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Bone > Dougherty Part 9 > Flashcards

Dougherty Part 9 Flashcards

1
Q

when and where does alveolar rhabdomyosarcoma present

A

early to mid-adolescence

deep muscle of extremities

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2
Q

what causes alveolar rhabdomyosarcoma?

A

t(2;13) or t(1;13)

PAX3 or PAX7 to FOXO1

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3
Q

what is seen in alveolar rhabdomyosarcoma

A

fibrous septa divide tumor into alveolar-like spaces centrally containing discohesive cells while peripheral cells stick to wall

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4
Q

pleomorphic rhabdomyosarcoma is presents where and when

A

deep soft tissue of adults

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5
Q

pleomorphic rhabdomyosarcoma is often mistaken for undifferentiated pleomorphic sarcoma. what differentiates the two

A

pleomorphic rhabdomyosarcoma is:
desmin positive
MyoD1 or myogenin positive

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6
Q

what are the different rhabdomyosarcoma prognosis’

A

best: sarcoma botryoides
second best: embryonal
poor: alveolar and pleomorphic
65% of children are cured

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7
Q

what is a leiomyoma

A

benign smooth muscle tumor

bland smooth muscle cells in fasicles

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8
Q

where is the most common site of a leimyoma

A

uterus

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9
Q

other than the uterus leiomyoma is found at what sites

A

skin: erector pili, nipples, scrotum, labia

pilar leiomyoma often painful and multiple

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10
Q

what is found in leiomyosarcoma and what tests is it negative for

A

smooth muscle actin and desmin
malignant spindle cells
negative for MyoD1 and myogenin

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11
Q

leiomyosarcoma can be found at what sites

A

skin, deep extremities, and retroperitoneum

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12
Q

synovial sarcoma is caused by what

A

t(X;18)

SS18-SSX fusion

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13
Q

synovial sarcoma is found in what age group and where

A

20-40s
deep and around large joints
60-70% lower extremities (KNEE/THIGH)

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14
Q

biphasic (dual) differentiation is what and seen in what

A

epithelial cells as glands, cords, or nests
spindle cells: cellular in fascicles
synovial sarcoma

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15
Q

what types of synovial sarcoma has what types

A

biphasic (dual) differentiation

monophasic (spindle cell) variant

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16
Q

what is seen in the monophonic (spindle cell) variant of synovial sarcoma

A

positive for cytokeratins (epithelial marker)

epithelial membrane antigen (EMA)

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17
Q

what are undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma)

A

group of aggressive neoplasms

found in proximal extremities and retroperitoneum

18
Q

what is seen in undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma)

A

cell pleomorphism and storiform pattern (diagnosis of exclusion)
large grey, hemorrhagic and/or necrotic

19
Q

what is in immunohistochemistry that is a marker of epithelium

A

epithelial membrane antigen

cytokeratin

20
Q

what is in immunohistochemistry that is a marker of skeletal muscle

A

MyoD1 and Myogenin

Actin and Desmin

21
Q

what is in immunohistochemistry that is a marker of smooth muscle

A

smooth muscle actin

Actin and Desmin

22
Q

what is in immunohistochemistry that is a marker of a non epithelial tumor

A

vimentin

23
Q

what is a myopathy

A

disorder of the muscles

24
Q

what is muscular dystrophy

A

inherited disorder

progressive muscle weakness and wasting

25
Q

what is segmental muscle necrosis

A

destruction of a portion of the myocyte length

26
Q

what is muscle regeneration

A

satellite cells reconstitute destroyed muscle

27
Q

what is fiber hypertrophy in response to

A

increased load (working out)

28
Q

what is myotonia

A

tonic spasm of one or more muscles

a condition characterized by such spasms

29
Q

what is hypotonia

A

deficient tone or tension

30
Q

what is arthrogryposis

A

fixation joints in an extended or flexed potions

31
Q

what is gowers sign and what does it indicate

A

use the hands and arms to “walk” up from a squatting position
indicates weakness of proximal muscles

32
Q

what is the pneumonic for type 1 fiber types and what does it mean

A 
"one slow fat red ox"
type 1 fibers
slow fibers
lipid rich (fat)
high myoglobin (red)
oxidative (ox)
33
Q

what is grouped atrophy

A

loss of motor neuron leads to atrophy of associated muscle

34
Q

what is denervation atrophy

A

disorder of motor neurons
breakdown of myosin and actin
resorption of myofibrils

35
Q

Spinal muscle atrophy (SMA) is what and cased by what

A

progressive distortion of anterior horn cells and cranial nerve motor neurons
AR- Survival Motor Neuron 1 (SMN1)

36
Q

what is seen histologically in spinal muscle atrophy (SMA)

A

panfascicular atrophy with scattered large fibers 2-4x normal size

37
Q

Duchenne Muscular Dystrophy (DMD) is caused by what

A

dystrophin defect from abnormal gene at Xp21 (deletion most of the time)
X-linked

38
Q

DMD manifests when and what is the timeline from there

A

manifests before 5
wheel chair by 12
death in 20s

39
Q

what does DMD present as

A

normal at birth
delayed walking then clumsy
weak pelvic then shoulder girdle
pseudo hypertrophy of calves as fivers increase in size
increased CK (returns to normal with muscle loss)

40
Q

what causes death in DMD

A

respiratory failure
cardiac decompensation
lung infection

41
Q

Becker Muscular Dystrophy is due to what

A

defect in dystrophin quality/quantity of protein from abnormal Xp21

42
Q

the onset of becker’s is what compared to DMD

A

backers has later onset, is less severe and less common

Bone (19 decks)

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