Benign Neoplasia of Bone Flashcards

1
Q

Benign bone forming tumors produce…

A

unmineralized osteoid or mineralized woven bone

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2
Q

An osteoma enostoma is ___-growing and comprised of ___

A

slow-growing and comprised of dense cortical bone

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3
Q

What are the clinical subtypes of osteoma/enostoma by location?

A

Osteoma:
1. Calvarial and mandibular
2. Sinonasal and orbital bone
Enostoma (hamartoma):
1. Medullary bone “islands”
2. Long bone surfaces

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4
Q

What is Gardner syndrome?
How does it relate to bone tumors?

A

Inherited condition causing multiple polyps in colon and possibly multiple osteomas

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5
Q

Are osteomas usually solitary or multiple?

A

Solitary

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6
Q

In what case would an osteoma be removed?

A

Surgical removal if in sensitive location

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7
Q

Enostomas can mimic ___

A

blastic metastasis

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8
Q

What is the clinical significance of osteoma/enostoma?

A

Usually minimal

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9
Q

What is the distribution of osteoid osteoma among sexes?

A

Male > Female (3:1)

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10
Q

What age group is most affected by osteoid osteoma?

A

5-35 years of age

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11
Q

Where in the bone does osteoid osteoma typically arise?

A

In cortex, but may arise in medullary cavity

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12
Q

What is the size of osteoid osteoma?

A

< 1 cm in diameter

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13
Q

How does osteoid osteoma present radiographically?

A

Radiolucent lesion surrounded by sclerotic bone

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14
Q

What does nidus refer to?

A

Osteoid osteoma

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15
Q

What does nidus contain?

A

Irregular trabeculae of woven bone within a vascular fibrous stroma containing osteoblasts and osteoclasts

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16
Q

How do trabeculae in the center of a nidus compare to those in the peripheral?

A

Center trabeculae are more mature and may be ossified

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17
Q

With osteoid osteoma/nidus, there are ___ levels of Prostaglandin E2

A

high levels

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18
Q

PGE2 is produced by ___ increasingly with osteoid osteoma

A

proliferating osteoblasts

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19
Q

osteoid osteoma

PGE2 is up to ___ in nidus than outside

A

12x greater in nidus than outside

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20
Q

What are some reasons pain is worse at night with osteoid osteoma?

A
  • PGE2 produced by proliferating osteoblasts increases
  • PGE2 is up to 12x greater in nidus than outside
  • Vasodilation of vascular components in the stroma
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21
Q

Osteoid osteoma pain is relieved by ___
Why?

A

relieved by aspirin/NSAIDs because of vasoconstriction mechanism

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22
Q

Osteoblastoma is similar histologically to ___

A

osteoid osteoma

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23
Q

Which bones do osteoblastoma generally occur?

A

Axial skeleton: laminae and pedicles

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24
Q

What is the size of an osteoblastoma?

A

> 2 cm diameter with progressive enlargement

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25
Q

How does an osteoblastoma appear radiographically?

A

Radiolucent lesion without sclerotic reaction

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26
Q

Is osteoblastoma painful?

A

Painful, but less so than an osteoid osteoma

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27
Q

Is osteoblastoma pain responsive to aspirin?

A

No

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28
Q

Osteoid osteoma and osteoblastoma are often treated similarly:

A
  • Surgical excision via curette
  • Radiofrequency ablation
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29
Q

What may be required when surgically excising osteoblastoma via curette?

A

Wide bloc resection

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30
Q

What is radiofrequency ablation?

A

An electric probe is inserted into the tumor

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31
Q

What is the name for a benign cartilage forming tumor?

A

Osteochondroma

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32
Q

An osteochondroma is also called an ___

A

exostosis

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33
Q

What is the most common benign skeletal tumor?

A

Osteochondroma

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34
Q

How is osteochondroma distributed among sexes?

A

Male > Female (3:1)

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35
Q

When is osteochondroma usually diagnoses in life?

A

Skeletally immature individuals

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36
Q

What are the two types of osteochondromas?

A
  • Pedunculated
  • Sessile
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37
Q

__% of osteochondromas are solitary

A

85%

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38
Q

__% of osteochondroma are hereditary multiple exostosis

A

15%

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39
Q

What is osteochondromatosis?

A

Hereditary multiple exostosis: autosomal dominant hereditary disease

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40
Q

Up to __% of those with hereditary multiple exostosis may undergo malignant degeneration

A

25%

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41
Q

With osteochondroma, there is loss of function mutations in ___ which may disrupt local cartilage growth and chondrocyte differentiation

A

mutations in tumor suppressor genes EXT1 or EXT2

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42
Q

If a patient is found to have loss of function mutations in tumor suppressor genes EXT1 or EXT2, what may be the result?

A

Osteochondroma

43
Q

How does pedunculated osteochondroma appear?

A

Bony stalk capped by cartilage

44
Q

How does sessile osteochondroma appear?

A

Don’t often have a cap, cartilage is intermingled

45
Q

Osteochondroma only affects ___ bones

A

endochondral bones

46
Q

Osteochondroma arises from ___

A

metaphysis near growth plate

47
Q

Osteochondroma usually stops growing with ___

A

epiphyseal fusion

48
Q

What are common sites for osteochondroma?

A

Knee is common site
Also, pelvis, scapula, ribs

49
Q

The cartilaginous cap of osteosarcoma is covered by ___

A

perichondrium

50
Q

The cartilaginous cap of osteosarcoma merges into ___ below where chondrocytes arrange according to the growth plate

A

spongiosa

51
Q

How many osteochondromas are considered solitary?

A

< 6

52
Q

Are osteochondromas usually solitary or multiple?

A

Solitary

53
Q

Is osteochondroma generally painful or painless?

A

Generally form painless, potentially palpable lumps

54
Q

In which type of osteochondroma does pathological fracture occur?

A

Pedunculated

55
Q

What are some reasons osteochondroma might be painful?

A
  • Pathological fracture (pedunculated only)
  • Tendinosis/bursitis
  • Neurovascular bundle impingement
  • Malignant transformation (<1% for solitary)
56
Q

How are bones shaped when hereditary multiple exostosis is present?

A

Affected bones may be bowed and shortened
Multiple bones with osteochondromas (6-100s)

57
Q

Benign cartilaginous tumors form ___ cartilage

A

hyaline cartilage

58
Q

Benign cartilaginous tumors mostly form when in life?

A

Prior to 20 years of age

59
Q

Benign cartilaginous tumors form in ___ bones

A

endochondral

60
Q

What are the two subtypes of benign cartilaginous tumors?

A
  1. Enchondroma
  2. Juxtacortical chondroma
61
Q

benign cartilaginous tumors

Enchondroma arises in the ___

A

medullary cavity

62
Q

benign cartilaginous tumors

Juxtacortical chondroma arises on the ___

A

surface of bone

63
Q

What is the etiology of benign cartilaginous tumors?

A

Mutations in genes related to chondrocyte development

64
Q

If a benign cartilaginous tumor arises in the medullary cavity, what type is it?

A

Enchondroma

65
Q

If a benign cartilaginous tumor arises on the surface of bone, what type is it?

A

Juxtacortical chondroma

66
Q

Is enchondroma solitary or multiple?

A

Solitary lesion

67
Q

___% of enchondromas undergo malignant transformation

A

<1%

68
Q

What is Ollier disease?

A

Multiple enchondromas (benign cartilaginous tumors), possible hereditary component

69
Q

___% of enchondromas related to Ollier disease undergo malignant transformation

A

25%

70
Q

What is Mafucci syndrome?

A

Rare
Multiple enchondromas (benign cartilaginous tumors) and soft tissue hemangiomas

71
Q

___% of enchondromas related to Mafucci syndrome undergo malignant transformation

A

50%

72
Q

How common is periosteal/juxtacortical chondroma?

A

Rare

73
Q

What is the most common benign tumor of hands and feet?

A

Benign cartilaginous tumor (enchomdroma)

74
Q

What are some symptoms of a benign cartilaginous tumor?

A
  • Usually asymptomatic, but occasionally painful
  • Pathological fractures
75
Q

If a patient has the following radiographic findings, what is suspected?

  • Geographic lytic lesion with or without a sclerotic border
  • May or may not have matrix calcification
  • Intact and thinned cortex with endosteal scalloping
  • Deformities
A

Benign cartilaginous tumor

76
Q

What are the radiographic findings for a benign cartilaginous tumor?

A
  • Geographic lytic lesion with or without a sclerotic border
  • 50% have matrix calcification
  • Intact and thinned cortex, endosteal scalloping
  • Deformities
77
Q

What are the treatments for asymptomatic benign cartilaginous tumors?

A

None, maybe monitor

78
Q

What is the treatment for disruptive osteochondromas or juxtacortical chondromas?

A

Surgical removal

79
Q

What is the treatment for problematic enchondromas?

A

Curettage

80
Q

How are complications of benign cartilage tumors treated?

A
  • Surgical removal (disruptive osteochondromas or juxtacortical chondromas)
  • Curettage (problematic enchondromas)
  • Treatment for pathological fracture
  • Montor for and treat malignant degeneration
81
Q

Chondroblastoma occurs most commonly in ___ of ___ bones

A

epiphyses and apophyses of endochondral bones

82
Q

What is the distribution of chondroblastoma among sexes?

A

Males > Females (2:1)

83
Q

What age group is typically affected by chondroblastoma?

A

Ages 5-25

84
Q

What are the radiographic features of chondroblastoma?

A
  • Slow growing
  • Geographic lytic
  • Well-defined borders
85
Q

What are symptoms of chondroblastoma?

A

Presents with joint pain

86
Q

Giant cell tumor is also known as ___

A

Osteoclastoma (benign tumor of unknown origin)

87
Q

Giant cell tumors typically occur in what age group?

A

Ages 20-40

88
Q

How are giant cell tumors distributed among sexes?

A

Female > Male (3:2)

89
Q

___% of giant cell tumors are benign
___% are malignant

A

> 80% are benign
<20% are malignant

90
Q

Giant cell tumors consist of ___ giant cells with proliferating ___ cells

A

osteoclastic multinucleated giant cells with proliferating mononuclear cells

91
Q

What gives giant cell tumors the gross appearance of a sponge filled with blood?

A

Multiple hemorrhagic areas

92
Q

Neoplastic cells found in a giant cell tumor express high levels of ___

A

RANKL

93
Q

giant cell tumor

What is the function of RANKL?

A
  • Promotes osteoclast precursor proliferation
  • Affects expression of RANK which leads to differentiation of mature osteoclasts
94
Q

How is the bone matrix affected by giant cell tumors?

A

Localized, highly destructive resorption of bone matrix

95
Q

Are giant cell tumors solitary or multicentric/multilocular?

A

Usually solitary lesions, but may be multicentric or multilocular

96
Q

Giant cell tumors arise in the ___ and extend into ___
What is this called?

A

arise in the metaphysis and extend into epiphysis
This is called subarticular extension

97
Q

Where to 50% of giant cell tumors occur?

A

50% of cases occur around the knee (distal femur and proximal tibia)

98
Q

Besides the knee, where might you find a giant cell tumor?

A

Distal radius, humerus, and fibula

99
Q

How often do giant cell tumors become malignant and metastasize to the lungs?
How often are they fatal?

A

Rarely do the metastasize to the lungs
Rarely are they fatal
(Often spontaneously regress)

100
Q

What are the clinical manifestations of giant cell tumors?

A
  • Non-specific regional pain
  • Pathologic fractures
  • Joint pain and decreased range of motion
  • Possible functional disability
101
Q

What are radiographic characteristics of giant cell tumors?

A
  • Geographic lytic lesion
  • If expansile, bone surrounding lesion is often very thin
  • Frequently multiloculated appearance
102
Q

Why do giant cell tumors frequently have a multiloculated appearance?

A

Endosteal bone resorption

103
Q

What are treatments for giant cell tumors?

A
  • Curettage (40-60% recur locally)
  • Denosumab (RANKL inhibitor)