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Neuro II > Path I > Flashcards

Path I Flashcards

1
Q

Group atrophy of skeletal muscles suggests what cause?

A

neurogenic disease

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2
Q

Perifascicular atrophy of skeletal muscle suggests what cause?

A

dematomyositis

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3
Q

Type II fiber atrophy of skeletal muscle suggests what cause?

A

corticosteroid therapy or disuse

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4
Q

Whta pathology is shown in the provided image?

A

Denervation & atrophy

some of the cells are very atrophic & more angulated

not dead - just atrophic b/c not getting the stimulation from the nerve

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5
Q

During reinnervation, what can happen to fiber type?

A

they can switch depending on what type of nerve the get re-innervated by

axon determine what type o myofibril you have

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6
Q

What type of pathology is shown in teh provided iamge?

How do you know?

A

Group atrophy & then regenration separated by type

  • loss of “checkerboard” appearance to fiber type
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7
Q

What condition is seen in this provided image?

A

Myofiber hypertophy

can have many causes

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8
Q

What is the difference in presentation between Duchenne muscular dystrophy & Becker muscular dystrophy?

Presence of dystrophin?

A
  • Duchenne
    • severe chronic muslce damage
    • dystrophin absent (frameshift)
  • Becker
    • milder chronic muscle damage
    • dystrophin reduced (truncation)
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9
Q

What is the inheritance pattern of Muscular dysrophy with dystrohin mutation?

A

X-linked

(Duchenne & Becker)

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10
Q

Dystrophin is encoded by what gene?

A

IHC

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11
Q

If this is a stain for dystophin, what condition is depicted in the provided slide?

A

Duchenne muscular dystrophy

the brown outline of the myofibers in the right-hand box is the dystrophin; notice this is absent in the larger image, indicative of the absence of dystrphin

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12
Q

What condition is depicted in the provided slide?

The sample on the left is froma 3 yr old and the sample on the right is from a 9 yr old

A

Muscular Dystrophy

The collagen fibrosis that course between & around the myofibers is characteristic of muscular dystrophy

In the sample on the right, even more myofibers have been replaced by collagen & adipose

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13
Q

Identify the types of peripheral nerve damage shown in the diagram

A
  • Top: axonal degeneration
    • significant atrophy of myofibers
  • Bottom: demyelination
    • no signifiant atrophy of myofibers
    • transmission will slow
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14
Q

What are the characteristics of nerves after regeration of axons after injury?

What are the characteristics of nerves after remission of demyelinating disease?

A
  • Axon injury
    • connections with myofibers reform
    • new internodes are shorter
    • sheaths are thinner
  • Demyelinating
    • remyelination
    • new internodes are shorter
    • thinner myelins sheaths than the undamaged nodes
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15
Q

What types of tumors have evidence of Schwann cell differentiation?

A

Peripheral Nerve Sheath Tumos

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16
Q

Peripheral nerve sheath tumors are associated with what common familial tumor syndromes?

A

neurofibromatosis 1 & 2

schwanomatosis

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17
Q

How do malignant peripheral nerve sheath tumors most commonly arise?

A

malignant transformation of preexisting benign plexiform neurofibromas

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18
Q

What pathology is shown on the provided image?

Benign or malignatn?

Arising from what cells?

A

Schwannoma

(well-circumscribed, encapsulated, firm gray masses, loosely attached to, but not invadign associated nerve)

benign tumor

often arist directly from peripheral nerves

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19
Q

Schwannomas asre associated with what gene mutation?

A

inactivating mutation in NF2 gene on chromosome 22

wiht loss of gene product (merlin)

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20
Q

The provided microscopic samples are from what larger pathology? Describe the two samples.

A

Schwannoma

  • Left
    • Verocay bodies (arrow) in Antoni A areas
      • tumor cells are very elongated & somewhat spindle-shaped
      • sometimes lined up like a picket fence & in between them will look like a little pathway that has been cleared
  • Right
    • Cellular Antoni B region (left) and loose Antoni A (right)
    • think “B for Barren”
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21
Q

What is the composition of neurofibromas?

A

schwann cells are admixed with perineurial-like cells, fibroblasts, mast cells & CD34+ spindle cells

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22
Q

What are the 3 types of neurofibromas?

A

superficial cutaneous

diffuse

plexiform

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23
Q

Neurofibromas can be associated with what gene mutation?

A

NFT1

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24
Q

What pathology is shown on the provided image?

A

Cutaneous superficial neurofibroma

ovoid or fusiformmass with gelatinous or wavy cut surface

(transected nerve fibers can be seen at the ends)

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25
Q

The provided microscopic sample is from what pathology?

A

Neurofibroma

small, elongated spindle cellw & collagenous stroma

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26
Q

What pathology is shown in the provided image?

A

Plediform Neurofibromas

“bag of worms”

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27
Q

The provided cross section sample is take from what pathology?

A

fascicles of plexiform neurofibroma

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28
Q

What is a feature of plexiform neurofibromas that is very rarely seen on other types of neurofibromas?

A

“shredded carrots” collagen bundles

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29
Q

What pathology is shown in the provided images?

A

Malignant Peripheral Nerve Sheath Tumor

large, fusiform masses arising from & incorporating nerves

bulky, circumscribed, pseudo-encapsulated mass w/ hemorrhage (black curved arrow) & cystic areas (blue arrow)

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30
Q

The provided microscopic images are from what larger pathology?

A

Malignant Peripheral Nerve Sheath Tumor

higih-grade, cellular neoplasm with numerous mitoses & some nuclear atypia

Right - focal area of nerosis indicated by black curved arrow

31
Q

How often do primary CNS tumors metastasize outside of the CNS?

A

very rarely

32
Q

What are the most common CNS tumors in adults?

A

glioblastoma

meningioma

ependyoma

33
Q

What are the most common CNS tumors in children?

A

cystic (pilocystic) astrocytoma

medulloblastoma

brainstem glioma

34
Q

Compare / contrast the following features for CNS tumors in adults & children:

Supra vs. infratentorial?

prevalence

A
  • adult
    • 70% supratentorial
    • not in top 10
  • children
    • 70% infratentorial
    • 2nd most common general type of cancer
35
Q

What are the risk factors for developing a CNS tumor?

A
  • Turcot Syndrome
  • Neurofibromatosis
    • Type 1
    • Type 2
  • Cigarette smoking
36
Q

What pathologies are associted with Turcot syndrome?

A

multiple adenomatous colon polyps

brain tumors

37
Q

What pathologies are associted with Neurofibromatosis Type 1?

A
  • neurofibromas
  • MPNSTs
  • optic nerve gliomas
  • other glial tumors
  • pheochromocytomas
38
Q

What pathologies are associted with Neurofibromatosis Type 2?

A
  • bilateral 8th nerve schwannomas
  • mulitple meningiomas
  • ependymomas of spinal cord
39
Q

What are the categories of giomas?

A

astrocytoma

oligodendrogliomas

ependymomas

40
Q

Identify the following characteristics for Pilocytic Astrocytoma:

Benign vs malignant

demographics affected

location

grade

A
  • relatively benigh
  • children & young adults
  • cerebellum
  • grade I
    • grow very slow
41
Q

What pathology is depicted in the provided image?

A

Pilocytic Astrocytoma

cystic & solid part located in the cerebellum

42
Q

What pathology is depicted in the provided image?

A

Pilocytic Astrocytoma

cystic & solid part located in the cerebellum

43
Q

The provided microscopic slides are a sample from what pathology?

A

Pilocytic Astrocytoma

  • Left
    • biphasic patter w/ loose, myxoid areas ademixed w/ eosinophilic compact regions
  • Right
    • rosenthal fibers are eosinophilic, irregulat, elongated “corkscrew” protein aggregates
44
Q

Identify the following characteristics for Diffuse Astrocytoma:

demographics affected

grade

A

grade II

young - middle aged adults

45
Q

What pathology is shown in the provided images?

A

Diffuse Astrocytoma

infiltrative margins

left : right frontal tumor w/ expanded gyri & flattening

right: bilateral expansion of septum pellucidum

46
Q

What features would you expect to see in a microscopic sample from a Diffuse Astrocytoma?

A
  • subtle
  • some nuclear pleomorphism
  • increased cellularity
    • GFAP
47
Q

Anaplastic Astrocytom is what WHO grade?

A

III

48
Q

What features would you expect to see in a microscopic sample from a Anaplastic Astrocytoma?

What features will you NOT see?

A
  • more pleomorphism
  • higher cellularity
  • higher mitotic rate

than Diffuse Astrocytoma

  • NO necrosis
  • NO vascular proliferation
49
Q

What pathology is shown in the provided image?

A

Glioblastoma

necrotic, granular-looking, hemorrhagic

50
Q

Glioblastoma is what WHO grade?

A

IV

51
Q

Glioblastomas can cause what complications?

A

mass effect

herniation

52
Q

What features would you expect to see on a microscompic sample of a glioblastoma?

A
  • increased cellularity
  • increased mitotic features
  • increaed nuclear pleomorphism
  • areas of necrosis w/ pallisading cells around the necrosis
  • vascular proliferation
53
Q

The provided microscopic sample of from what pathology?

A

Glioblastoma

necrosis w/ palisading cells is characteristic

54
Q

What glioblastoma feature is shown in the provided microscopic sample?

A

glomeruloid body

small-type vascular proliferation

55
Q

Identify the following characteristics for Oligodendroglioma:

demographics affected

location

grade

A

4th - 5th decade

cerebral hemispheres

grade II

56
Q

What is the mutation associated with oligodendrogliomas?

Is this seen in adult or pediatric populations?

A

1p19q deletion, due to unbalanced translocation t(1;19)(q10;p10)

adult cases

57
Q

What grade is anaplastic oligodendrogliomas?

A

grade III

58
Q

What pathology is shown in the provided image?

A

Oligodendroglioma

curvilinea calcifications

59
Q

What microscopic features would you expect to see in a sample of an oligodendrioma?

A

“fried egg” appearance & delicat meshwork of capillaries

60
Q

Where are ependyomas found in children vs. adults?

Clinical features?

Which demographic has a better prognosis?

A
  • children
    • 4th ventricle
      • hydrocephalus if in posterior fossa b/c destruction of 4th ventricle
    • unfavorable diagnosis
  • adults (4th decade)
    • spinal cord (lumbosacral)
    • favorable diagnosis

CSF dissemination is common

61
Q

Ependymoma is frequently seen in the setting of what condition?

A

neurofibromatosis type 2

62
Q

What type of tumor is this tumor of the 4th ventricle?

A

Ependymoma

63
Q

What would you expect to see in a microscopic sample of an ependymoma?

A

perivascular pseudorosettes (solid blue arrow)

true rosettes (open blue arrow)

64
Q

What does “embryonal” indicate in the context of CNS tumors?

A

poorly differentiated

65
Q

What is the key embryonal tumor?

A

medulloblastoma

66
Q

Identify the characteristic of a medulloblastoma:

demographics

location

clinical features

treatment

A
  • demographics
    • children (5-10 yr)
  • location
    • exclusively cerebellum
  • clinical features
    • rapid growth - hydrocephalus
    • dissemination through CSF
      • metastases to cauda equina
  • treatment
    • highly malignant; exquisitely radiosensitive
67
Q

What pathology is shown in the provided image?

A

medulloblastoma

destroying superior midline cerebellum

68
Q

What features would you expect to see in a microscopic sample of a medulloblastoma?

A

sheets of small cells with elongated nuclei & scant cytoplasm

69
Q

Meningioma is derived from what cells?

demographics?

grade?

location?

A
  • mengiothelial cells
  • adults - F
  • grade I
  • attached to dura
70
Q

Meningiomas are associated with what mutations & disorders?

A

loss of entire chromosome 22 or 22q

neurofibromatosis type 2

71
Q

What pathology is shown in the provided image?

A

Meningioma

well circumscribed mass attached to meninges, not invading brain parenchyma

72
Q

What would you expect to see in a microscopic sample from the various variants of a meningioma?

A

left - psammoma bodies (some variants)

middle - Washed out nuclei & pseudo inclusions

right - whorls of cells & lobules (transitional variant)

73
Q

What are the most common primary site for metastatic CNS tumor?

typical location of metastisis?

A
  • Primary site
    • lung
    • breast
    • melanoma
    • kidney
    • colorectal
  • gray-white junction of cerebral hemisphere
74
Q

Which primary sites can cause meningeal carcinomatosis?

A

lung & breast

Neuro II (30 decks)

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