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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
The provided microscopic sample is from what pathology?
Neurofibroma small, elongated spindle cellw & collagenous stroma
26
What pathology is shown in the provided image?
Plediform Neurofibromas "bag of worms"
27
The provided cross section sample is take from what pathology?
fascicles of **plexiform neurofibroma**
28
What is a feature of plexiform neurofibromas that is very rarely seen on other types of neurofibromas?
"shredded carrots" collagen bundles
29
What pathology is shown in the provided images?
**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)
30
The provided microscopic images are from what larger pathology?
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
How often do primary CNS tumors metastasize outside of the CNS?
very rarely
32
What are the most common CNS tumors in adults?
glioblastoma meningioma ependyoma
33
What are the most common CNS tumors in children?
cystic (pilocystic) astrocytoma medulloblastoma brainstem glioma
34
Compare / contrast the following features for CNS tumors in adults & children: Supra vs. infratentorial? prevalence
* adult * 70% supratentorial * not in top 10 * children * 70% infratentorial * 2nd most common general type of cancer
35
What are the risk factors for developing a CNS tumor?
* Turcot Syndrome * Neurofibromatosis * Type 1 * Type 2 * Cigarette smoking
36
What pathologies are associted with Turcot syndrome?
multiple adenomatous colon polyps brain tumors
37
What pathologies are associted with Neurofibromatosis Type 1?
* neurofibromas * MPNSTs * optic nerve gliomas * other glial tumors * pheochromocytomas
38
What pathologies are associted with Neurofibromatosis Type 2?
* bilateral 8th nerve schwannomas * mulitple meningiomas * ependymomas of spinal cord
39
What are the categories of giomas?
astrocytoma oligodendrogliomas ependymomas
40
Identify the following characteristics for Pilocytic Astrocytoma: Benign vs malignant demographics affected location grade
* relatively benigh * children & young adults * cerebellum * grade I * grow very slow
41
What pathology is depicted in the provided image?
Pilocytic Astrocytoma cystic & solid part located in the cerebellum
42
What pathology is depicted in the provided image?
Pilocytic Astrocytoma cystic & solid part located in the cerebellum
43
The provided microscopic slides are a sample from what pathology?
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
Identify the following characteristics for Diffuse Astrocytoma: demographics affected grade
grade II young - middle aged adults
45
What pathology is shown in the provided images?
Diffuse Astrocytoma infiltrative margins _left_ : right frontal tumor w/ expanded gyri & flattening _right:_ bilateral expansion of septum pellucidum
46
What features would you expect to see in a microscopic sample from a Diffuse Astrocytoma?
* subtle * some nuclear pleomorphism * increased cellularity * + GFAP
47
Anaplastic Astrocytom is what WHO grade?
III
48
What features would you expect to see in a microscopic sample from a Anaplastic Astrocytoma? What features will you NOT see?
* more pleomorphism * higher cellularity * higher mitotic rate than Diffuse Astrocytoma * NO necrosis * NO vascular proliferation
49
What pathology is shown in the provided image?
Glioblastoma necrotic, granular-looking, hemorrhagic
50
Glioblastoma is what WHO grade?
IV
51
Glioblastomas can cause what complications?
mass effect herniation
52
What features would you expect to see on a microscompic sample of a glioblastoma?
* increased cellularity * increased mitotic features * increaed nuclear pleomorphism * **areas of necrosis** **w/ pallisading cells around the necrosis** * **vascular proliferation**
53
The provided microscopic sample of from what pathology?
Glioblastoma necrosis w/ palisading cells is characteristic
54
What glioblastoma feature is shown in the provided microscopic sample?
glomeruloid body small-type vascular proliferation
55
Identify the following characteristics for Oligodendroglioma: demographics affected location grade
4th - 5th decade cerebral hemispheres grade II
56
What is the mutation associated with oligodendrogliomas? Is this seen in adult or pediatric populations?
**1p19q deletion**, due to unbalanced translocation t(1;19)(q10;p10) _adult cases_
57
What grade is anaplastic oligodendrogliomas?
grade III
58
What pathology is shown in the provided image?
**Oligodendroglioma** curvilinea calcifications
59
What microscopic features would you expect to see in a sample of an oligodendrioma?
"fried egg" appearance & delicat meshwork of capillaries
60
Where are ependyomas found in children vs. adults? Clinical features? Which demographic has a better prognosis?
* 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
Ependymoma is frequently seen in the setting of what condition?
neurofibromatosis type 2
62
What type of tumor is this tumor of the 4th ventricle?
Ependymoma
63
What would you expect to see in a microscopic sample of an ependymoma?
perivascular pseudorosettes (solid blue arrow) true rosettes (open blue arrow)
64
What does "embryonal" indicate in the context of CNS tumors?
poorly differentiated
65
What is the key embryonal tumor?
medulloblastoma
66
Identify the characteristic of a medulloblastoma: demographics location clinical features treatment
* _demographics_ * children (5-10 yr) * _location_ * exclusively cerebellum * _clinical features_ * rapid growth - hydrocephalus * dissemination through CSF * metastases to cauda equina * **_treatmen_**t * highly malignant; exquisitely radiosensitive
67
What pathology is shown in the provided image?
medulloblastoma destroying superior midline cerebellum
68
What features would you expect to see in a microscopic sample of a medulloblastoma?
sheets of small cells with elongated nuclei & scant cytoplasm
69
Meningioma is derived from what cells? demographics? grade? location?
* mengiothelial cells * adults - F * grade I * attached to dura
70
Meningiomas are associated with what mutations & disorders?
loss of entire chromosome 22 or 22q neurofibromatosis type 2
71
What pathology is shown in the provided image?
Meningioma well circumscribed mass attached to meninges, not invading brain parenchyma
72
What would you expect to see in a microscopic sample from the various variants of a meningioma?
left - psammoma bodies (some variants) middle - Washed out nuclei & pseudo inclusions right - whorls of cells & lobules (transitional variant)
73
What are the most common primary site for metastatic CNS tumor? typical location of metastisis?
* Primary site * lung * breast * melanoma * kidney * colorectal * gray-white junction of cerebral hemisphere
74
Which primary sites can cause meningeal carcinomatosis?
lung & breast

Neuro II (30 decks)

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