Muscle Tissue Pathology Flashcards

1
Q

what is myasthenia gravis?

A

autoimmune or congenital neuromuscular disease

muscle weakness

antibodies block ach receptors at the postsynaptic neuromuscular junction

inhibits excitatory effects at nicotinic receptors

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2
Q

what are diplopia and ptosis and what are they a symptom of?

A

ptosis is drooping of eyelid

diplopia is double vision

–myasthenia gravis

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2
Q

rhabdomyosarcoma

A

malignant tumor of skeletal muscle

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2
Q

t(2;13) translocation

A

alveolar rhabdomyosarcoma

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2
Q
A
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3
Q

endomysial cd8 tcells (cell mediated)

A

polymyositis

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4
Q
A

polymyositis

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4
Q

sarcoma of retroperitoneum and deep soft tissues

A

malignant fibrous histiocytoma

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5
Q

vimentin immunohistochemical stain signifies what?

A

sarcoma/rhabdomyosarcoma

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6
Q

liposarcoma

A

most common soft tssue sarcoma in adults, large,

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7
Q

+ for myogenin

A

pleomorphic rhabdomyosarcoma

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8
Q

most common malignant tumor in adults

A

liposarcoma

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9
Q

endomysial inflammation (CD*+ t cells) with necrotic muscle fibers; skin is not involved

A

polymyositis

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9
Q

antibodies against presynaptic Ca channels of the NeuroMuscularJunction

A

lambert-eaton syndrome

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10
Q

which resonds poorly to glucocorticoids, dermatomyositis or polymyositis or inclusion body myositis?

A

inclusion body myositis

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11
Q

telangiecstasias

A

dilated capillary loops (dermatomyositis)

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12
Q

rhabdomyosarcoma is frequent in…

A

children, head and neck, gentourinary, retroperitoneum

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14
Q

what do you treat dermatomyositis with?

A

corticosteroids

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16
Q

myasthenia gravis ______ with exertion

A

worsens

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17
Q

what arises as a paraneoplastic syndrome due to small cell carcinoma of the lung

A

lambert-eaton syndrome

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18
Q

neuron specific enolase immunohistochemical stain

A

neoplasms with neuronal differentiation

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20
Q

nodular fasciitis follows…

A

trauma

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21
Q

cause of and clinical features of lambert-eaton syndrome?

A

cause: presynaptic ca channels cannot fire so acetylcholine is not released
findings: proximal muscle weakness that improves with use, eyes usually not affected, anticholinesterase agents do not help, and resolves with resection(removal) of cancer

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21
Q

most sarcomas are ____+ and most carcinomas are ____+

A

vimentin ; cytokeratin

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22
fibrous septae dividing cells into clusters t(2;13) children
alveolar rhabdomyosarcoma
23
Lab findings of dermatomyositis?
increase creatine kinase +ANA, + anti-Jo1 antibody
24
what defect causes x linked muscular distrophy?
mutated dytrophin gene (xp21) spontaneous - large gene size predisposes to high mutation rate
25
muscle weakness that worsens with use and improves with rest
myastheni gravis
25
UPS
undifferentiated pleomorphic sarcoma lack of differentiation, deep in extremities, poor prognosis sheets of anaplastic bizarre cells/nuclei, high mitotic rate
27
what is chronic inflammation of multiple muscles?
polymyositis
27
28
Causes of duchenne and becker muscular dystrophies
duchenne - deletion of xp21 (dystrophin) becker - mutated dystrophin (xp21)
29
what type of tumor is associated w tuberous sclerosis??
cardiac rhabdomyoma
30
synovial sarcoma
found in joint or deep tissue arise from mesenchymme not synovium
31
malignant tumor of adipose
liposarcoma
33
how do you treat myasthenia gravis?
acetylcholinesterase inhibitors or anticholinesterase agents immunosuppressants in selected cases, thymectomy(if thymic hyperplasia or thymoma present)
34
white superficial nodules (plantar palmar penile) or deep large and infiltrative in girdles and abdominal cavity low mitotic activity
fibromatosis
35
most common benign adult soft tissue tumor
lipoma
36
38
dematomyositis
39
most common post-irradiation soft tissue sarcoma
malignant fibrous histiocytoma
40
marker for epithelial originating tumors
cytokeratin
42
what is a systemic CT disease that causes inflammation of the skin and muscle?
dermatomyositis
43
perimysial CD4/b cells
dermatomyositis
44
duchenne muscular dystrophy
45
in rhabdomyosarcoma, a t(2;13) translocation does what?
makes a PAX3-FKHR protein that disregulates muscle differentiation
46
diplopia and ptosis are signs of
myasthenia gravis
47
autoantibodies against presynaptic Ca++ channels and block Ach release
eaton lambert syndrome
48
histology comparison of dermatomyositis and polymyositis
DM: lymphocytes (mostly B cells) are perivascular and parimysial; atrophic muscle fibers are at periphery and not next to lymphocytes --\> intramuscular capillaries are main immune attack site PM: lymphocytes (cd8 T cells) and macrophages surround strophic/necrotic muscle tissue --\> muscle fibers are directly injured by cell-mediated immune attack
49
rimmed vacuoles like polymyositis with endomysial cd8 infiltrates
inclusion body myositis
50
clinical features of dermatomyositis?
bilateral proximal muscle weakness -distal involvement later on heliotrope rash on upper eyelids malar (facial butterfly) rash Gottron papules (scaly erythematous papules on elbows, knuckles, and knees)
51
boytryoides
grape like - assocaiated form of rhabdomyosarcoma
52
benign tumor of adipose
lipoma
53
how does duchenne muscular dystrophy present?
1 yo - proximal muscle weakness --\> progresses to distal muscles elevates serum CK and calf pseudohypertrophy death usually from cardiac or respiratory failure (myocardium involvement)
54
most common malignant soft tissue tumor in children
rhabdomyosarcoma
55
strap cell mimicking skeletal muscle cross striations
rhabdomyosarcoma indicator
57
eaton lambert syndrome ________ with repetitive stimulation
improves
58
degenerative disorder characterized by muscle wasting and replacement of skeletal muscle by adipose tissue
x linked muscular dystrophy
59
herringbone pattern in histology
fibrosarcoma
60
benign tumor of skeletal muscle
rhabdomyoma
61
What is dystrophin?
cytoplasmic protein that anchors the muscle fiber cytoskeleton to the extracellular matrix
62
autoantibodies against AcH receptors
myasthenia gravis
63
rhabdomyosarcoma: characteristic cell? desmin + or -? most common site?
rhabdomyoblast desmin + head and neck (vagina for young girls)
64
amyotrophic lateral sclerosis (ALS)
upper and lower motor neuron damage causing muscle atrophy and weakness muscle denervation affects cerebral cortex not selective - can affect type 1 and 2 muscle fibers early atrophy is angular and scattered but later on grouped