Malignant Neoplasia of Bone Flashcards

1
Q

Primary malignant neoplasia of bone tissues account for ___% of all bone malignancy in the U.S.

A

<30%

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2
Q

What are the most common primary malignancies of bone?

A

Bone forming tumors: osteosarcoma
Cartilage forming tumors: chondrosarcoma
Tumors of other origin: Ewing sarcoma, multiple myeloma

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3
Q

Secondary malignant neoplasia of bone tissues account for ___% of all bone malignancy in the U.S.

A

70%

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4
Q

What would secondary malignant neoplasia of bone tissues be secondary to?

A

Metastatic disease

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5
Q

Osteosarcoma accounts for ___% of primary bone cancers

A

20%

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6
Q

What is the most common primary bone tumor in children?

A

Primary osteosarcoma (75% in individuals younger than 20 years)

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7
Q

What kind of osteosarcoma is more common in older adults with predisposing conditions such as Paget’s, previous radiation therapy, or bone infarcts?

A

Secondary osteosarcoma

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8
Q

Which bones are most commonly affected by primary osteosarcoma?

A

Metaphyseal region of long bones; knee accounts for 50% of cases

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9
Q

Which bones are most commonly affect by secondary osteosarcoma?

A

Flat bones

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10
Q

Which genes are mutated in the pathogenesis of osteosarcoma?

A

Tumor suppressor and oncogenes:

  • RB
  • TP53
  • INK4a
  • MDM2 and CDK4
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11
Q

osteosarcoma

What is the RB gene?

A

Tumor suppressor gene; negative regulator of cell cycle

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12
Q

osteosarcoma

What is the TP53 gene?

A

Tumor suppressor gene; encode p53 which plays an important role in promoting DNA repair and apoptosis of damaged cells

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13
Q

osteosarcoma

What is the INK4a gene?

A

Gene that encodes 2 tumor suppressors (p16 and p14)

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14
Q

osteosarcoma

What are the MDM2 and CDK4 genes?

A

Inhibit p53 and RB function; overexpressed in many osteosarcomas

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15
Q

Is osteosarcoma blastic or lytic?

A

Mixed blastic and lytic mass; formation of bone by tumor is diagnostic in biopsy

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16
Q

Osteosarcoma features ___ margins

A

irregular margins

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17
Q

How might periosteum be affected by osteosarcoma?

A

May life or penetrate periosteum; reactive bone formation

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18
Q

Does osteosarcoma invade other tissues?

A

Yes, frequently metastasizes to the lung, brain, or other bones

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19
Q

What is the 5-year survival rate for osteosarcoma?

A

60%

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20
Q

What are clinical manifestations of osteosarcoma?

A
  • Pain and swelling around the site of the tumor
  • Possibly palpable tumor
  • Pathological fracture
  • Abnormal labs
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21
Q

What abnormal lab findings are present with osteosarcoma?

A
  • Increased ESR/C-reactive protein
  • Increased alkaline phosphatase
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22
Q

If the following radiographic characteristics are found, what is suspected?

  • Pathological fracture
  • Radiopaque mass with spiculated pattern
  • Periosteal lifting/disruption is Codman’s triangle pattern
  • Long zone of transition
A

Osteosarcoma

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23
Q

What are the radiographic characteristics of osteosarcoma?

A
  • Pathological fracture
  • Radiopaque mass with spiculated pattern
  • Periosteal lifting/disruption in Codman’s triangle pattern
  • Long zone of transition
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24
Q

What is a malignant bone forming tumor?

A

Osteosarcoma; produces unmineralized osteoid or mineralized woven bone

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25
Q

What is a malignant cartilage forming tumor?

A

Chondrosarcoma

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26
Q

Chondrosarcomas are subclassified by…

A

tissue and location

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27
Q

The conventional type of chondrosarcoma is most common
What cartilage is produced by this cancer?

A

Hyaline cartilage

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28
Q

What are the classifications of chondrosarcoma by location?

A
  • Central (intramedullary)
  • Juxtacortical (peripheral)
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29
Q

At what rate do chondosarcomas grow?

A

Very slow growing

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30
Q

What histological differences can be found in chondrosarcoma?

A
  • Malignant chondrocytes
  • Necrosis
  • Hemorrhage
  • Stippled calcifications
  • Cystic change
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31
Q

What are common bone locations for chondrosarcoma?

A
  • Pelvis
  • Ribs
  • Long bones (especially femur and humerus)
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32
Q

At what ages are most chondrosarcomas diagnosed?

A

40-60 years of age
(exception: clear and mesenchymal subtypes in teens or 20s)

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33
Q

What is the distribution of chondrosarcoma among sexes?

A

Male > Female (2:1)

34
Q

Where is chondrosarcoma most likely to metastasize?

A
  • Lungs
  • Liver
  • Kidneys
  • Brain
35
Q

Depending upon differentiation, what is the 5-year survival rate for chondrosarcoma?

A

20-80%

36
Q

What are the clinical manifestations of chondrosarcoma?

A
  • Insidious onset; up to 2 years
  • Pain at affected site
  • Possible palpable soft tissue mass
37
Q

If the following radiographic findings are present, what is suspected?

  • Localized bone destruction
  • Poorly-defined borders with cortex perforation
  • Radiopacities due to calcification or endochondral ossification
  • Soft tissue masses may be visible
A

Chondrosarcoma

38
Q

What are the radiographic findings of chondrosarcoma?

A
  • Localized bone destruction
  • Poorly-defined borders with cortex perforation
  • Radiopacities due to calcification or endochondral ossification
  • Soft tissue masses may be visible
39
Q

Drug and radiation therapy are ___ effective in treating chondrosarcomas than other malignant bone tumors

A

less effective (cartilage isn’t well vascularized)

40
Q

What is a cause of malignant tumors of bone marrow cells?

A

Multiple myeloma

41
Q

What is the most common primary malignant bone tumor?

A

Multiple myeloma

42
Q

What is the average age of diagnosis for multiple myeloma?

A

65 years of age (50-70)

43
Q

What is the distribution of multiple myeloma among sexes?

A

Male > Female (2:1)

44
Q

What is the etiology of multiple myeloma?

A

Unknown

45
Q

Myeloma cells secrete ___ that recruit ___

A

cytokines that recruit osteoclasts

46
Q

What type of bone lesions are produced by multiple myeloma cytokine secretion?

A

Lytic bone lesions (osteoclasts recruited)

47
Q

What is the size of multiple myeloma lesions?

A

Multiple 1-5mm lesions

48
Q

Which bones are most frequently affected by multiple myeloma?

A
  • Skull
  • Spine
  • Ribs
  • Pelvis
  • Femur
49
Q

Histologically, multiple myeloma lesions consist of…

A

sheets of plasma cells with varying degrees of differentiation

50
Q

Which cardinal signs of inflammation are present in multiple myeloma?

A

Lack of cardinal signs

51
Q

What are some clinical manifestations of multiple myeloma?

A
  • Weight loss, cachexia (muscle loss, fat retained)
  • Pain
  • Hypercalcemia
  • Anemia
52
Q

What sort of pain presents with multiple myeloma?

A
  • Often intermittent, becomes continuous
  • Possible rapid onset following slight strain or mild trauma
  • Worse during day, aggravated by activity and weight bearing
53
Q

A patient reports the following about their pain:

  • Rapid onset following slight strain or mild trauma
  • At first, intermittent, then continuous
  • Worse during day
  • Aggravated by activity and weight bearing

Which malignant bone tumor is suspected?

A

Multiple myeloma

54
Q

___ is seen in 10% of patients with multiple myeloma

A

Amyloidosis (amyloid deposits)

55
Q

How does multiple myeloma present radiographically?

A
  • Punched out lesions
  • Rain drop skull
56
Q

If a patient has production of serum and urinary proteins called Bence-Jones proteins, which malignant bone tumor is suspected?

A

Multiple myeloma

57
Q

There is overproduction of ___ in 95% of multiple myeloma patients

A

immunoglobulins

58
Q

Multiple myeloma may cause production of serum and urinary proteins called Bence-Jones proteins
What may be the result of this?

A

Renal failure

59
Q

What are treatment options for multiple myeloma?

A

Treatment options are still very poor

60
Q

What is the average survival rate for multiple myeloma?
What is the common cause of death?

A

Average survival rate of 5 years
Infection or renal failure are common causes of death

61
Q

What is a malignant tumor of neuroectodermal cells?

A

Ewing sarcoma

62
Q

What is the second most common primary bone tumor in children accounting for up to 10% of primary bone tumors?

A

Ewing sarcoma

63
Q

80% of Ewing sarcomas are diagnosed in individuals ___ years of age

A

<20 years of age

64
Q

What is the distribution of Ewing sarcoma among sexes?

A

Males > Females (2:1)

65
Q

What is the etiology of Ewing sarcoma?

A

Still poorly understood; 90% of cases show translocation between chromosomes 11 and 22

66
Q

If histology presents as follows, what is suspected?

  • Closely packed, small, round cells without obvious differentiation
  • Arranged in uniform sheets
  • Cells have little cytoplasm
A

Ewing sarcoma

67
Q

What is the histology of Ewing sarcoma?

A
  • Primitive neuroectodermal cells
  • Closely packed, small, round cells, without obvious differentiation
  • Arranged in uniform sheets
  • Cells have little cytoplasm
68
Q

___% of Ewing sarcoma cases are extraskeletal

A

10-20%

69
Q

Ewing sarcoma arises in ___ and grows ___

A

arises in medullary cavity and grows outward

70
Q

Which bones are commonly affected by Ewing sarcoma?

A

Femur, other long bones, and pelvis
(Mid-diaphysis of long bones)

71
Q

If a patient presents with the following, which malignant bone tumor is suspected?

  • Deep boring pain in enlarging masses
  • Tender, warm, and or swollen site
  • Mimicking infection: fever, increased ESR, leukocytosis
  • Unexplained weight loss
  • Weakness
  • Anemia
A

Ewing sarcoma

72
Q

What are some clinical manifestations of Ewing sarcoma?

A
  • Deep boring pain in enlarging masses
  • Tender, warm, and or swollen site
  • Mimicking infection: fever, increased ESR, leukocytosis
  • Unexplained weight loss
  • Weakness
  • Anemia
73
Q

What are some treatments for Ewing sarcoma?
What is the 5-year survival rate?

A

Chemotherapy, radiation, and limb-sparing surgery
60-75% 5-year survival rate

74
Q

If the following radiographic characteristics are present, which malignant bone tumor is suspected?

  • Permeative lytic destruction
  • Long zone of transition
  • Cortical disruption
  • Laminated or spiculated periosteal response
  • May extend into soft tissue
A

Ewing sarcoma

75
Q

What are the radiographic characteristic of Ewing sarcoma?

A
  • Permeative lytic destruction
  • Long zone of transition
  • Cortical disruption
  • Laminated or spiculated periosteal response
  • May extend into soft tissue
76
Q

Metastatic bone disease is also known as…

A

secondary bone tumors

77
Q

What is the most common malignant tumor of bone?

A

Secondary bone tumors (metastatic)

78
Q

Metastatic bone tumors have most commonly metastasized from:

A

breast, prostate, kidney, or lung cancer

79
Q

Which components of the skeletal system are the most common sites of metastasis/metastatic tumors?

A
  • Vertebrae
  • Pelvis
  • Ribs
  • Skull
  • Sternum
80
Q

What are some clinical manifestations of metastatic bone tumors?

A
  • Unexplained weight loss
  • Anemia
  • Possible fever with late-stage disease
  • Abnormal labs
81
Q

How do metastatic bone tumors present radiographically?

A

Osteoblastic, osteolytic, or mixed