Inherited: autosomal dominant
- hamartomas and benign neoplasms involving brain and tissues
–> cortical tubers and subependymal nodules
–> renal angiomyolipomas, retinal glial hamartomas, pulmonary lymphangioleiomyomatosis, cardiac rhabdomyomas
- cyts found in liver, kidneyes, pancreas
Molecular: TSC1 encodes hamartin or TSC2 encodes tuberin
Morph: firm areas of cortex, haphazardly arranged neurons that lack organization
Sx: seizures

111
Q

Von Hippel-Lindau disease

A

Inheritance: autosomal dominant disorder
- hemangioblastomas and cysts in pancreas, liver, kidneys, renal cell carcinoma and pheochromocytoma
Molecular: VHL is a tumor suppressor gene that regulates hypoxia induced factor I
Morph: hemangioblastomas highly vascularized neoplasms that occur with a large fluid-filled cyst

Pathology (12 decks)

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