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MSK Part 2 (nlg) > Muscle Tissue Pathology > Flashcards

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
Q

fibrous septae dividing cells into clusters

t(2;13)

children

A

alveolar rhabdomyosarcoma

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23
Q

Lab findings of dermatomyositis?

A

increase creatine kinase +ANA, + anti-Jo1 antibody

24
Q

what defect causes x linked muscular distrophy?

A

mutated dytrophin gene (xp21)

spontaneous - large gene size predisposes to high mutation rate

25
Q

muscle weakness that worsens with use and improves with rest

A

myastheni gravis

25
Q

UPS

A

undifferentiated pleomorphic sarcoma

lack of differentiation, deep in extremities, poor prognosis

sheets of anaplastic bizarre cells/nuclei, high mitotic rate

27
Q

what is chronic inflammation of multiple muscles?

A

polymyositis

27
Q
A
28
Q

Causes of duchenne and becker muscular dystrophies

A

duchenne - deletion of xp21 (dystrophin)

becker - mutated dystrophin (xp21)

29
Q

what type of tumor is associated w tuberous sclerosis??

A

cardiac rhabdomyoma

30
Q

synovial sarcoma

A

found in joint or deep tissue

arise from mesenchymme not synovium

31
Q

malignant tumor of adipose

A

liposarcoma

33
Q

how do you treat myasthenia gravis?

A

acetylcholinesterase inhibitors or anticholinesterase agents

immunosuppressants

in selected cases, thymectomy(if thymic hyperplasia or thymoma present)

34
Q

white superficial nodules (plantar palmar penile) or deep large and infiltrative in girdles and abdominal cavity

low mitotic activity

A

fibromatosis

35
Q

most common benign adult soft tissue tumor

A

lipoma

36
Q
A
38
Q
A

dematomyositis

39
Q

most common post-irradiation soft tissue sarcoma

A

malignant fibrous histiocytoma

40
Q

marker for epithelial originating tumors

A

cytokeratin

42
Q

what is a systemic CT disease that causes inflammation of the skin and muscle?

A

dermatomyositis

43
Q

perimysial CD4/b cells

A

dermatomyositis

44
Q
A

duchenne muscular dystrophy

45
Q

in rhabdomyosarcoma, a t(2;13) translocation does what?

A

makes a PAX3-FKHR protein that disregulates muscle differentiation

46
Q

diplopia and ptosis are signs of

A

myasthenia gravis

47
Q

autoantibodies against presynaptic Ca++ channels and block Ach release

A

eaton lambert syndrome

48
Q

histology comparison of dermatomyositis and polymyositis

A

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
Q

rimmed vacuoles like polymyositis with endomysial cd8 infiltrates

A

inclusion body myositis

50
Q

clinical features of dermatomyositis?

A

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
Q

boytryoides

A

grape like - assocaiated form of rhabdomyosarcoma

52
Q

benign tumor of adipose

A

lipoma

53
Q

how does duchenne muscular dystrophy present?

A

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
Q

most common malignant soft tissue tumor in children

A

rhabdomyosarcoma

55
Q

strap cell mimicking skeletal muscle cross striations

A

rhabdomyosarcoma indicator

57
Q

eaton lambert syndrome ________ with repetitive stimulation

A

improves

58
Q

degenerative disorder characterized by muscle wasting and replacement of skeletal muscle by adipose tissue

A

x linked muscular dystrophy

59
Q

herringbone pattern in histology

A

fibrosarcoma

60
Q

benign tumor of skeletal muscle

A

rhabdomyoma

61
Q

What is dystrophin?

A

cytoplasmic protein that anchors the muscle fiber cytoskeleton to the extracellular matrix

62
Q

autoantibodies against AcH receptors

A

myasthenia gravis

63
Q

rhabdomyosarcoma: characteristic cell? desmin + or -? most common site?

A

rhabdomyoblast

desmin +

head and neck (vagina for young girls)

64
Q

amyotrophic lateral sclerosis (ALS)

A

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

MSK Part 2 (nlg) (9 decks)

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