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NYCC MSP > Bone Cysts & Tumor-like Bone Pathologies > Flashcards

Bone Cysts & Tumor-like Bone Pathologies Flashcards

1
Q

What are 2 alternative names for a simple bone cyst?

A
  • Unicameral bone cyst
  • Solitary bone cyst
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2
Q

What causes a simple bone cyst?

A

disturbance of bone growth following trauma

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3
Q

What age group is primarily affected by simple bone cysts?

A

<20 years

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4
Q

What is the preferential location of simple bone cysts?

A

proximal metaphysis
- humerus
- femur
- tibia

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5
Q

Describe the pathogenesis of a simple bone cyst

A
  • trauma –> hematoma forms & resolves
  • formation of fibrous lining
  • fibrous tissue pulls serous fluid into lesion
  • fluid pressure leads to bone resorption
  • some reactive bone formation (solid)
  • produces lytic lesion with thin, well-defined margin and sclerotic border
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6
Q

A simple bone cyst occurs over the course of ____

A

months

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7
Q

What pattern of destruction is seen in a simple bone cyst?

A

geographic lytic

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8
Q

A simple bone cyst has a ____ zone of transition

A

short/narrow

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9
Q

How does the cortical bone appear in a simple bone cyst?

A

thinning of cortex due to resorption of bone, may lead to pathologic fractures

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10
Q

Why is a simple bone cyst referred to as a “migrating tumor”?

A

Lesion tends to develop in metaphysis, and can go into diaphysis with age
(NOT a neoplasia)

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11
Q

Is a simple bone cyst a true cyst? Why or why not?

A
  • not a true cyst because there is no distinct cell lining/membrane (which would completely separate it from surrounding tissues)
  • has a fibrous lining that allows serous fluid in
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12
Q

What type of cells are found in a simple bone cyst?

A
  • osteoclastic giant cells
  • macrophages
  • chronic inflammatory cells
  • osteoclasts in advancing area of cyst
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13
Q

What type of periosteal reaction may be present in a simple bone cyst?

A

solid (non-aggressive)

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14
Q

How would a patient present clinically with a simple bone cyst?

A
  • asymptomatic
  • incidental finding, usually due to patho. Fx
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15
Q

What are the radiographic characteristics of a simple bone cyst?

A
  • unilocular radiolucent lesion with sclerotic border
  • “fallen fragment” sign
  • usually centrally located
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16
Q

What is the treatment for a simple bone cyst?

A
  • intralesional corticosteroids
  • curettage (scoop out) and bone grafting (pack with bone chips)
  • tends to recur
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17
Q

How is a simple bone cyst prevented from recurring?

A

inject with phenol to kill fibrous lining

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18
Q

What is an aneurysmal bone cyst?

A

multiloculated lytic lesion with septa composed of fibrous tissue and reactive bone

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19
Q

What age group is primarily affected by aneurysmal bone cysts?

A

<20 years

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20
Q

What is the preferential location of aneurysmal bone cysts?

A

80% occur in metaphyseal region of long bones & in the spine

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21
Q

What parts of the spine do aneurysmal bone cysts prefer?

A

posterior elements:
- articular processes
- pedicles
- lamina
- SPs

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22
Q

What is a primary aneurysmal bone cyst?

A

(genetic) USP6 overexpression, which leads to ^proteases and transcription factors that upregulate cystic resorption of bone

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23
Q

What is a secondary aneurysmal bone cyst?

A

develop with other primary tumors
eg. giant cell tumor, osteosarcoma

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24
Q

What does multiloculated mean?

A

multiple chambers

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25
Q

Aneurysmal bone cysts are filled with ____

A

blood

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26
Q

Simple bone cysts are filled with ____

A

serous fluid

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27
Q

What does the appearance of an aneurysmal bone cyst resemble?

A

“soap bubble” appearance or sponge filled with blood and blood clots

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28
Q

Aneurysmal bone cysts grow (rapidly/slowly)

A

rapidly

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29
Q

Simple bone cysts grow (rapidly/slowly)

A

slowly

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30
Q

Are aneurysmal bone cysts typically central or eccentric?

A

can be either

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31
Q

Are simple bone cysts typically central or eccentric?

A

central

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32
Q

What are the radiographic characteristics of aneurismal bone cysts?

A
  • expansile
  • intact periosteum & cortex, with thinning of cortex (balloon-like)
  • multilocular radiolucent lesion
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33
Q

What pattern of destruction occurs in an aneurysmal bone cyst?

A

geographic lytic

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34
Q

An aneurysmal bone cyst has a ____ zone of transition

A

short/narrow

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35
Q

What does expansile mean?

A

enlarged bone

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36
Q

How will a patient with an aneurysmal bone cyst present clinically?

A
  • pain
  • swelling
  • cyst may blow out and cause local hemorrhage
  • patho. Fx (rare)
  • possible neurological deficits
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37
Q

Why would a patient with an aneurysmal bone cyst present with neurological deficits?

A

central canal stenosis when in posterior spinal elements
(this is odd in young ppl!!!)

38
Q

What is the treatment for an aneurysmal bone cyst?

A
  • curettage and bone grafting
  • variable recurrence rate
39
Q

Which is more likely to be removed:
simple bone cyst // aneurysmal bone cyst

A

aneurysmal bone cyst due to severe pain

40
Q

What are the 3 categories of fibrous cortical defect?

A
  • fibrous cortical defect
  • non-ossifying fibroma
  • fibrous xanthoma
41
Q

How are the categories of fibrous cortical defect differentiated?

A

age
(all appear the same histologically, just written about at different times & ages)

42
Q

What is the term for a fibrous cortical defect that occurs in patients younger than 8 years old?

A

fibrous cortical defect

43
Q

What is the term for a fibrous cortical defect that occurs in patients ages 8-19 years?

A

non-ossifying fibroma

44
Q

What is the term for a fibrous cortical defect that occurs in patients 20 years and older?

A

fibrous xanthoma

45
Q

What is the preferential location of fibrous cortical defect?

A
  • metaphyseal region
  • tibia or femur
46
Q

How would a patient with fibrous cortical defect present clinically?

A
  • usually asymptomatic
  • pathologic fracture (thin cortex) -> pain
  • may be associated with neurofibromatosis type 1 & Jaffe-Campanacci syndrome
47
Q

At what point is a patient with fibrous cortical defect at increased risk of pathologic fracture?

A

When tumor occupies at least 50% of diameter of bone

48
Q

An 18-year-old patient presenting with multiple non-ossifying fibromas (NOFs) and cafe au lait spots may have what associated condition?

A

Jaffe-Campanacci syndrome
(associated w/ fibrous cortical defect)

49
Q

If a patient has multiple fibrous cortical defects, non-ossifying fibromas, or fibrous xanthomas, what 2 conditions might you be concerned about?

A
  • neurofibromatosis type 1
  • Jaffe-Campanacci syndrome
50
Q

What are the radiographic characteristics of fibrous cortical defect?

A
  • cortical location
  • eccentric
  • geographic lytic
  • scalloped margin
  • radiolucent lesion with sclerotic border
51
Q

What periosteal reaction is observed in fibrous cortical defect?

A

none

52
Q

Fibrous cortical defect has a ____ zone of transition

A

short/narrow

53
Q

What matrix calcification is observed in fibrous cortical defect?

A

none (purely lytic)

54
Q

Is there any soft tissue mass involved with fibrous cortical defect? How could you tell?

A

no
- no mass extending beyond bone
- no (aggressive) periosteal reaction
- no displacement of fascial planes

55
Q

What is the treatment for fibrous cortical defect?

A
  • spontaneous resolution (no recurrence) -> replaced by normal bone
  • curettage & bone grafting (for expanded or symptomatic lesions)
  • fracture repair
56
Q

What age group is primarily affected by fibrous dysplasia?

A

children/young adults
(usually <10 yrs)

57
Q

What is the etiology of fibrous dysplasia?

A
  • mutation of GNAS1 gene
  • abnormal cellular development
58
Q

What are the 3 forms of fibrous dysplasia? Which is most common?

A
  • monostotic (MC)
  • polyostotic
  • McCune-Albright syndrome
59
Q

The term “fibrous dysplasia” is referring to which form?

A

monostotic

60
Q

What is fibrous dysplasia?

A

failure to form mature lamellar bone in the lesion, resulting in fibrous replacement of bony stroma

61
Q

How does a fibrous dysplasia lesion begin?

A

focal, slowly expanding lesion

62
Q

What does a lesion of fibrous dysplasia consist of?

A
  • fibroblasts
  • collagen
  • irregular trabeculae
  • cystic degeneration + hemorrhage
63
Q

How common is malignant transformation of fibrous dysplasia, and what would it transform to?

A

<1% of cases
- osteosarcoma
- chondrosarcoma
- fibrosarcoma

64
Q

What are the radiographic characteristics of fibrous dysplasia?

A
  • geographic lytic lesions (well-defined borders, thin cortex)
  • expansile
  • bony deformation (Sheppard’s crook)
  • ground glass matrix (uniform density)
65
Q

What is the most common benign tumor of the rib?

A

fibrous dysplasia

66
Q

Fibrous dysplasia has a ____ zone of transition

A

short/narrow

67
Q

What age group is primarily affected by monostotic fibrous dysplasia?

A

10-30 years old

68
Q

How would a patient with monostotic fibrous dysplasia present clinically?

A
  • asymptomatic
  • patho Fx (incidental finding)
  • bone enlargement or deformation
  • possible altered gait biomechanics
69
Q

What are the most common locations of monostotic fibrous dysplasia?

A
  • ribs
  • femur
  • tibia
  • facial bones
70
Q

What populations are primarily affected by polyostotic fibrous dysplasia?

A
  • usually observed in childhood
  • may be quiescent in puberty
  • lesion growth may increase with pregnancy
71
Q

How would a patient with polyostotic fibrous dysplasia present clinically?

A
  • > 50% skeleton effected
  • bone deformities (size & shape)
  • limb length discrepancies
  • spine alignment issues
  • patho Fx
  • Mazabraud syndrome
72
Q

What is a Sheppard’s crook deformity?

A

excessive curvature of proximal femur seen in fibrous dysplasia due to bone softening
(bowing deformity of hips)

73
Q

What is Mazabraud syndrome?

A

Soft tissue myxomas (benign mesenchymal tumors)

74
Q

What is the preferential location of polyostotic fibrous dysplasia?

A
  • any bone
  • can be unilateral or monomelic
75
Q

What does monomelic mean?

A

Affecting 1 limb

76
Q

What matrix calcification is seen in polyostotic fibrous dysplasia?

A

ground glass

77
Q

What form of fibrous dysplasia is exclusive to females?

A

McCune-Albright syndrome

78
Q

What is McCune-Albright syndrome?

A

Polyostotic fibrous dysplasia + endocrine abnormality

79
Q

What endocrine dysfunctions may present with McCune-Albright syndrome?

A
  • acromegaly
  • Cushing syndrome
  • hyperthyroidism
  • vit D resistant rickets
80
Q

How does precocious puberty present in females?

A
  • Premature onset of menstrual cycle (~age 8)
  • Premature closure of growth plates —> shorter stature
81
Q

How would a patient with McCune-Albright syndrome present clinically?

A
  • asymptomatic
  • patho Fx (incidental finding)
  • bone enlargement or deformation
  • cafe au lait spots on skin with “coast of Maine” borders
82
Q

What is the treatment for Fibrous Dysplasia?

A
  • depends on monostotic vs polyostotic
  • curettage
  • fracture repair
  • prevent deformities (bracing)
  • manage endocrine hormones
83
Q

A 5 year old has pain after a fall. In the proximal humerus, there is a geographic lytic lesion with a pathologic fracture, and a cortical fragment inside the lesion. What is your diagnosis?

A

Simple bone cyst
- <20yrs
- incidental finding (patho Fx)
- prefers proximal metaphysis (humerus)
- geographic lytic
- fallen fragment sign

84
Q

An 18 year old patient was x-rayed because they twisted their ankle. There is a geographic lytic lesion in the distal tibia. It has a narrow zone of transition, no periosteal reaction, no matrix calcification, and no soft tissue mass. The lesion occupies 10% of the diameter of the tibia. If left alone, what will the likely outcome be?

A

Spontaneous resolution
(Non-ossifying fibroma)

85
Q

What lesion MUST be eccentric and cortically based?

A

fibrous cortical defect/non-ossifying fibroma/fibrous xanthoma

86
Q

Precocious puberty is associated with ____

A

Fibrous dysplasia (McCune-Albright Syndrome)

87
Q

Which lesion does NOT have a geographic lytic appearance on radiograph?
A) aneurysmal bone cyst
B) paget disease
C) enchondroma
D) non-ossifying fibroma

A

Paget disease

88
Q

What age group is primarily affected by fibrous cortical defect?

A

<8 years

89
Q

What age group is primarily affected by non-ossifying fibroma?

A

8-19 years

90
Q

What age group is primarily affected by fibrous xanthoma?

A

20+ years

NYCC MSP (28 decks)

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