Chapter 14: Bone Pathology part I Flashcards

1
Q

what is necessary for diagnosis of focal osteoporotic marrow defects? what is the treatment?

A
  • incisional biopsy
  • no treatment necessary
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2
Q

how is idiopathic osteosclerosis diagnosed?

A
  • can be made based on history, clinical features, and radiographic findings
  • biopsy is considered only if there are symptoms, contingued growth, or cortical expansion
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3
Q

idiopathic osteosclerosis affects approximately ___% of americans

A

5%

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4
Q

___ is a group of heritable disorders characterized as a defect in type I collagen maturation

A

osteogenesis imperfecta

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5
Q

what is the treatment of bone anomalies in cleidocranial dysplasia?

A
  • no treatment
  • patients usually function well regardless
    • patients are usually unaware they have a syndrome
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6
Q

are focal osteoporotic marrow defects symptomatic? how are they usually discovered?

A

no, and they are typically found incidentally on radiographs

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7
Q

90% of idiopathic osteosclerosis cases are in what location? is there cortical expansion?

A
  • mandible
  • no cortical expansion
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8
Q

infantile osteopetrosis is a ___ disease

A

severe

associated with a poor prognosis

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9
Q

central giant cell granuloma is considered a ___ lesion, but some cases demonstrate aggressive behavior

A

nonneoplastic

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10
Q

what is the most common clinical manifestation of aneurysmal bone cysts?

A

swelling that has developed rapidly

pain is often reported

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11
Q

cherubism causes marked widening and distortion of ___, which may lead to ___

A
  • alveolar ridges
  • failure of tooth eruption amongst other things
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12
Q
A

osteogenesis imperfecta as characterized by bowed long bones

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13
Q

with jaw involvement of paget’s disease, is it more common in the maxilla or mandible? what is the presentation?

A
  • maxillary disease > mandibular disease
  • enlargement of middle 1/3 of the face; severe cases results in leontiasis ossea, or lionlike facial deformity
  • alveolar ridges are symmetrical and grossly enlarged
    • patients classically complain dentures no longer fit (or, if present, increased space between teeth)
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14
Q

paget’s disease affecting the skull leads to a progressive increase in ___

A

the circumference of the head

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15
Q

describe benign bone neoplasia

A
  • asymptomatic
  • symmetrical
  • does not metastasize
  • grows slowly and by expansion; displaces teeth and expands the cortex
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16
Q

patients with osteopetrosis discovered at what time in life will have malignant osteopetrosis?

A

at birth or early infancy

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17
Q

what are the radiographic features of central giant cell granuloma? are radiographs diagnostic?

A
  • radiolucent lesions which may be multilocular or unilocular
  • typically well-delineated
  • noncorticated margins
  • not diagnostic
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18
Q
A

cherubism

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19
Q

what is the prognosis of central giant cell granuloma? is there a risk for metastasis?

A
  • long term prognosis is good
  • no risk for metastasis
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20
Q

describe malignant bone neoplasia:

is it symptomatic or asymptomatic? grows slowly or quickly? symmetrical or asymmetrical? capable of metastasis?

A
  • usually symptomatic
  • grows more rapidly than benign
  • often asymmetrical
  • capable or metastasis
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21
Q

how are most central giant cell granulomas discovered?

A
  • most are asymptomatic and discovered during routine xray or due to painless bone expansion
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22
Q

what is the prognosis of aneurysmal bone cysts?

A

good

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23
Q
A

osteopetrosis

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24
Q

development of osteosarcoma occurs in up to ___% of paget’s disease patients. most develop in what bones? what is the prognosis if an osteosarcoma develops?

A
  • 10%
  • most develop in the pelvis or long bones of lower extremities
  • very aggressive and associated with a poor prognosis
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25
Q

radiographically, how does paget’s disease present?

A
  • patchy sclerotic areas = cotton wool appearance
  • teeth often have hypercementosis
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26
Q

osteopetrosis is also called ___

A

marble bone disease

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27
Q

most aneurysmal bone cyst jaw lesions are found in patients around what age and what location? male or female predilection?

A
  • 20 years
  • posterior mandible
  • M = F
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28
Q

jaw involvement of paget’s disease occurs in ___% of cases

A

20%

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29
Q

___ is an area of hematopoietic marrow that produces a radiolucency

A

focal osteoporotic marrow defect

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30
Q

patients with paget’s disease will have high elevations in ___ and normal levels of ___ and ___

A
  • serum alkaline phosphatase
  • normal calcium and phosphorus
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31
Q

what are the radiographic features of osteogenesis imperfecta?

A
  • osteopenia (low bone density)
  • bowing
    • angulation or deformity of long bones
    • multiple fractures
    • wormian bones in the skull
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32
Q

microscopic findings of cherubism are identical to those found in ___

A

central giant cell granuloma

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33
Q

osteopetrosis results from a defect in remodeling caused by ___

A

a failure of normal osteoclast function

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34
Q

cherubism occurs between what ages? how does it progress?

A
  • 2-5 years old
  • clinical alterations progress until puberty, then stabilize and slowly regress
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35
Q

is an aneurysmal bone cyst a true cyst?

A

no; there is no epithelial lining

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36
Q

what is the treatment of paget’s disease? what cases is treatment unnecessary?

A
  • not given in asymptomatic patients with limited involvement
  • NSAIDs are given for pain
  • pharmacologic antiresorptive agents are given for symptomatic patients or those with alkaline phosphatase levels 50% above normal
    • bisphosphonates often used
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37
Q

___ is a common complaint of paget’s disease

A
  • bone pain
    • pagetic bone forms near joints and promotes osteoarthritic changes, with associated joint pain and limited mobility
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38
Q

aneurysmal bone cysts are typically seen in ___ bones; it is uncommon in the ___

A
  • long
  • jaw
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39
Q

what do mandibular lesions look like in cherubism? what about maxillary?

A
  • painless, bilateral, posterior, and expansile
  • maxillary involvement occurs posteriorly as well
    • in severe cases, entire maxilla and mandible are involved
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40
Q

which of the 4 major types of osteogenesis imperfecta is characterized as not compatible with life

A

type II

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41
Q
A

osteogenesis imperfecta as characterized by wormian bones of the skull (sutures)

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42
Q

what are the radiographic findings of traumatic bone cysts?

A
  • well-delineated
  • radiolucent
  • margins can be ill- or well-defined
  • when several teeth are involved, the defect shows domelike projections that scallop between the roots; this feature is highly suggestive
  • radiographic findings are suggestive but not diagnostic
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43
Q

what type of treatment is contraindicated in aneurysmal bone cyst cases?

A

irradiation

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44
Q

patients with cleidocranial dysplasia will have prolonged retention of ___, and delayed or failur of eruption of ___

A
  • deciduous teeth
  • permanent teeth
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45
Q

development of ___ is a recognized complication of paget’s disease

A

osteosarcoma (malignant bone tumor)

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46
Q

what 6 things does infantile osteopetrosis result in?

A
  • marrow failure
  • frequent fractures
  • cranial nerve compression
  • facial deformities
  • delayed tooth eruption
  • osteomyelitis post tooth infraction
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47
Q

describe the clavicle characteristics in cleidocranial dysplasia

A
  • clavicles are usually present and show varying degrees of hyploplasia
    • clavicles are absent in 10% of cases
    • unusual mobility of shoulders
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48
Q

___% of adult osteopetrosis are asymptomatic

A

40%

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49
Q

___ is a skeletal disorder with a marked increase in bone density

A

osteopetrosis

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50
Q

cherubism is a developmental jaw condition with autosomal ___ inheritance

A

dominance

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51
Q

the histopathology of central giant cell granuloma is identical to ___ and ___. what is the histology?

A
  • a brown tumor of hyperparathyroidism (so patients MUST be evaluated for this condition) and lesions of cherubism
  • histology is a cellular, vascular stroma with prominent multinucleated giant cells
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52
Q

describe the prognosis of a patient with cherubism

A

unpredictable

  • usually, the lesions show varying degress of remission and involution after puberty
  • by age 30, most patient’s facial features are normal
  • however, some patients are left with facial deformities
  • early surgical intervention with curettage has lead to both good results or rapid regrowth with worsening deformity; therefore, therapy hasn’t been determined
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53
Q

what 3 things can idiopathic osteosclerosis be confused with, and what are the distinguishing features of each?

A
  • condensing osteitis - associated with an infection
  • focal cemento-osseous dysplasia - has radiolucent rim
  • cementoblastoma - fused with the tooth
54
Q

radiation therapy is contraindicated for patients with cherubism due to risk of ___

A

postirradiation sarcoma

55
Q

in osteogenesis imperfecta cases, upon fractures, what is the healing process?

A

healing occurs, but it may heal inappropriately

56
Q

what are the radiographic features of cherubism?

A
  • multilocular
  • expansile
  • radiolucent
    • the appearance is virtually diagnostic due to the bilateral location in a young person
57
Q

what is the treatment for osteopetrosis?

A
  • bone marrow transplant is the only hope
  • only 50% of those eligible survive the transplant
58
Q

what are the radiographic features of infantile osteopetrosis?

A
  • widespread increase in skeletal density (increased radiopaque)
  • roots of teeth are difficult to visualize because of the density of the surrounding bone
59
Q

a minority of central giant cell granuloma cases are aggressive and associated with what 3 things?

A

pain, parasthesia, and perforation of the cortical plate

60
Q
A

osteopetrosis

61
Q

what are the two major clinical patterns of osteopetrosis?

A

infantile and adult

62
Q

___ is the most common type of inherited bone disease

A

osteogenesis imperfecta

63
Q

what is the etiology of aneurysmal bone cysts?

A

unknown

64
Q

how are traumatic bone cysts treated? what is the prognosis?

A
  • surgical exploration is curative; there will be rapid obliteratoin of the defect by new bone formation
  • periodic xrays should be taken until complete resolution has occurred; the prognosis is excellent
65
Q

why is a traumatic bone “cyst” a misnomer?

A

it is not a true cyst because the lesion does not have an epithelial lining

66
Q

malignant bone neoplasia invade and destroy ___

A

adjacent structures (cortex)

ragged/poorly defined margins

67
Q
A

focal osteoporotic marrow defect

68
Q

adult osteopetrosis is also called ___

A

benign osteopetrosis

69
Q

numerous unerupted permanent and supernumerary teeth are common in what condition?

A

cleidocranial dysplasia

70
Q

how are traumatic bone cysts diagnosed?

A
  • surgical exploration is necessary
    • little or no tissue will be obtained, so the diagnosis is made with the clinical and radiographic features together with surgical findings
    • during surgery, the wall of the cavity should be curetted to rule out thin-walled lesions
71
Q

which of the 4 major types of osteogenesis imperfecta is characterized as the most common, mildest, blue sclera throughout life

A

type I

72
Q

what is the goal of therapy for osteogenesis imperfecta?

A

symptomatic treatments and management of fractures

73
Q

most conditions of idiopathic osteosclerosis arise in ___ years and remain ___

A

teenage, static

74
Q

how are aneurysmal bone cysts treated?

A
  • curettage or enucleation
    • surgical defect typically heals within 1 year without the need of bone grafting
    • recurrence ranges from 10-60% depending on the study and is usually due to inadequate removal of the first lesion
75
Q

what are the distinctive, occasional findings noted in the oral cavity of patients with osteogenesis imperfecta?

A
  • teeth with a blue to brown translucence
  • premature pulpal obliteration
  • class III malocclusion
76
Q

is there jaw expansion associated with focal osteoporotic marrow defects?

A

no

77
Q

___ is a relatively common disease characterized by abnormal resorption and deposition of bone

A

paget’s disease of bone

78
Q

central giant cell granulomas are more common in what location of the jaw?

A

anterior jaw (more common in mandible) and frequently crosses the midline

79
Q

how do focal osteoporotic marrow defects present radiographically?

A
  • radiolucent lesion that varies in size
  • typically has ill-defined borders with fine central trabeculations
80
Q

likely filled with blood

no epithelial lining

A

aneurysmal bone cyst

81
Q

what is the exposed sclera caused by in cherubism?

A
  • involvement of the infraorbital rim and orbital floor = eyeballs tilted upward
  • stretching of the upper facial skin = pulls lower lid downward
82
Q

___ is a focal area of increased radiodensity that is of unknown cause and cannot be attributed to anything else

A

idiopathic osteosclerosis

83
Q

what are significant complications of adult osteopetrosis?

A

fracture and osteomyelitis after tooth extraction

84
Q
A

osteogenesis imperfecta as characterized by blue sclera

85
Q

which of the 4 major types of osteogenesis imperfecta is characterized as the most severe form, 1/3 survive into adulthood

A

type III

86
Q

what is the treatment of idiopathic osteosclerosis if the lesion is discovered during adolescence?

A
  • periodic xrays are prudent until the area stabilizes
  • after that, no treatment is necessary
87
Q

the radiographic features of focal osteoporotic marrow defect are similar to ___, but it is actually not a ___

A
  • intraosseious neoplasm
  • it is not a pathology
88
Q

in the jaws, traumatic bone cysts are found in pateints of what age? which jaw? are they symptomatic?

A
  • 10-20 years old
  • almost exclusively in the mandible
  • asymptomatic; 20% have swelling
89
Q
A

central giant cell granuloma

90
Q

what is the appearance of someone with cleidocranial dysplasia?

A
  • short stature; big head
  • pronounced frontal bossing
  • ocular hypertelorism
  • broad base of nose
91
Q

likely an empty cavity

no epithelial lining

A

traumatic bone cyst

92
Q

most cases of central giant cell granuloma occur before what age? male or female? mandible or maxilla?

A
  • before age 30
  • F>M
  • mandible > maxilla
93
Q

what are the signs/symptoms of osteogenesis imperfecta?

A
  • bone fragility
  • blue sclera
  • altered teeth
  • hearing loss (hypoacusis)
  • long bone and spine deformities
  • joint hyperextensibility
94
Q

75% of focal osteoporotic marrow defects occur in males or females? typically in what location?

A
  • females
  • typically in posterior mandible
95
Q

what is common during surgery of an aneurysmal bone cyst?

A

venous blood wells up and there may be bleeding

appearance is like a blood-soaked spongue

96
Q

___ is a syndrome complex characterized by dental and clavicle abnormalities (hyploplastic clavicles)

A

cleidocranial dysplasia

97
Q

the majority of traumatic bone cysts are found where?

A

in long bones

98
Q

most patients with infantile osteopetrosis die during the first ___ of life

A

decade

99
Q
A

paget’s disease of bone

100
Q

cleidocranial dysplasia occurs in 1 out of every ___ people

A

1,000,000

101
Q
A

cleidocranial dysplasia

102
Q

how does adult osteopetrosis compare to the infantile form?

A
  • is it discovered later in life and has less severe manifestation
  • disease is mild and has long-term survival
  • marrow failure is rare
103
Q

traumatic bone cyst is also called a ___

A

simple bone cyst

104
Q

which of the 4 major types of osteogenesis imperfecta is characterized as resembling type I, slightly more severe (more fractures)

A

type IV

105
Q

what is the most widely accepted etiology of traumatic bone cyst?

A
  • trauma-hemorrhage theory
    • trauma to the bone which is insufficient to cause a fracture, resulting in intraosseous hematoma
    • if the hematoma does not undergo organization and repair, it may liquefy and result in a defect
106
Q

what is the treatment of central giant cell granuloma? what is the recurrence?

A
  • curettage with a recurrence of 20%
    • recurrence likelihood is greater in young patients
    • most recurrent lesions resond to further curettage
  • new treatments are used with aggressive tumors, such as injections of corticosteroids
107
Q
A

paget’s disease of bone

108
Q

what are the characteristic facial features of charubism?

A
  • bilateral involvement of the posterior mandible that produces chubby cheeks
  • wide rim of exposed sclera below the iris causes an “eyes upturned to heaven” appearance
109
Q

what are characteristics of bone in osteogenesis imperfecta?

A
  • thin cortex
  • fine trabeculation
  • diffuse osteoporosis
110
Q

patients with cleidocranial dysplasia have ___ shaped teeth

A

abnormally

111
Q
A

central giant cell granuloma

112
Q

what are the most commonly affected bones in paget’s disease of bone?

A
  • vertebrae
  • pelvis
  • skull
  • femur
113
Q

___ is an intraosseous accumulation of blood-filled spaces surrounded by connective tissue

A

aneurysmal bone cyst

114
Q
A

osteogenesis imperfecta as characterized by brown translucence of teeth and radiographs showing shell teeth with thin dentin and enamel of normal thickness

115
Q

how does idiopathic osteosclerosis present radiographically?

A
  • well-defined, round or elliptical, radiopaque
  • usually associated with root apex, 20% aren’t
  • vary from 3mm to 2cm in greatest diameter
  • there is NO radiolucent rim surrounding the lesion
116
Q

in paget’s disease, involvement of weight-bearing bones leads to ___, resulting in a ___ stance

A
  • bowing
  • simian (monkeylike)
117
Q

osteogenesis imperfecta affects 1 in ___

A

8000

118
Q

paget’s disease is polyostotic. what does that mean?

A

it affects more than one bone

119
Q

what medications might patients with osteogenesis imperfecta be on?

A

bisphosphonates

reserved for moderately to severely affected patients

120
Q

what is the cause of paget’s disease?

A

unknown

121
Q

paget’s disease of bone result in what?

A

distortion and weakening of affected bones

122
Q

what population does paget’s disease often affect?

A

M>F

caucasians > african americans

1/125 people > 45

123
Q

___ is a benign, empty or fluid filled cavity within bone

A

traumatic bone cyst

124
Q

are the teeth affected by traumatic bone cysts vital?

A

yes

125
Q

___ is slowly and chronically progressive, but rarely causes death

A

paget’s disease

126
Q
A

idiopathic osteosclerosis

127
Q

what is the treatment of dental problems associated with cleidocranial dysplasia?

A
  • can be difficult
    • can include full-mouth extractions with dentures or various other prosthodontics, ortho, and oral surgery related options
128
Q

what are the palate characterics of someone with cleidocranial dysplasia?

A
  • high-arched palate
  • increased prevalence of cleft-palate
129
Q

pt also has hypoplastic clavicles

A

cleidocranial dysplasia

130
Q

what are the radiographic features of aneurysmal bone cysts?

A
  • radiolucent lesion with marked cortical expansion and thinning
  • usually unilocular; can be multilocular
  • borders are variable; can be well-defined or diffuse
  • frequently described is a “blow-out” or ballooning distention of the countour of the affected bone
131
Q

there is no association between focal osteoporotic marrow defect and any ___ disorder

A

hematologic