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Gen. Path > Musculoskeletal System > Flashcards

Musculoskeletal System Flashcards

1
Q

What is the brittle bone disease?

A

Osteogenesis Imperfecta

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2
Q

What are the features of osteogenesis imperfecta due solely to?

A

Defective collagen synthesis

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3
Q

What type of osteogenesis imperfecta is the most severe?

A

Type II

Usually lethal with 50% stillborn due to multiple fractures in the body

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4
Q

What type of osteogenesis imperfecta is the most common? Is is severe or mild?

A

Type I

The most common and mildest

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5
Q

What are some features of type I osteogenesis imperfecta?

A
  • Variable inheritance
  • Blue sclera, hearing defects
  • Fractures at birth in 10%
  • Mild long bone deformities
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6
Q

What type of osteogenesis imperfecta is most common in children?

A

Type I

Note: not on slides but she said verbally

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7
Q

What type of osteogenesis imperfecta occurs beyond the perinatal period?

A

Type III

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8
Q

What are some features of type IV osteogenesis imperfecta?

A

Bone fragility only, without other classic features

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9
Q

What are some features of type III osteogenesis imperfecta?

A
  • Moderate to severe fractures

- Cardiopulmonary complications

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10
Q

What are some classic features of osteogenesis imperfecta in general?

A
  • Fragile bones
  • Numerous fractures
  • Blue sclera
  • Lax ligaments
  • Hearing loss
  • Dentinogenesis imperfecta/Opalescent dentin
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11
Q

What is the difference between dentinogenesis imperfecta and opalescent dentin?

A

When the oral manifestation is apparent WITHOUT other features of osteogenesis imperfecta = dentinogenesis imperfecta
When the oral manifestation is apparent WITH other features of osteogenesis imperfecta = opalescent dentin

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12
Q

What are some oral manifestations of osteogenesis imperfecta?

A
  • Teeth are fray-amber-yellow
  • Enamel is normal, dentin is soft
  • Severe attrition
  • Bell shaped crowns with thin short roots
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13
Q

Do people with osteogenesis imperfecta oral manifestations have an increased caries risk?

A

No increased susceptibility to caries

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14
Q

Are oral manifestations more common in osteogenesis imperfecta patients on their primary teeth or their permanent teeth?

A 
More common (about 80%) on primary teeth
Only about 33% of patients show on their permanent teeth.
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15
Q

What is another name for Osteopetrosis?

A

Marble Bone Disease or Albers-Schonberg Disease

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16
Q

Is osteopetrosis acquired or inherited?

A

It is a rare hereditary condition

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17
Q

What is the cause of osteopetrosis?

A

Osteoclasts don’t remodel properly

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18
Q

What are some features of osteopetrosis?

A
  • Bones are dense, brittle
  • Fractures
  • Growth retardation
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19
Q

What is the name of the most common type of bacterial infection that develops in the type of bone formed by osteopetrosis?

A

Osteomyelitis

-poor vascularity

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20
Q

What is the histology of osteopetrosis?

A
  • Sclerotic bone

- Obliterated marrow spaces

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21
Q

Is osteoporosis acquired or inherited?

A

Acquired bone disease

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22
Q

What are some features of osteoporosis?

A
  • Reduced bone mass
  • Increased bone fragility
  • Broken bones (mainly occurs in the hip)
  • Localized OR a manifestation of a metabolic bone disease
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23
Q

What two groups is osteoporosis commonly found in?

A

Senile - all aging individuals

Postmenopausal- only women, after menopause

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24
Q

What causes osteoporosis in postmenopausal women?

A

Decreased serum estrogen.
Increased IL-1, IL-6, TNF levels with increased expression of RANK and RANKL.
INCREASED osteoclast activity.

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25
Q

What are some causes of osteoporosis in senile individuals?

A
  • Decreased replicative ability of osteoprogenitor cells
  • Decreased synthetic ability of the osteoblasts
  • Decreased biologic ability of matrix-bound growth factors
  • Reduced physical activity
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26
Q

What is osteomyelitis?

A

An acute or chronic inflammatory process of the bone

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27
Q

What is a possible drainage in osteomyelitis?

A

Sinus tracts may drain to the skin

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28
Q

What are 3 predisposing factors to osteomyelitis?

A
  • Compromised blood supply (like in osteopetrosis)
  • Lowered host resistance
  • Virulent organisms (usually bacteria)
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29
Q

What are the “specific” predisposing factors to osteomyelitis that increases the susceptibility to develop?

A
  • Tobacco/Alcohol
  • Diabetes Mellitus
  • AIDS
  • Radiation therapy
  • Osteopetrosis/ other bone disorders
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30
Q

How long are the signs and symptoms of acute osteomyelitis in duration for?

A

Typically less than 1 month

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31
Q

What are the signs and symptoms for acute osteomyelitis?

A

Fever
Sensitivity
Swelling
Lymphadenopathy

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32
Q

How does the X-ray look for acute osteomyelitis?

A

Unremarkable or an ill-defined radiolucency

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33
Q

What is a sequestrum?

A

A fragment of necrotic bone

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34
Q

What is rarely seen in acute osteomyelitis?

A

Paresthesia
Drainage
Exfoliation of fragments of necrotic bone

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35
Q

What does the histopathology for acute osteomyelitis contain?

A

Lots of NEUTROPHILS (acute = neutrophils)

Empty lacunae

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36
Q

What will be viewed in chronic osteomyelitis?

A

Dead bone “islands”
Repair - fibrosis and new bone
Chronic inflammation

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37
Q

What is contained in dead bone islands?

A

Dead bone with formation of new vital bone around
Dead bone = detached sequestrum
Live bone = Involucrum

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38
Q

What is another name for Osteitis Deformans?

A

Paget’s Disease of Bone

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39
Q

What is the reason for signs and symptoms of Paget’s Disease of Bone?

A

Abnormal resorption and deposition of bone. Net effect is deposition. Distorts and weakens the bones.

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40
Q

Who is affected by Paget’s Disease of Bone?

A

Affects older people. Rarely seen in people below 40

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41
Q

What is the cause of Paget’s Disease of Bone?

A

Cause is unknown. Possibilities: Inflammatory, genetic, endocrine, viral factors

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42
Q

Are most cases of Paget’s Disease of Bone monoostotic or polyostotic?

A

Most cases are polyostotic

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43
Q

What are some signs and symptoms of Paget’s Disease of Bone?

A
  • severe bone pain
  • teeth may migrate and loosen
  • jaw(s) may enlarge
  • comments about needing a bigger hat or denture
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44
Q

How does Paget’s Disease of Bone appear on radiographs initially?

A

Patchy sclerotic area- “Cotton wool” appearance because of the excess formation and resorption initially.

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45
Q

What happens to the cementum in Paget’s Disease of Bone?

A

Hypercementosis- cementum gets bigger in size

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46
Q

Which arch is more likely to increase in size in Paget’s Disease of Bone?

A

Maxilla

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47
Q

What happens to the alveolar ridges in Paget’s Disease of Bone?

A

Alveolar ridges are grossly and symmetrically enlarged.

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48
Q

What will a lab test find in blood of patient with Paget’s Disease of Bone?

A

Elevated alkaline phosphatase- indicates increased osteoblastic activity

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49
Q

What are some microscopic findings in Paget’s Disease of Bone?

A

Mosaic bone

Basophilic reversal lines

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50
Q

What is the treatment for Paget’s Disease of Bone?

A

No treatment is usually required if there is limited involvement and no symptoms. The disease is usually chronic, slowly progressive and seldom causes death. Calcitonin can be used to stop remodelling.

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51
Q

What does Paget’s Disease of Bone put the patient at increased risk for?

A

Osteosarcoma.

52
Q

What is an osteoma composed of? Malignant or benign?

A

Benign tumors. Composed of mature compact or cancellous bone.

53
Q

What are histologically identical to osteomas but not considered osteomas themselves?

A

Platal and mandibular tori and buccal exostoses.

54
Q

What syndrome did we learn about that has multiple osteomas?

A

Gardner Syndrome

55
Q

What is the most common area for an osteoma?

A

Head and neck area-craniofacial skeleton

56
Q

Where is a periosteal osteoma?

A

Arises on surface of bone, as polypoid or sessile mass

57
Q

Where is endosteal osteoma?

A

In medullary bone

58
Q

What age range are osteoma more common?

A

Young adults

59
Q

Are osteomas always symptomatic? How would you treat?

A

They can be asymptomatic and solitary. Larger osteomas can cause symptoms and/or cosmetic deformities, can be fixed with conservative surgical excision. Smaller osteomas are just left and periodically observed.

60
Q

What is osteoblastoma? Malignant or benign?

A

Benign tumors arising from osteoblasts.

61
Q

How do osteoblastomas and osteomas differ?

A

Osteoblastoma pain is not relived with aspirin, osteoid osteoma pain IS relived with aspirin.
Osteoblastoma size is greater than 2 cm. Osteoid osteoma size is less than 2 cm.`

62
Q

Is osteosarcoma malignant or benign?

A

Bone malignancy.

63
Q

What groups is osteosarcoma more common in?

A

More common in males and in teens to early 20s.

64
Q

What is the number one site of osteosarcoma?

A

The knee: proximal tibia, distal femur.

65
Q

What is the presenting symptom of osteosarcoma?

A

Pain and swelling

66
Q

What is the difference between primary and secondary osteosarcoma?

A

Primary arises on its own. Secondary develops from another condition, making that condition a higher risk for developing osteosarcoma.

67
Q

What are the 3 secondary causes of osteosarcoma?

A
  • Paget’s
  • Radiation
  • Fibrous dysplasia
68
Q

What is the genetic predisposition to osteosarcoma?

A

Retinoblastoma p53 gene

69
Q

Who has a worse prognosis for osteosarcoma?

A

Older patients

70
Q

What is the radiographic appearance of osteosarcoma?

A
  • Sunburst appearance
  • Radiolucency and radiopacity
  • Mottled borders
71
Q

Can osteosarcoma occur in the jaw? Is there a mean age?

A

Yes, 7% occur in jaws. Mean age is 10-15 years older than the mean age for development in the long bones = 33

72
Q

What periodontal changes occur in osteosarcoma?

A
  • Resorption of roots of teeth

- Early radiographic change symmetric widening of periodontal ligament space

73
Q

What are the 3 microscopic types of osteosarcoma?**

A

Fibroblastic
Chondroblastic
Osteoblastic

74
Q

What is common to all 3 types of osteosarcoma?

A

All contain malignant osteoid, just the amount differs

75
Q

What does fibroblastic osteosarcoma produce?

A

Collagen

76
Q

What does chondroblastic osteosarcoma produce?

A

Cartilage

77
Q

What does osteoblastic osteosarcoma produce?

A

Osteoid or bone

78
Q

What is chondrosarcoma?

A

Sarcoma of chondroblasts

79
Q

Is the pain less or worse in chondrosarcoma compared to osteosarcoma?

A

Pain less often than osteosarcoma

80
Q

What percentage of bone malignancies are chondrosarcoma?

A

20%

81
Q

How does chondrosarcoma appear on a radiograph?

A

Radiolucent lesion with poorly defined borders, contain radiopaque foci

82
Q

What groups is chondrosarcoma most common in? How does this compare to osteosarcoma?

A

More common in males (same as with osteosarcoma). Age range of 50-70 (older than in osteosarcoma).

83
Q

Where is the #1 site of chondrosarcoma?

A

Pelvis

84
Q

Where can chondrosarcoma be found orally?

A

Anterior jaws

Condyle

85
Q

What is the most common bone malignancy in children?**

A

Ewing’s Sarcoma**

86
Q

What is Ewing’s Sarcoma?

A

Distinctive primary malignant tumour of bone of uncertain histogenesis.

87
Q

How is Ewing’s Sarcoma believed to be caused?

A

Neuroectodermal derivation. Translocation of chromosomes 11 and 22**

88
Q

Is the jaw involved in Ewing’s Sarcoma? Which arch is more common?

A

Uncommon. Mandible is more common than maxilla though.

89
Q

Where are common sites of Ewing’s Sarcoma?

A

Femur, pelvic bones

90
Q

What is the characteristic reaction seen in Ewing’s Sarcoma?

A

Characteristic onion-skin periosteal reaction of long bones

91
Q

What is the survival rate like in Ewing’s Sarcoma?

A

40-80%

92
Q

What are some signs and symptoms of Ewing’s Sarcoma?

A

Fever
Leukocytosis
Elevated erythrocyte sedimentation rate
Pain and swelling

93
Q

What is fibrous dysplasia?

A

A developmental tumour-like lesion. Bone is replaced with fibrous tissue.

94
Q

Is monostotic (involving one bone) or polystotic (multiple bones) fibrous dysplasia more common?

A

Monostotic disease in 80-85%

95
Q

Where is monostotic fibrous dysplasia found?

A

Jaws

Ribs

96
Q

Where is polyostotic fibrous dysplasia found?

A

Long bones

97
Q

Is fibrous dysplasia malignant?

A

There is a rare chance of malignant transformation

98
Q

What are the 2 polyostotic fibrous dysplasia conditions?**

A

Jaffe-Lichenstein**

McCune-Albright Syndrome**

99
Q

How do the 2 polyostotic fibrous dysplasia conditions compare?

A

McCune-Albright is more severe and diffuse.
Both contain deformation of multiple bones (polyostotic) and both contain cafe au lait spots.
McCune-Albright has endocrine disturbances like precocious puberty and pituitary adenoma.

100
Q

Is it better to diagnose fibrous dysplasia from biopsy or radiograph?

A

Radiograph

101
Q

What is the X-ray appearance of fibrous dysplasia?

A

Ground glass radiopacity*
Mottled lesion
Hard to see border

102
Q

What are the 2 types of muscle atrophy and their cause?

A

Neurogenic Atropy- loss of nerve supply resulting from something like spinal cord trauma
Simple Disuse Atropy- prolonged bed rest, immobilization

103
Q

What is Myasthenia Gravis? What does it target?

A

An autoimmune disorder of neuromuscular junction. Circulating antibodies that target/receptor of AChRs (acetylcholine)

104
Q

What is the result of Myasthenia Gravis?

A

Muscle weakness. Skeletal muscle only.

105
Q

What gender is Myasthenia Gravis more common in?

A

Females, like most autoimmune disorders

106
Q

What is the #1 cause of Myasthenia Gravis that starts the production of the auto-antibodies?

A

90% thymic disorders of thymoma

107
Q

What are some signs and symptoms of Myasthenia Gravis?

A

Abnormal muscle fatigue as the day goes on.

Small muscles of head and neck will go first- eye focus, swallowing, slurred speech

108
Q

What is muscular dystrophy?

A

Progressive degeneration of skeletal muscle. Nerves are intact. Muscle weakness and wasting.

109
Q

What are the 2 most common muscular dystrophies? What gender is are they more common in?

A

Duchene
Becker
More common in males because X-linked

110
Q

Is Becker or Duchene more common? Which is more severe?

A

Duchene is more common.

Duchene is more severe.

111
Q

What gene is affected in both Duchene and Becker?

A

Dystrophin gene on X chromosome. Responsible for the protein called dystrophin, which is needed for attachment of muscle to the skeleton.

112
Q

Is heart involvement found in Duchene and/or Becker?

A

Heart involvement in Duchene.

May of may not have hear involvement in Becker.

113
Q

When is Duchene seen?

A

Early life, delay in walking. Will die in 20s from respiratory failure, pneumonia, CHF.

114
Q

When is Becker seen?

A

Symptoms are seen later in life. Onset is usually in 20s and often dies in 50s.

115
Q

What is a benign tumour of adipose tissue called?

A

Lipoma

116
Q

Is lipoma common or rare?

A

Very common. Solitary and painless

117
Q

What is liposarcoma?

A

Malignancy of adipose differentiation.

118
Q

What age does liposarcoma peak? Where is it usually found?

A

Peak 50-60. Usually in legs.

119
Q

What is fibromatosis?

A

Aggressive, but benign fibroblast proliferations.

120
Q

Where can fibromatosis be found?

A

Superficial- hands, feet, penis, knuckles, neck
Deep- Called demoed tumours: abdomen, trunk, extremities.
Superficial are less aggressive than deep.

121
Q

What is fibrosarcoma?

A

Fibroblastic malignancy

122
Q

Age range for fibrosarcoma? Where is it common and how is the onset?

A

30-55 years
Thigh, trunk, forearm
Slow, painless onset

123
Q

What is rhabdomyosarcoma?

A

Malignancy of skeletal muscle.

124
Q

What is the most common soft tissue malignancy in children/young individuals?

A

Rhabdomyosarcoma

125
Q

What are the 3 types of rhabdomyosarcoma and how are they classified?

A

Classified based on appearance histopathologically.
Embryonal: 0-15 years, botryoid subtype looks like grapes.
Alveolar: 10-25 years
Pleomorphic: Older adults, 45+ years

126
Q

Which type of rhabdomyosarcoma has the worst prognosis?

A

Pleomorphic

Gen. Path (7 decks)

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