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Skeletal Tumors Flashcards

1
Q

Benign osteogenic tumor of small size and disproportionate pain responsive to NSAIDs

A

Osteoid osteoma

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2
Q

Neoplasm that is central nidus of (usually) unmineralized osteoid rimmed by osteoblasts (sclerotic rim)

A

Osteoid osteoma

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3
Q

Typical age of patient with osteoid osteoma

A

5-25 years

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4
Q

What is the size of Osteoid osteoma?

A

Always less than 2cm

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5
Q

Does Osteoid osteoma have a sclerotic rim?

A

Yes

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6
Q

Is Osteoid osteoma painful?

A

Yes, especially at night

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7
Q

Is Osteoid osteoma pain relieved by aspirin?

A

Yes

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8
Q

Condition where radiograph shows central lucency with sclerotic rim

A

Osteoid osteoma

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9
Q

Osteoid osteoma is located in this part of the bone

A

Cortex

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10
Q

Condition where unmineralized osteoid is neoplastic focus
Reactive sclerotic rim of bone surrounds this

A

Osteoid osteoma

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11
Q

What size is Osteoblastoma?

A

May be greater than 2cm

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12
Q

Most common site of origin for Osteoblastoma

A

Posterior vertebra

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13
Q

Does Osteoblastoma have a sclerotic rim?

A

Often no

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14
Q

Is Osteoblastoma painful?

A

Not very often; lacks nocturnal pain
Nerve impingement may occur and become painful

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15
Q

Is nerve impingement pain from Osteoblastoma relieved by aspirin?

A

No

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16
Q

Condition that is very similar to osteoid osteoma but may be >2cm

A

Osteoblastoma

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17
Q

Which of these can be larger than 2cm:
Osteoblastoma or Osteoid osteoma

A

Osteoblastoma

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18
Q

Malignant tumor that produces osteoid

A

Osteosarcoma

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19
Q

Most common malignancy primary bone tumor

A

Osteosarcoma

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20
Q

When does Osteosarcoma occur?

A

Most common before 20 years
2nd peak in late adulthood

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21
Q

Adult form of this condition often occurs with Pagets, infarct, or radiation

A

Osteosarcoma

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22
Q

What is the most common site of Osteosarcoma?

A

Metaphysis around knee
(distal femur/proximal tibia during rapid growth phase - will have open growth plate)

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23
Q

Two genetic conditions associated with Osteosarcoma

A

Rb gene (retinoblastoma)
Li-Fraumeni syndrome

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24
Q

What causes Li-Fraumeni syndrome?

A

Mutation in p53
leads to sarcomas, adrenal cortical carcinoma

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25
Q

Condition involving a mutation in p53, leading to conditions such as Osteosarcoma

A

Li-Fraumeni syndrome

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26
Q

Is Osteosarcoma painful?

A

Usually yes

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27
Q

Condition involving mixed lytic and blastic lesion
Often with cotton ball/cloudy appearance

A

Osteosarcoma

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28
Q

This describes a periosteal reaction where the periosteum is lifted off the bone
Occurs when a bone lesion grows so quickly that it prevents new born from forming

A

Codmans Triangle

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29
Q

Condition where radiographs show Codmans triangle, sunburst, and hair on end appearance

A

Osteosarcoma

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30
Q

Condition where the lesion grows through cortex, and lifts periosteum and causes periosteal reaction

A

Osteosarcoma

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31
Q

Condition involving malignant cells which produce osteoid

A

Osteosarcoma

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32
Q

Are metastases present in Osteosarcoma?

A

Often yes, check lungs, bones, brain

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33
Q

Condition involving a mixed lytic and blastic lesion where you should assume distant metastases are present (lung, bones, brain)

A

Osteosarcoma

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34
Q

Benign bone tumor formed from lateral extension of growth plate

A

Osteochondroma
(aka Exostosis)

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35
Q

What age is a typical patient with Osteochondroma?

A

Late adolescent/early adult

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36
Q

Where does Osteochondroma occur on the body?

A

Near growth plate of long tubular bones, especially knee

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37
Q

15% of this condition occurs in hereditary exostosis syndrome

A

Osteochondroma

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38
Q

Condition where growth produces bony stalk (cortex contiguous with cortex) with cartilage cap (exostosis)

A

Osteochondroma

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39
Q

Morphology may show cartilage cap (exostosis) in this condition

A

Osteochondroma

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40
Q

Is Osteochondroma painful?

A

May produce pain from mass effect or fracture
But often asymptomatic

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41
Q

What age is a patient with Enchondroma?

A

20’s and up

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42
Q

What part of the bone is Enchondroma?

A

In medullary

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43
Q

Condition involving a juxtacortical (periosteal) chondroma

A

Enchondroma

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44
Q

Condition involving an area of lucency in long bone with chondroid type calcification

A

Enchondroma

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45
Q

Condition that morphologically appears as popcorn, rings and arcs

A

Enchondroma

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46
Q

Is Enchondroma painful?

A

Most asymptomatic

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47
Q

Conditions with non-hereditary mutations of IDH-1 are more likely to form this

A

Chondrosarcoma

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48
Q

Conditions with non-hereditary mutations of this are more likely to form chondrosarcoma

A

IDH-1

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49
Q

Condition caused by a mutation of IDH-1 that involves the formation of multiple enchondromas

A

Ollier’s disease

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50
Q

Ollier’s disease is caused by this

A

Non-hereditary mutations of IDH-1

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51
Q

Maffucci syndrome is caused by this

A

Non-hereditary mutations of IDH-1

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52
Q

Condition caused by a mutation of IDH-1 that involves multiple chondromas and hemangiomas

A

Maffucci syndrome

53
Q

Ollier’s disease and Maffucci syndrome are two forms of multifocal tumors that are rare but more likely to form this

A

Chondrosarcoma

54
Q

Malignant tumor of chondrocytes

A

Chondrosarcoma

55
Q

What age is a typical patient with Chondrosarcoma?

A

Older ages, >50 usually

56
Q

IDH (isocitrate dehydrogenase) gene mutations can cause either of these tumors

A

Chondrosarcoma
Chondromas

57
Q

Mutations in this gene can cause chondrosarcoma

A

IDH (isocitrate dehydrogenase)

58
Q

Is Chondrosarcoma painful?

59
Q

Patients with this condition present with pain and expansile mass (destructive mass with chondroid calcification)

A

Chondrosarcoma

60
Q

Condition with an expansile mass, usually with chondroid calcification
Shows permeative growth - may extend into soft tissues

A

Chondrosarcoma

61
Q

Condition where endosteal scalloping (scallop edge, irregular margin) and Codman triangle are seen morphologically

A

Chondrosarcoma

62
Q

Condition involving malignant cells in chondroid matrix

A

Chondrosarcoma

63
Q

Small blue cell tumor of neuroectodermal origin

A

Ewing Sarcoma

64
Q

Unidfferentiated tumor that is the second most common bone sarcoma in children

A

Ewing Sarcoma

65
Q

What age is the typical patient with Ewing Sarcoma?

A

Less than 20 years old

66
Q

Ethnicity most commonly affected by Ewing Sarcoma

A

Caucasians

67
Q

Is Ewing Sarcoma painful?

A

Yes - painful mass with swelling and warmth

68
Q

Condition involving painful mass with swelling and warmth, may have constitutional symptoms (fever, leukocytosis)

A

Ewing Sarcoma

69
Q

Elevated levels of this cell type are seen in Ewing Sarcoma

A

Leukocytes

70
Q

Destructive intramedullary tumor with permeative moth-eaten margins

A

Ewing Sarcoma

71
Q

In this condition, reactive periosteal bone forms “onion skin” appearance

A

Ewing Sarcoma

72
Q

In Ewing Sarcoma, reactive periosteal bone has this appearance

A

Onion skin

73
Q

In Chondrosarcoma, periosteal reaction has this appearance

A

Endosteal scalloping
Codman triangle

74
Q

Tumor that extends into soft tissues, with reactive periosteal bone that forms “onion skin” appearance

A

Ewing Sarcoma

75
Q

Translocation in Ewing Sarcoma

76
Q

t(11,22) causes this condition

A

Ewing Sarcoma

77
Q

In Ewing Sarcoma, t(11,22) forms this

A

EWSR1/FLI1 fusion protein
Results in unregulated growth on undifferentiated mesenchymal cell

78
Q

Condition where a translocation forms a fusion protein that results in unregulated growth on unknown undifferentiated mesenchymal cell

A

Ewing Sarcoma

79
Q

This is a primitive neuroectodermal tumor (PNET)

A

Ewing Sarcoma

80
Q

Condition where sheets of small round blue cells are seen morphologically

A

Ewing Sarcoma

81
Q

CD99 is a marker for this condition

A

Ewing Sarcoma

82
Q

IHC marker for Ewing Sarcoma

83
Q

Condition that begins in marrow space, erodes through cortex into soft tissues

A

Ewing Sarcoma

84
Q

Condition that sometimes forms Homer-Wright rosettes with central fibrillary core

A

Ewing Sarcoma

85
Q

Benign tumor of osteoblasts with numerous non-neoplastic osteoclasts

A

Giant cell tumor

86
Q

What is the typical age of a patient with Giant cell tumor?

87
Q

Condition that occurs with closed growth plates, and expansile growth without penetrating joint space

A

Giant cell tumor

88
Q

Does Giant cell tumor have surrounding sclerosis?

89
Q

Condition with radiology showing thin rim of cortex and usually no surrounded sclerosis

A

Giant cell tumor

90
Q

In Giant cell tumor, neoplastic osteoblasts produce this

A

RANK-L
results in osteoclast activation and proliferation (many cells with numerous nuclei)

91
Q

Condition where neoplastic osteoblasts produce RANK-L, resulting in osteoclast activation and proliferation

A

Giant cell tumor

92
Q

Abnormal bone growth resulting in abnormal osteoid in fibrous matrix

A

Fibrous dysplasia

93
Q

Condition where clonal expansion occurs due to GNAS mutations

A

Fibrous dysplasia

94
Q

Fibrous dysplasia involves clonal expansion due to mutations in this

95
Q

In Fibrous dysplasia, clonal expansion due to GNAS mutations occur during this process

A

Embryogenesis

96
Q

In this condition, osteoblasts do not differentiate, leading to intramedullary lytic lesions
Proliferate but unable to produce mature lamellar bone

A

Fibrous dysplasia

97
Q

What age does Fibrous dysplasia occur?

A

Early adolescence

98
Q

Is Fibrous dysplasia painful?

A

No, most are symptomatic and discovered incidentally

99
Q

Condition with monostotic and polyostotic forms that may be disfiguring or cause growth imbalances

A

Fibrous dysplasia

100
Q

Polyostotic forms of Fibrous dysplasia are often part of this condition

A

McCune Albright Syndrome

101
Q

Polyostotic forms of this condition are often part of McCune Albright Syndrome

A

Fibrous dysplasia

102
Q

Condition involving endocrine dysfunction, female precocious puberty, and areas of cutaneous hyperpigmentation (cafe au lait)

A

McCune Albright Syndrome

103
Q

Condition with expansile remodeling of marrow cavity, causing bone deformity

A

Fibrous dysplasia

104
Q

Condition with a well-circumscribed intramedullary lesion and ground glass opacifications (may be lucent or opaque)

A

Fibrous dysplasia

105
Q

Does Fibrous dysplasia have a sclerotic rim?

106
Q

Condition with a fibrous stroma, variably mineralized osteoid with curvilinear shapes, and no osteoblastic rimming

A

Fibrous dysplasia

107
Q

In Fibrous dysplasia, there is variably mineralized osteoid with this shape

A

Curvilinear

108
Q

Condition with variably mineralized osteoid with curvilinear shapes

A

Fibrous dysplasia

109
Q

Condition involving clonal proliferation of Langerhans cells

A

Langerhans cell histiocytosis

110
Q

Condition that has the following three forms with identical histology:
Eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease

A

Langerhans cell histiocytosis

111
Q

CD1a, S100, and Langerhin are IHC markers of this condition

A

Langerhans cell histiocytosis

112
Q

Condition with mononuclear cells with grooved nuclei, eosinophilic infiltrate

A

Langerhans cell histiocytosis

113
Q

Non-cutaneous form of Langerhans cell histiocytosis that is a localized single lesion, mostly lytic bone lesion, with spontaneous regression

A

Eosinophilic granuloma

114
Q

Is Eosinophilic granuloma a localized or disseminated form of Langerhans cell histiocytosis?

115
Q

What age is the typical patient with Eosinophilic granuloma (localized form of Langerhans cell histiocytosis)?

A

Birth to 6 years

116
Q

2 most common sites of Eosinophilic granuloma (localized form of Langerhans cell histiocytosis)

A

Skull and vertebrae

117
Q

Two main forms of disseminated Langerhans cell histiocytosis

A

Hand-Schuller-Christian disease
Letterer-Siwe disease

118
Q

Is Hand-Schuller-Christian disease a localized or disseminated form of Langerhans cell histiocytosis?

A

Disseminated

119
Q

Is Letterer-Siwe disease a localized or disseminated form of Langerhans cell histiocytosis?

A

Disseminated

120
Q

Disseminated form of Langerhans cell histiocytosis involving multifocal bone lesions and can affect cranial nerve foramina

A

Hand-Schuller-Christian disease

121
Q

Disseminated form of Langerhans cell histiocytosis with the classic triad of Multifocal bone lesions, retro-orbital and pituitary effects

A

Hand-Schuller-Christian disease

122
Q

What is the classic triad seen in Hand-Schuller-Christian disease?

A

Multifocal bone lesions
Retro-orbital (exophthalmos)
Pituitary region (diabetes insipidus)

123
Q

Disseminated form of Langerhans cell histiocytosis that is aggressive with early death
Dominated by visceral involvement

A

Letterer-Siwe disease

124
Q

Disseminated forms of Langerhans cell histiocytosis present at this age

A

Birth to 6 years

125
Q

Condition that begins as pink papules and pustules, looks like seborrheic dermatitis or eczema, with eventual multiorgan and bone involvement (hepatosplenomegaly, lymphadenopathy often)

A

Disseminated forms of Langerhans cell histiocytosis

126
Q

Levels of Ca in metastatic bone tumors

126
Q

4 most common locations where metastatic tumors from bone neoplasms occur

A

Prostate, breast, kidney, lung

127
Q

Levels of alk phos in metastatic bone tumors

128
Q

This cancer is the most common blastic metastasis

A

Prostate cancer

Skin & MSK (20 decks)

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