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MSK SB > Pathology > Flashcards

Pathology Flashcards

1
Q

what are the types of skeletal muscle fibres

A

Red fibres i.e. Type 1, slow twitch.
- large mitochondria and increase myoglobin

White fibres i.e. Type 2, fast twitch
- small mitochondria and large motor end plates

Intermediate fibres

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2
Q

what are indications for muscle biopsy

A

evidence of muscle disease (elevated CK, weakness)
Presence of neuropathy
Presence of vascular disorder (vasculitis)

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3
Q

when are CK levels 20-30 times of normal

A

inflammatory myopathy

intermediate levels of CK

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4
Q

what are changes seen in muscles with dystrophic diseases

A 
variability in muscle fibre size
endomysial fibrosis
fatty infiltration and replacement
myocyte hypertrophy 
Increased central nuclei
Segmental necrosis
Regeneration
Ring fibres- condensation
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5
Q

what are common muscular dystrophies and what do they affect

A

Duchenne - pelvic girdle
Becker - pelvic girdle
Limb girdle - pelvic girdle
Myotonic dystrophy - face, respiratory, limbs

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6
Q

typical case of DMD

A

present in boys 2-4 years of age
proximal weakness
pseudo hypertrophy of calves
very raised levels of CK

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7
Q

where is the mutation in DMD and what does it cause

A

in dystrophin gene on long arm chromosome X
causes alterations in anchorage of actin cytoskeleton basement membrane
fibres liable to tearing
uncontrolled calcium entry into cells

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8
Q

what are the characteristics of BMD

A

later onset and milder symptoms that DMD
mutations in the dystrophin gene
slower progress, not as severe as DMD
more calf enlargement

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9
Q

what are the features of Myotonic dystrophy

A

Muscle weakness, myotonia, non-muscle features
Most common dystrophy to affect adults
Adolescence – face predominantly affected, distal limbs
Later – respiratory muscles most affected

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10
Q

what are examples of inflammatory myopathies

A

infective agents
polymyositis
dermatomyositis

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11
Q

what is polymyositis

A

chronic inflammatory disease

progressive muscular weakness, pain and tenderness

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12
Q

pathology of polymyositis

A

Cell-mediated immune response to muscle antigens
Endomysial lymphocytic infiltrate, invasion of muscle by CD8 + T lymphocytes
Segmental fibre necrosis

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13
Q

what is dermatomyositis

A

skin changes plus polymyositis
upper body erythema
swelling of eyelids with purple discolouration

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14
Q

pathology of dermatomyositis

A

Immune complex and complement deposition within and around capillaries within muscle
Perifascicular muscle fibre injury
B-lymphocytes and CD4 + T cells > cf polymyositis

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15
Q

what is Motor Neuron Disease

A

progressive degeneration of anterior horn cells

Denervation atrophy, fasciculation and weakness

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16
Q

what is spinal muscular atrophy

A

inherited; autosomal recessive
Degeneration of anterior horn cells in spinal cord
Denervation of muscle
Types 1 - 4

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17
Q

features of myasthenia graves

A

Autoimmune
Weakness, proptosis, fatigue, and dysphagia
Women between 20 - 40

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18
Q

what is rhabdomyolysis

A

breakdown of skeletal muscle

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19
Q

what antibodies are associated with SLE

A

Antinuclear antibodies (ANAs)

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20
Q

symptoms/signs of SLE

A

Skin –“butterfly” rash (sun exposed areas), discoid lupus erythematosus (DLE)

Joints – arthralgia

Kidneys – glomerulonephritis

CNS – psychiatric symptoms, focal neurological symptoms

CVS – pericarditis, myocarditis, necrotising vasculitis

Lymphoreticular – lymphadenopathy & splenomegaly

Lungs – pleuritis, pleural effusions

Haematological – anaemia, leucopenia, thrombophilia

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21
Q

what hypersensitivity is responsible for SLE symptoms

A

Visceral lesions – mediated by Type III hypersensitivity

Haematological effects – mediated by Type II hypersensitivity

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22
Q

what is Polyarteritis nodosa

A

Inflammation and fibrinoid necrosis of small/medium arteries

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23
Q

symptoms and signs of PAN

A

Non-specific +/- organ specific features such as hypertension, haematuria, abdominal pain, melaena, diarrhoea, mononeuritis multiplex, rash, cough, dyspnoea

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24
Q

investigation/diagnosis of PAN

A

Biopsy; fibrinoid necrosis of vessels

Serum contains pANCA (perinuclear antineutrophil cytoplasmic autoantibody)

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25
Q

features of PMR

A

elderly
pain, stiffness in shoulder and pelvic girdles
no muscle weakness
responds to corticosteroids

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26
Q

features of Temporal arteritis

A

a.k.a GCA
inflammation affecting cranial vessels
Risk of blindness of terminal branches of opthalmic artery affected
Headache & scalp tenderness

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27
Q

diagnosis of temporal arteritis

A

raised ESR

temporal artery biopsy; inflammation +/- giant cells

28
Q

what is scleroderma

A

Excessive fibrosis of organs and tissues (excessive collagen production)

29
Q

symptoms/signs of scleroderma

A

Skin – tight, tethered, ↓ joint movement

GI tract – fibrous replacement of muscularis

Heart – pericarditis and myocardial fibrosis

Lungs – interstitial fibrosis

Kidneys – affects arteries, leads to hypertension

Musculoskeletal system – polyarteritis and myositis

30
Q

what is scleroderma associated with

A 
CREST syndrome
Calcinosis
Raynaud’s
Esophageal dysfunction
Sclerodactyly
Telangiectasia
31
Q

what can kill in scleroderma

A

Renal failure secondary to malignant hypertension
Sever respiratory compomise
Cor pulmonale
Cardiac failure or arrhythmias secondary to myocardial fibrosis

32
Q

what are the benign tumours of MSK

A

Osteochondroma
Chondroma
Osteoid osteoma
Chondroblastoma

33
Q

what is osteochondroma

A

Cartilage capped bony projection arising on external surface of bone containing a marrow cavity that is continuous with that of the underlying bone.

34
Q

features of osteochondroma

A

affects the young
M = F
tend to develop near epiphyses of long bones
cell of origin - chondrocytes

35
Q

what is a chondroma

A

Benign hyaline cartilage tumour arising in medullary cavity of bones of hands and feet

36
Q

what are the two types of chondroma

A

Single or multiple (Ollier’s disease and Mafucci’s syndrome)

37
Q

what is Ollier’s disease

A

Rare developmental disorder
Enchondromas (metaphyses and diaphyses)
Typically unilateral and involving one extremity

38
Q

what is Maffucci’s syndrome

A

Multiple enchondromatosis with soft tissue and visceral haemangiomas
greater malignant risk than Ollier’s

39
Q

what is an osteoid osteoma

A

benign osteoblastic tumour composed of a central core of vascular osteoid tissue and a peripheral zone of sclerotic bone

40
Q

features of osteoid osteoma

A

Benign - usually found in children and young adults
Found in femur, tibia, hands/feet, and axial skeleton (spine)
resolve w/out tx in average of 33 months

41
Q

symptoms of osteoid osteoma

A 
joint tenderness
swelling 
synovitis
dull pain
worse at night
pain relieved by aspirin and NSAIDs
42
Q

what are chondroblastoma

A

Benign cartilage tumour arising in bone

43
Q

what are features of chondroblastoma

A

found at epiphysis of long bones

20-30 y/o

44
Q

histology of chondroblastoma

A

Closely packed polygonal cells plus areas of immature chondroid.
Mitotic activity is low.
Distinct cytoplasmic borders with foci of “chicken-wire” calcification.

45
Q

tumours that are benign but locally aggressive

A

Giant cell tumour
Osteoblastoma
Chordoma

46
Q

features of giant cell tumour

A

cell of origin - osteoclast

site - long bones, often around knee

47
Q

radiology of giant cell tumour

A

Radiolucent with increasing density towards periphery.
Destruction of medullary cavity and adjacent cortex.
May expand into soft tissue - locally aggressive

48
Q

what are Osteoblastoma

A

Solitary, benign and self-limited tumour that produces osteoid and bone but can damage surrounding structures

49
Q

where are osteoblastomas found and what are the symptoms

A

metaphysis or diaphysis of long bones

pain of long duration, swelling, tenderness

50
Q

treatment of osteoblastomas

A

surgical resection by-

  • curettage
  • intralesional excision
51
Q

features of chordoma

A

Very rare tumour arising from notocord remnants (midline tumour, often in sacral region)
40+ years
Benign but locally destructive and invasive - due to areas affected can cause spinal problems and symptoms

52
Q

where do macroscopic tumours grow

A

often tracks along nerve roots in the sacral plexus or out the sciatic notch in planes of least resistance

53
Q

malignant MSK tumours

A

Osteosarcoma
Chondrosarcoma
Ewing’s sarcoma

54
Q

what is the commonest primary malignant tumour

A

osteosarcoma

55
Q

features of osteosarcoma

A

Malignant osteoblasts forming osteoid

Young adults; 60%

56
Q

sites of osteosarcoma

A

Ends of long bones particularly distal femur, proximal tibia, and proximal humerus

57
Q

osteosarcomas grow slowly - true or false

A

false

Rapid growth; mitotically active - patients may well already have metastasise when they present

58
Q

what is Codman’s triangle

A

raised triangle seen in aggressive bone lesions e.g. osteosarcoma, Ewing sarcoma

59
Q

osteosarcoma treatment

A

Biopsy, CT, bone scan
Pre-operative chemotherapy
Surgical resection
Post-operative chemotherapy

60
Q

what is a chondrosarcoma

A

The second commonest primary malignant tumour of bone
Exhibits pure hyaline cartilage differentiation
Composed of malignant chondrocytes
can extend into soft tissue

61
Q

treatment of chondrosarcoma

A

Wide surgical excision - best option, but often impossible due to location of tumour
Limited use of chemotherapy and radiotherapy

62
Q

features of Ewing’s sarcoma

A

highly malignant
Mainly occurs in the metaphysis and diaphysis of femur, tibia, then humerus.
Most common in second decade of life.

63
Q

how does Ewing’s sarcoma appear histologically

A

small round blue cell

64
Q

treatment of Ewing’s sarcoma

A

surgery
radiation therapy
chemotherapy

65
Q

what cancers commonly cause bony mets

A

thyroid, breast, lung, kidney, prostate

66
Q

what is multiple myeloma

A

Malignant proliferation of plasma cells in bone marrow

67
Q

what does multiple myeloma often cause

A

renal failure

destruction of axial skeleton

MSK SB (15 decks)

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