Skeletal Tumors Flashcards
1
Q
Benign osteogenic tumor of small size and disproportionate pain responsive to NSAIDs
A
Osteoid osteoma
2
Q
Neoplasm that is central nidus of (usually) unmineralized osteoid rimmed by osteoblasts (sclerotic rim)
A
Osteoid osteoma
3
Q
Typical age of patient with osteoid osteoma
A
5-25 years
4
Q
What is the size of Osteoid osteoma?
A
Always less than 2cm
5
Q
Does Osteoid osteoma have a sclerotic rim?
A
Yes
6
Q
Is Osteoid osteoma painful?
A
Yes, especially at night
7
Q
Is Osteoid osteoma pain relieved by aspirin?
A
Yes
8
Q
Condition where radiograph shows central lucency with sclerotic rim
A
Osteoid osteoma
9
Q
Osteoid osteoma is located in this part of the bone
A
Cortex
10
Q
Condition where unmineralized osteoid is neoplastic focus
Reactive sclerotic rim of bone surrounds this
A
Osteoid osteoma
11
Q
What size is Osteoblastoma?
A
May be greater than 2cm
12
Q
Most common site of origin for Osteoblastoma
A
Posterior vertebra
13
Q
Does Osteoblastoma have a sclerotic rim?
A
Often no
14
Q
Is Osteoblastoma painful?
A
Not very often; lacks nocturnal pain
Nerve impingement may occur and become painful
15
Q
Is nerve impingement pain from Osteoblastoma relieved by aspirin?
A
No
16
Q
Condition that is very similar to osteoid osteoma but may be >2cm
A
Osteoblastoma
17
Q
Which of these can be larger than 2cm:
Osteoblastoma or Osteoid osteoma
A
Osteoblastoma
18
Q
Malignant tumor that produces osteoid
A
Osteosarcoma
19
Q
Most common malignancy primary bone tumor
A
Osteosarcoma
20
Q
When does Osteosarcoma occur?
A
Most common before 20 years
2nd peak in late adulthood
21
Q
Adult form of this condition often occurs with Pagets, infarct, or radiation
A
Osteosarcoma
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)
23
Q
Two genetic conditions associated with Osteosarcoma
A
Rb gene (retinoblastoma)
Li-Fraumeni syndrome
24
Q
What causes Li-Fraumeni syndrome?
A
Mutation in p53
leads to sarcomas, adrenal cortical carcinoma
25
Condition involving a mutation in p53, leading to conditions such as Osteosarcoma
Li-Fraumeni syndrome
26
Is Osteosarcoma painful?
Usually yes
27
Condition involving mixed lytic and blastic lesion
Often with cotton ball/cloudy appearance
Osteosarcoma
28
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
Codmans Triangle
29
Condition where radiographs show Codmans triangle, sunburst, and hair on end appearance
Osteosarcoma
30
Condition where the lesion grows through cortex, and lifts periosteum and causes periosteal reaction
Osteosarcoma
31
Condition involving malignant cells which produce osteoid
Osteosarcoma
32
Are metastases present in Osteosarcoma?
Often yes, check lungs, bones, brain
33
Condition involving a mixed lytic and blastic lesion where you should assume distant metastases are present (lung, bones, brain)
Osteosarcoma
34
Benign bone tumor formed from lateral extension of growth plate
Osteochondroma
(aka Exostosis)
35
What age is a typical patient with Osteochondroma?
Late adolescent/early adult
36
Where does Osteochondroma occur on the body?
Near growth plate of long tubular bones, especially knee
37
15% of this condition occurs in hereditary exostosis syndrome
Osteochondroma
38
Condition where growth produces bony stalk (cortex contiguous with cortex) with cartilage cap (exostosis)
Osteochondroma
39
Morphology may show cartilage cap (exostosis) in this condition
Osteochondroma
40
Is Osteochondroma painful?
May produce pain from mass effect or fracture
But often asymptomatic
41
What age is a patient with Enchondroma?
20's and up
42
What part of the bone is Enchondroma?
In medullary
43
Condition involving a juxtacortical (periosteal) chondroma
Enchondroma
44
Condition involving an area of lucency in long bone with chondroid type calcification
Enchondroma
45
Condition that morphologically appears as popcorn, rings and arcs
Enchondroma
46
Is Enchondroma painful?
Most asymptomatic
47
Conditions with non-hereditary mutations of IDH-1 are more likely to form this
Chondrosarcoma
48
Conditions with non-hereditary mutations of this are more likely to form chondrosarcoma
IDH-1
49
Condition caused by a mutation of IDH-1 that involves the formation of multiple enchondromas
Ollier's disease
50
Ollier's disease is caused by this
Non-hereditary mutations of IDH-1
51
Maffucci syndrome is caused by this
Non-hereditary mutations of IDH-1
52
Condition caused by a mutation of IDH-1 that involves multiple chondromas and hemangiomas
Maffucci syndrome
53
Ollier's disease and Maffucci syndrome are two forms of multifocal tumors that are rare but more likely to form this
Chondrosarcoma
54
Malignant tumor of chondrocytes
Chondrosarcoma
55
What age is a typical patient with Chondrosarcoma?
Older ages, >50 usually
56
IDH (isocitrate dehydrogenase) gene mutations can cause either of these tumors
Chondrosarcoma
Chondromas
57
Mutations in this gene can cause chondrosarcoma
IDH (isocitrate dehydrogenase)
58
Is Chondrosarcoma painful?
Yes
59
Patients with this condition present with pain and expansile mass (destructive mass with chondroid calcification)
Chondrosarcoma
60
Condition with an expansile mass, usually with chondroid calcification
Shows permeative growth - may extend into soft tissues
Chondrosarcoma
61
Condition where endosteal scalloping (scallop edge, irregular margin) and Codman triangle are seen morphologically
Chondrosarcoma
62
Condition involving malignant cells in chondroid matrix
Chondrosarcoma
63
Small blue cell tumor of neuroectodermal origin
Ewing Sarcoma
64
Unidfferentiated tumor that is the second most common bone sarcoma in children
Ewing Sarcoma
65
What age is the typical patient with Ewing Sarcoma?
Less than 20 years old
66
Ethnicity most commonly affected by Ewing Sarcoma
Caucasians
67
Is Ewing Sarcoma painful?
Yes - painful mass with swelling and warmth
68
Condition involving painful mass with swelling and warmth, may have constitutional symptoms (fever, leukocytosis)
Ewing Sarcoma
69
Elevated levels of this cell type are seen in Ewing Sarcoma
Leukocytes
70
Destructive intramedullary tumor with permeative moth-eaten margins
Ewing Sarcoma
71
In this condition, reactive periosteal bone forms "onion skin" appearance
Ewing Sarcoma
72
In Ewing Sarcoma, reactive periosteal bone has this appearance
Onion skin
73
In Chondrosarcoma, periosteal reaction has this appearance
Endosteal scalloping
Codman triangle
74
Tumor that extends into soft tissues, with reactive periosteal bone that forms "onion skin" appearance
Ewing Sarcoma
75
Translocation in Ewing Sarcoma
t(11,22)
76
t(11,22) causes this condition
Ewing Sarcoma
77
In Ewing Sarcoma, t(11,22) forms this
EWSR1/FLI1 fusion protein
Results in unregulated growth on undifferentiated mesenchymal cell
78
Condition where a translocation forms a fusion protein that results in unregulated growth on unknown undifferentiated mesenchymal cell
Ewing Sarcoma
79
This is a primitive neuroectodermal tumor (PNET)
Ewing Sarcoma
80
Condition where sheets of small round blue cells are seen morphologically
Ewing Sarcoma
81
CD99 is a marker for this condition
Ewing Sarcoma
82
IHC marker for Ewing Sarcoma
CD99
83
Condition that begins in marrow space, erodes through cortex into soft tissues
Ewing Sarcoma
84
Condition that sometimes forms Homer-Wright rosettes with central fibrillary core
Ewing Sarcoma
85
Benign tumor of osteoblasts with numerous non-neoplastic osteoclasts
Giant cell tumor
86
What is the typical age of a patient with Giant cell tumor?
20-40's
87
Condition that occurs with closed growth plates, and expansile growth without penetrating joint space
Giant cell tumor
88
Does Giant cell tumor have surrounding sclerosis?
No
89
Condition with radiology showing thin rim of cortex and usually no surrounded sclerosis
Giant cell tumor
90
In Giant cell tumor, neoplastic osteoblasts produce this
RANK-L
results in osteoclast activation and proliferation (many cells with numerous nuclei)
91
Condition where neoplastic osteoblasts produce RANK-L, resulting in osteoclast activation and proliferation
Giant cell tumor
92
Abnormal bone growth resulting in abnormal osteoid in fibrous matrix
Fibrous dysplasia
93
Condition where clonal expansion occurs due to GNAS mutations
Fibrous dysplasia
94
Fibrous dysplasia involves clonal expansion due to mutations in this
GNAS
95
In Fibrous dysplasia, clonal expansion due to GNAS mutations occur during this process
Embryogenesis
96
In this condition, osteoblasts do not differentiate, leading to intramedullary lytic lesions
Proliferate but unable to produce mature lamellar bone
Fibrous dysplasia
97
What age does Fibrous dysplasia occur?
Early adolescence
98
Is Fibrous dysplasia painful?
No, most are symptomatic and discovered incidentally
99
Condition with monostotic and polyostotic forms that may be disfiguring or cause growth imbalances
Fibrous dysplasia
100
Polyostotic forms of Fibrous dysplasia are often part of this condition
McCune Albright Syndrome
101
Polyostotic forms of this condition are often part of McCune Albright Syndrome
Fibrous dysplasia
102
Condition involving endocrine dysfunction, female precocious puberty, and areas of cutaneous hyperpigmentation (cafe au lait)
McCune Albright Syndrome
103
Condition with expansile remodeling of marrow cavity, causing bone deformity
Fibrous dysplasia
104
Condition with a well-circumscribed intramedullary lesion and ground glass opacifications (may be lucent or opaque)
Fibrous dysplasia
105
Does Fibrous dysplasia have a sclerotic rim?
Yes
106
Condition with a fibrous stroma, variably mineralized osteoid with curvilinear shapes, and no osteoblastic rimming
Fibrous dysplasia
107
In Fibrous dysplasia, there is variably mineralized osteoid with this shape
Curvilinear
108
Condition with variably mineralized osteoid with curvilinear shapes
Fibrous dysplasia
109
Condition involving clonal proliferation of Langerhans cells
Langerhans cell histiocytosis
110
Condition that has the following three forms with identical histology:
Eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease
Langerhans cell histiocytosis
111
CD1a, S100, and Langerhin are IHC markers of this condition
Langerhans cell histiocytosis
112
Condition with mononuclear cells with grooved nuclei, eosinophilic infiltrate
Langerhans cell histiocytosis
113
Non-cutaneous form of Langerhans cell histiocytosis that is a localized single lesion, mostly lytic bone lesion, with spontaneous regression
Eosinophilic granuloma
114
Is Eosinophilic granuloma a localized or disseminated form of Langerhans cell histiocytosis?
Localized
115
What age is the typical patient with Eosinophilic granuloma (localized form of Langerhans cell histiocytosis)?
Birth to 6 years
116
2 most common sites of Eosinophilic granuloma (localized form of Langerhans cell histiocytosis)
Skull and vertebrae
117
Two main forms of disseminated Langerhans cell histiocytosis
Hand-Schuller-Christian disease
Letterer-Siwe disease
118
Is Hand-Schuller-Christian disease a localized or disseminated form of Langerhans cell histiocytosis?
Disseminated
119
Is Letterer-Siwe disease a localized or disseminated form of Langerhans cell histiocytosis?
Disseminated
120
Disseminated form of Langerhans cell histiocytosis involving multifocal bone lesions and can affect cranial nerve foramina
Hand-Schuller-Christian disease
121
Disseminated form of Langerhans cell histiocytosis with the classic triad of Multifocal bone lesions, retro-orbital and pituitary effects
Hand-Schuller-Christian disease
122
What is the classic triad seen in Hand-Schuller-Christian disease?
Multifocal bone lesions
Retro-orbital (exophthalmos)
Pituitary region (diabetes insipidus)
123
Disseminated form of Langerhans cell histiocytosis that is aggressive with early death
Dominated by visceral involvement
Letterer-Siwe disease
124
Disseminated forms of Langerhans cell histiocytosis present at this age
Birth to 6 years
125
Condition that begins as pink papules and pustules, looks like seborrheic dermatitis or eczema, with eventual multiorgan and bone involvement (hepatosplenomegaly, lymphadenopathy often)
Disseminated forms of Langerhans cell histiocytosis
126
Levels of Ca in metastatic bone tumors
High
126
4 most common locations where metastatic tumors from bone neoplasms occur
Prostate, breast, kidney, lung
127
Levels of alk phos in metastatic bone tumors
High
128
This cancer is the most common blastic metastasis
Prostate cancer
