Lab: Tumor-like Conditions & Neoplasias Flashcards

1
Q

What type of bone response is common in the first stage of Paget disease?
How does this appear radiographically?

A

Osteoclastic/osteolytic response appears radiolucent

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2
Q

What type of bone response is common in the second stage of Paget disease?
How does this appear radiographically?

A

Osteoclastic and osteoblastic bone response (mixed) appears radiolucent and radiopaque

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3
Q

What type of bone response is common in the third stage of Paget disease?
How does this appear radiographically?

A

“Burn out”; won’t see much activity, everything that has already happened is still radiographically visible

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4
Q

What are the signs/symptoms of a simple bone cyst vs an aneurysmal bone cyst?

A

Simple bone cyst: asymptomatic, incidental finding
Aneurysmal bone cyst: pain, swelling, fracture, palpable

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5
Q

What is the radiographic appearance of a simple bone cyst?

A
  • Radiolucent
  • Sclerotic border
  • Eucentric
  • Non-expansile
  • Unilocular
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6
Q

What is the radiographic appearance of an aneurysmal bone cyst?

A
  • Radiolucent
  • Sclerotic border
  • Eccentric
  • Expansile
  • Multilocular (soap bubble)
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7
Q

What is found histologically in biopsy of a simple bone cyst vs an aneurysmal bone cyst?

A

Simple bone cyst: serous fluid
Aneurysmal bone cyst: blood

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8
Q

What are the signs/symptoms of a benign bone tumor?

A
  • No pain
  • Slow growing
  • Palpable/swelling possible

osteoid osteomas are benign but very painful

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9
Q

What are signs/symptoms of a malignant bone tumor?

A
  • Cardinal signs of inflammation
  • Abnormal blood work
  • Fast growing/changing
  • Unexpected weight loss
  • Hypercalcemia
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10
Q

What is the radiographic appearance of a benign bone tumor?

A
  • Short zone of transition (defined border)
  • No cortical/periosteal disruption
  • No soft tissue effects
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11
Q

What is the radiographic appearance of a malignant bone tumor?

A
  • Long zone of transition
  • Cortical/periosteal disruption
  • Soft tissue effects
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12
Q

What are some benign bone forming tumors found in bone?

A
  • Osteoma
  • Osteoid osteoma
  • Osteoblastoma
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13
Q

What are some benign cartilage forming tumors found in bone?

A
  • Osteochondroma
  • Chondroma
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14
Q

What is a benign tumor of unknown origin found in bone?

A

Giant cell tumor

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15
Q

What are some malignant primary bone forming tumors found in bone?

A

Osteosarcoma (primary)

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16
Q

What are some malignant cartilage forming tumors found in bone?

A

Chondrosarcoma

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17
Q

What are some malignant primary tumors of unknown origin found in bone?

A
  • Ewing sarcoma
  • Multiple myeloma
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18
Q

This condition may be mistaken for a tumor-like condition.
What pathology is present?

A

Paget disease

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19
Q

What is an important lab result found in this patient?

A

Increased alkaline phosphatase

Paget disease

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20
Q

What are some manifestations of this pathology?

A
  • Osteoporosis circumscripta
  • Bone enlargement
  • Localized pain
  • Pagetic steal
  • Hearing impairment

Paget disease

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21
Q

What is the name for this deformity?
What is seen at the green arrow?

A

Sabre shin
Banana fracture/insuffiency pathological fracture

Paget disease

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22
Q

Is this bone tumor likely benign or malignant?

A

Benign

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23
Q

Notice the radiolucent radiopaque mixed appearance in this enlarged bone with cortical thickening.
What pathology is present?

A

Paget disease

sabre shin

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24
Q

What is the appearance of this lesion?
What pathology is present?

A

Lesion is unicameral/unilocular osteolytic lesion fibrous lining filled with serous fluid
Simple bone cyst

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25
Q

What is the typical location of this lesion?
How large will it grow?

A

Typically in eucentric metaphysis
Grows no larger than width of bone (non-expansile)

simple bone cyst

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26
Q

What is the typical age and sex of a patient with this presentation?

A

<20-year-old male

simple bone cyst

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27
Q

This lesion has led to what consequence shown in this radiograph?

A

Complete humeral fracture (pathological)

simple bone cyst

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28
Q

What is the appearance of this lesion?
What pathology is present?

A

Appears multilocular with visible fibrous septa, widening cortex (expansile)
Aneurysmal bone cyst

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29
Q

What is the typical age of those with monostotic fibrous dysplasia vs those with polyostotic fibrous dysplasia?

A

Monostotic: 10-30 years
Polyostotic: early childhood

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30
Q

What is the distribution of sex amongst those with monostotic fibrous dysplasia vs those with polyostotic fibrous dysplasia?

A

Monostotic: Males = Females
Polyostotic: Females > Males

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31
Q

What is the nature of lesions in monostotic fibrous dysplasia vs those in polyostotic fibrous dysplasia?

A

Monostotic: focal expanding lesion
Polyostotic: lesions in multiple bones

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32
Q

What are the locations of monostotic fibrous dysplasia vs polyostotic fibrous dysplasia?

A

Monostotic: (proximal) femur, ribs, tibia, facial bones
Polyostotic: more than 50% of skeleton affected

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33
Q

What are the effects of monostotic fibrous dysplasia on bone?

A
  • Enlargement or deformation
  • Pathologic fracture
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34
Q

What are the effects of polyostotic fibrous dysplasia on bone?

A
  • Enlargement or deformation
  • Limb length discrepancies
  • Spine alignment issues
  • Pathologic fracture
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35
Q

What are the syndromes associated with polyostotic fibrous dysplasia?

A
  • Mazabraud syndrome
  • Mcune-Albright syndrome
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36
Q

With fibrous dysplasia, failure to form ___ bone leads to ___ lesions

A

mature lamellar bone leads to osteolytic/radiolucent lesions

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37
Q

What is within the well defined lesions of fibrous dysplasia?

A
  • Fibroblasts
  • Collagen
  • Irregular trabeculae
  • Cystic degeneration
  • Slight hemorrhage
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38
Q

A well defined lesion is biopsied and finds the following contents:

  • Fibroblasts
  • Collagen
  • Irregular trabeculae
  • Cystic degeneration
  • Slight hemorrhage

What bone pathology is present?

A

Fibrous dysplasia

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39
Q

How does soft tissue involvement differ between benign and malignant neoplasia of bone?

A

Benign: no soft tissue involved
Malignant: soft tissue may be involved

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40
Q

How does cortex and periosteum involvement differ between benign and malignant neoplasia of bone?

A

Benign: cortex remains intact, periosteum unaffected
Malignant: cortex can be disrupted, periosteum can be perforated or lifted

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41
Q

How does the zone of transition differ between benign and malignant neoplasia of bone?

A

Benign: short (well-defined)
Malignant: long (poorly defined or irregular)

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42
Q

Is this bone neoplasia likely benign or malignant?

A

Malignant

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43
Q

A 6-year-old male presents for an annual physical. The parent explains that the child has been experiencing upper right thigh pain for 4 months. Previous doctor’s visits revealed no abnormalities upon a physical exam. The parent is seeking a second opinion. The physical exam does not indicate any issues and a radiograph is taken.

How would you describe the location of the lesion indicated by the green arrow?
How does it affect the cortex and periosteum?

A

Metaphysis medullary cavity location
Cortex and periosteum unaffected

osteoid osteoma

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44
Q

A 6-year-old male presents for an annual physical. The parent explains that the child has been experiencing upper right thigh pain for 4 months. Previous doctor’s visits revealed no abnormalities upon a physical exam. The parent is seeking a second opinion. The physical exam does not indicate any issues and a radiograph is taken.

Does soft tissue appear to be affected by the lesion?
Is the zone of transition short or long?

A

Soft tissues unaffected
Short zone of transition

osteoid osteoma

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45
Q

A 6-year-old male presents for an annual physical. The parent explains that the child has been experiencing upper right thigh pain for 4 months. Previous doctor’s visits revealed no abnormalities upon a physical exam. The parent is seeking a second opinion. The physical exam does not indicate any issues and a radiograph is taken.

Is this lesion consistent with a non-aggressive or aggressive lesion?

A

Non-aggressive (likely benign)

osteoid osteoma

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46
Q

What pathologies have we discussed that share a similar radiographic presentation of a radiolucency with a sclerotic border?

A
  • Brodie abscess
  • Osteoid osteoma
  • Simple bone cysts
  • Benign catilaginous tumor
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47
Q

A 6-year-old male presents for an annual physical. The parent explains that the child has been experiencing upper right thigh pain for 4 months. Previous doctor’s visits revealed no abnormalities upon a physical exam. The parent is seeking a second opinion. The physical exam does not indicate any issues and a radiograph is taken.

Which condition most closely aligns with this case?

A

Osteoid osteoma

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48
Q

A 6-year-old male presents for an annual physical. The parent explains that the child has been experiencing upper right thigh pain for 4 months. Previous doctor’s visits revealed no abnormalities upon a physical exam. The parent is seeking a second opinion. The physical exam does not indicate any issues and a radiograph is taken.

Why would aspirin relieve this patient’s pain?

A

Aspirin is a vasoconstrictor

osteoid osteoma

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49
Q

Is this lesion osteolytic or osteoblastic?
How would it appear on a radiograph?

A

Osteolytic
Would appear radiolucent likely with sclerotic border

simple bone cyst

50
Q

Is this lesion eucentric or eccentric?
Is it singular or multilocular?

A

Eucentric
Unilocular

simple bone cyst

51
Q

Note the appearance of this lesion.
What is the likely diagnosis?

A

Simple bone cyst

benign

52
Q

What is the appearance of this pathology?

A

Eucentric, unilocular, radiolucent lesion with sclerotic border

simple bone cyst

53
Q

Is this lesion likely expansile?

A

Non-expansile

simple bone cyst

typically in metaphysis, but has a tendency to diaphysis as maturity continues

54
Q

What type of fluid would be found in this patient’s lesion in a tissue biopsy?

A

Serous fluid

simple bone cyst

55
Q

How might this patient present clinically?

A

Asymptomatic, or a fracture leads to incidental finding

simple bone cyst

56
Q

Is this lesion eccentric or eucentric?
Unilocular or multilocular?
Expansile or non-expansile?

A

Eccentric
Multilocular
Expansile

aneurysmal bone cyst

57
Q

What type of fluid would fill this lesion?

A

Blood

aneurysmal bone cyst

58
Q

How will this bone cyst appear on a radiograph?

A
  • Multilocular
  • Radiolucent
  • Sclerotic border
  • Expansile
  • Eccentric

aneurysmal bone cyst

59
Q

A 35-year-old female presents with knee pain. When asked, the patient states that the pain began several months prior and has gotten worse. Radiographs are taken.

What is the location of this lesion?
Has the cortex been disrupted?

A

Located in epiphysis, metaphysis, and metadiaphysis medullary cavity
Cortex has not been disrupted

giant cell tumor

60
Q

A 35-year-old female presents with knee pain. When asked, the patient states that the pain began several months prior and has gotten worse. Radiographs are taken.

Is this lesion singular or multiloculated?
Is it aggressive?
Is it benign or malignant?

A

Multiloculated
Non-aggressive
Benign

giant cell tumor

61
Q

A 35-year-old female presents with knee pain. When asked, the patient states that the pain began several months prior and has gotten worse. Radiographs are taken.
Note this radiolucent lesion with a sclerotic border. It is multiloculated with a short zone of transition, not disrupting the cortex.

What pathologies share a similar radiographic presentation?

A
  • Giant cell tumor
  • Aneurysmal bone cyst

benign

62
Q

You are working at an off-campus sporting event at a local high school when you feel a lump on the thigh of a 15-year-old male. You ask him how long the lump has been present and he says “a while” and says it might have gotten bigger recently. He doesn’t feel pain. This is his radiograph.

What is the location of the lesion?

A

Metaphysis extending from the cortex

osteochondroma

63
Q

You are working at an off-campus sporting event at a local high school when you feel a lump on the thigh of a 15-year-old male. You ask him how long the lump has been present and he says “a while” and says it might have gotten bigger recently. He doesn’t feel pain. This is his radiograph.

Does the lesion have a short or long zone of transition?
Is it likely aggressive?

A

Short zone of transition
Likely non-aggressive

osteochondrome

64
Q

You are working at an off-campus sporting event at a local high school when you feel a lump on the thigh of a 15-year-old male. You ask him how long the lump has been present and he says “a while” and says it might have gotten bigger recently. He doesn’t feel pain. This is his radiograph.

This lesion is extending from the cortex without disrupting it. Is the periosteum intact? Is soft tissue affected?

A

Periosteum is not lifted
Lesion is not invading soft tissues

osteochondroma

65
Q

You are working at an off-campus sporting event at a local high school when you feel a lump on the thigh of a 15-year-old male. You ask him how long the lump has been present and he says “a while” and says it might have gotten bigger recently. He doesn’t feel pain. This is his radiograph.

What specific term could you use to describe the shape of this lesion?

A

Pedunculated

osteochondroma

66
Q

You are working at an off-campus sporting event at a local high school when you feel a lump on the thigh of a 15-year-old male. You ask him how long the lump has been present and he says “a while” and says it might have gotten bigger recently. He doesn’t feel pain. This is his radiograph.

What would you expect this patient’s lab results to be?
ESR:
Alkaline phosphatase:

A

ESR: normal
Alkaline phosphate: increased

osteochondroma

67
Q

You are working at an off-campus sporting event at a local high school when you feel a lump on the thigh of a 15-year-old male. You ask him how long the lump has been present and he says “a while” and says it might have gotten bigger recently. He doesn’t feel pain. This is his radiograph.

What is the most likely diagnosis for this individual?

A

Osteochondroma

painless, lumpy joint

68
Q

Label the image shown.

Green arrows:
Purple arrows:
Red arrows:

A

Green arrows: perichondrium (fibrous tissue lining)
Purple arrows: hyaline cartilage
Red arrows: bone

osteochondroma

69
Q

John is a teenage male experiencing knee pain for over a month. When you ask about the pain, he says it is deep, right above his knee. Sometimes it keeps him awake at night. He doesn’t recall any injuries. This is his radiograph.

What is the location of this lesion?
Is it disrupting the cortex? The periosteum?

A

Metaphysis, diaphysis, medullary cavity
Disrupting the cortex and affecting the periosteum

osteosarcoma

70
Q

John is a teenage male experiencing knee pain for over a month. When you ask about the pain, he says it is deep, right above his knee. Sometimes it keeps him awake at night. He doesn’t recall any injuries. This is his radiograph.

Does this lesion have a short or long zone of transition?
Is it likely aggressive?

A

Long zone of transition
Likely aggressive

osteosarcoma

71
Q

John is a teenage male experiencing knee pain for over a month. When you ask about the pain, he says it is deep, right above his knee. Sometimes it keeps him awake at night. He doesn’t recall any injuries. This is his radiograph.

Is this primarily an osteoblastic or osteolytic pathology?

A

Osteoblastic; sunburst appearance (Sharpey’s fibers) of spiculated periosteum

72
Q

John is a teenage male experiencing knee pain for over a month. When you ask about the pain, he says it is deep, right above his knee. Sometimes it keeps him awake at night. He doesn’t recall any injuries. This is his radiograph.

Is soft tissue likely to be affected by this neoplasia?
Is it benign or malignant?

A

Soft tissue likely affected because of cortex and periosteal involvement
Malignant

osteosarcoma

73
Q

John is a teenage male experiencing knee pain for over a month. When you ask about the pain, he says it is deep, right above his knee. Sometimes it keeps him awake at night. He doesn’t recall any injuries. This is his radiograph.

What would be this patient’s lab results?
ESR:
CRP:
Alkaline phosphatase:

A

ESR: elevated
CRP: elevated
Alkaline phosphatase: increased

osteosarcoma

74
Q

John is a teenage male experiencing knee pain for over a month. When you ask about the pain, he says it is deep, right above his knee. Sometimes it keeps him awake at night. He doesn’t recall any injuries. This is his radiograph.

What is the most likely diagnosis for this patient?

A

Osteosarcoma

spiculated radiopaque mass, deep bone pain keeping patient up at night

75
Q

A 57-year-old man presents with long-standing, painful deformation of the right tibia. He reported increased volume of the right leg since late adolescence and three low-impact fractures in the fourth decade of life. He was treated 15 years before presentation with calcitonin for 6 months, without symptomatic relief. Physical examination revealed a prominent deformity of the tibia and superficial venous circulation related to chronic venous insufficiency.

What pathologies have we discussed that could cause tibial bowing?
Which of these cause bowing unilaterally vs bilaterally?

A

Unilateral bowing: Pagets disease
Bilateral bowing: Syphilis, Rickets

76
Q

A 57-year-old man presents with long-standing, painful deformation of the right tibia. He reported increased volume of the right leg since late adolescence and three low-impact fractures in the fourth decade of life. He was treated 15 years before presentation with calcitonin for 6 months, without symptomatic relief. Physical examination revealed a prominent deformity of the tibia and superficial venous circulation related to chronic venous insufficiency.
These are radiographs of his right tibia.

What observations can you make about the changes in this bone? Are these changes osteoblastic, osteolytic, or mixed?

A

Coarse trabecular expansion (highly vascular); bone enlargement with cortical thickening
Mixed blastic and lytic response

Pagets disease

77
Q

A 57-year-old man presents with long-standing, painful deformation of the right tibia. He reported increased volume of the right leg since late adolescence and three low-impact fractures in the fourth decade of life. He was treated 15 years before presentation with calcitonin for 6 months, without symptomatic relief. Physical examination revealed a prominent deformity of the tibia and superficial venous circulation related to chronic venous insufficiency.
These are radiographs of his right tibia.

Based on the number of bones affected in this clinical presentation and the radiograph, what term could you use to describe this condition?

A

Monostotic

Pagets disease

78
Q

What could increased alkaline phosphatase indicate about bone metabolism?

A

Increased osteoblastic activity

79
Q

What do increased type I collagen byproducts (P1NP and b-CTX) indicate about bone metabolism?

A

Increased bone turnover (decreased bone quality, increased fracture risk)

80
Q

A 57-year-old man presents with long-standing, painful deformation of the right tibia. He reported increased volume of the right leg since late adolescence and three low-impact fractures in the fourth decade of life. He was treated 15 years before presentation with calcitonin for 6 months, without symptomatic relief. Physical examination revealed a prominent deformity of the tibia and superficial venous circulation related to chronic venous insufficiency.
These are radiographs of his right tibia.

What pathology do you think this patient is most likely experiencing?

A

Pagets disease

81
Q

What term describes the anterior bowing of the tibia in this patient’s case?

A

Sabre shin

Pagets disease

82
Q

How is the cause of this anterior bowing different from that seen in syphilis?

A

Pagets disease: physical tibial bowing with coarse trabecular expansion
Syphilis: periosteal reaction creates appearance of tibial bowing without actual bowing

83
Q

Your 55-year-old male patient is usually lively, but has recently been withdrawn and tired. He complains of back pain and has lost some weight recently without explanation. You perform soft tissue work on his back and order blood work and radiographs.

Are the changes in the bones of this skull osteolytic or osteoblastic?
Does it appear aggressive?

A

Osteolytic, appears aggressive

multiple myeloma

84
Q

Your 55-year-old male patient is usually lively, but has recently been withdrawn and tired. He complains of back pain and has lost some weight recently without explanation. You perform soft tissue work on his back and order blood work and radiographs.

His lab results are as follows:

  • ESR normal
  • WBC normal
  • RBC decreased (anemia)
  • Urinalysis positive for proteins

Which proteins were likely in the urine?

A

Bence-Jones proteins

multiple myeloma

85
Q

Your 55-year-old male patient is usually lively, but has recently been withdrawn and tired. He complains of back pain and has lost some weight recently without explanation. You perform soft tissue work on his back and order blood work and radiographs.

Is the zone of transition of these lesions long or short?
Is there evidence of cortical disruption or periosteal lifting?

A

Long zone of transition
Cortical disruption and periosteal lifting (purple arrow)

multiple myeloma

86
Q

Your 55-year-old male patient is usually lively, but has recently been withdrawn and tired. He complains of back pain and has lost some weight recently without explanation. You perform soft tissue work on his back and order blood work and radiographs.

Given that this patient has a normal ESR and WBC count, but presents with anemia and proteinuria, what is their diagnosis?

A

Multiple myeloma

notice the “rain drop” skull; also no inflammatory signs

87
Q

Your 55-year-old male patient is usually lively, but has recently been withdrawn and tired. He complains of back pain and has lost some weight recently without explanation. You perform soft tissue work on his back and order blood work and radiographs.

The presence of Bence-Jones proteins in the urine will lead to what sequela of this disease?

A

Renal failure (hypercalcemia due to osteolysis)

multiple myeloma

88
Q

What is the most common primary malignant bone tumor?

A

Multiple myeloma

also note rain drop skull and other flat bones affected

89
Q

Your 55-year-old male patient is usually lively, but has recently been withdrawn and tired. He complains of back pain and has lost some weight recently without explanation. You perform soft tissue work on his back and order blood work and radiographs.

What would explain this patient’s lab result of anemia?

A
  • Flat bones (intramembranous) are responsible for making bone marrow
  • Multiple myeloma is osteolytic and destroys bone marrow
  • Less bone marrow means less red blood cells
90
Q

Your 55-year-old male patient is usually lively, but has recently been withdrawn and tired. He complains of back pain and has lost some weight recently without explanation. You perform soft tissue work on his back and order blood work and radiographs.

A biopsy is taken from this patient. After seeing “rain drop” lesions in their skull and finding Bence-Jones protein in their urine, what would you expect the histological composition of the biopsy to be?

A

Lesions have sheets of plasma cells with varying degrees of differentiation

multiple myeloma

91
Q

A 50-year-old female presents with elbow pain. She has no history of cancer and no other cardinal signs of inflammation. Radiographs are taken of her elbow.

Is this lesion likely aggressive?

A

Yes: cortex completely disrupted, long zone of transition

secondary bone tumor: metastatic from breast

92
Q

This is a case of metastatic prostate cancer.

Is this an osteolytic or blastic expression?
Is the zone of transition long or short?
Is the cortex affected?

A

Mixed osteolytic/blastic
Long zone of transition
Cortex disrupted

93
Q

This is a case of metastatic prostate cancer.

How might this patient present in your office?

A
  • Unexplained weight loss
  • Anemia with fatigue
  • Pain
  • Abnormal labs
  • Possible fever with late stage disease
94
Q

What is the difference between a primary and secondary bone tumor?

A

Primary is malignant neoplasia of bone tissue origin
Secondary is metastatic (always malignant) disease moved to bone

95
Q

Primary bone tumors make up ___% of bone malignancy in the US
Secondary bone tumors make up ___%

A

Primary: less than 30%
Secondary: 70%

96
Q

What are the four most common primary bone malignancies?

A
  • Osteosarcoma (primary or secondary)
  • Chondrosarcoma
  • Ewing sarcoma
  • Multiple myeloma
97
Q

What are some examples of benign primary bone tumors?

A
  • Osteoma
  • Osteochondroma
98
Q

What are three pathologies that have multiple large osteolytic lesions in bone as seen?

A
  • Metastatic tumors (secondary bone neoplasia)
  • Multiple myeloma (primary bone neoplasia)
  • Chronic osteomyelitis (chronic infection)
99
Q

What is shown in this spine?

A

Chronic infection (osteomyelitis)

cortical destruction and lytic migration into multiple tissue types

100
Q

This was a 75-year-old male. Tissue biopsy reveals malignant plasma cells.

What is the correct diagnosis?

A

Multiple myeloma

plasma based

101
Q

The skeleton can be affected by spread through Batson’s venous plexus.
What is the term for this type of spread of neoplasia?

A

Hematogenous spread

102
Q

Which areas of the skeleton are most affected by spread through Batson’s venous plexus?

A
  • Thoracolumbar spine
  • Vertebrae (in general)
  • Pelvis
  • Ribs
  • Skull
  • Sternum
103
Q

This is a primary bone tumor.
Is it malignant or benign?

A

Malignant

Ewing sarcoma

disrupted cortex (purple arrow), lifted periosteum (red arrow)

104
Q

Where did this tumor originate in the bone?

A

Originated in mid diaphysis medullary cavity and expanded outwards

Ewing sarcoma

105
Q

A tissue biopsy is performed on this specimen and reveals small, round, closely packed, malignant cells.

What is the most likely pathology?

A

Ewing sarcoma

primary malignant bone neoplasia

106
Q

Note the appearance indicated by the purple arrow.
What would create this appearance?
Is this aggressive?

A

Laminating periosteal reaction
Aggressive

Ewing sarcoma

107
Q

An 80-year-old woman presents with headaches and occasional dizziness. She has recently fallen and hit her head, resulting in some bruising and external wounds. She has a history of diabetes mellitus, hearing loss, and advanced arthritis. To rule out fracture, radiographs are taken.

What are the effects on the cortex?
Are the changes osteoclastic or osteolytic?

A

Cortex is dramatically thickened (outward)
Mixed blastic and lytic changes

osteitis deformans (Paget disease)

cotton wool skull

108
Q

An 80-year-old woman presents with headaches and occasional dizziness. She has recently fallen and hit her head, resulting in some bruising and external wounds. She has a history of diabetes mellitus, hearing loss, and advanced arthritis. To rule out fracture, radiographs are taken.

What pathologies have we discussed that may have this appearance?

A
  • Osteitis deformans (Paget disease)
  • Secondary bone tumors (can be mixed reaction)
109
Q

An 80-year-old woman presents with headaches and occasional dizziness. She has recently fallen and hit her head, resulting in some bruising and external wounds. She has a history of diabetes mellitus, hearing loss, and advanced arthritis. To rule out fracture, radiographs are taken.

Blood work indicates normal ESR and CRP, but an elevated alkaline phosphatase levels.
Based on the radiograph and this information, what pathology is likely present?

A

Osteitis deformans (Paget disease)

110
Q

Given that this patient has Paget disease, what is expected to be found in biopsy?

A
  • Irregular woven bone
  • Intertrabecular fibrosis
  • High vascularity
  • Deep resorption lacunae
  • Multiple, large osteoclasts
111
Q

This is a biopsy of a mixed osteoblastic and lytic bone neoplasia. Trabeculae has coarsened and the cortex has thickened. The biopsy shows irregular woven bone with intertrabecular fibrosis. The red portions in this image evidence high vascularity.
Higher magnifications show resorption of lacunae with many large osteoclasts.

What pathology is present?

A

Pagets disease (osteitis deformans)

112
Q

This patient has Pagets disease/osteitis deformans.
What term describes this pathology when it affects one bone?
Two or more bones?
All bones?

A

One bone: monostotic
Two or more bones: polyostotic
All bones: systemic

113
Q

An older patient is reporting that his hats are fitting tighter lately. You know he has a history of osteoarthritis and wears hearing aids. You notice his jaw is mishapen and when asked about any trauma, he reports he’s lost a few teeth in the past year and had a low impact fracture through his shin recently. Upon examination, your patient displays cerebellar gait changes. He experiences pain when performing spinal extension, but is palliated in flexion.

Without further radiographs or labs, what bony pathology is on your differential diagnosis?

A

Pagets disease/osteitis deformans

note: possible pathological “banana” fracture of sabre shin, “hat doesn’t fit” is possible cortical thickening in skull

114
Q

A 10-year-old male presents with pain and swelling in the right lower extremity after a sharp pain while running during football. He can no longer put weight on his leg. You note swelling and tenderness of the right ankle. The boy describes a throbbing pain during examination.

What type of injury is indicated by the discontinuity at the orange arrow?

A

Pathological fracture: closed, non-comminuted, oblique

115
Q

A 10-year-old male presents with pain and swelling in the right lower extremity after a sharp pain while running during football. He can no longer put weight on his leg. You note swelling and tenderness of the right ankle. The boy describes a throbbing pain during examination.

How many lesions are present? What are they?

A

Two:

  • Fracture
  • Osteolytic geographic lesion with sclerotic border

non-ossifying fibroma

116
Q

A 10-year-old male presents with pain and swelling in the right lower extremity after a sharp pain while running during football. He can no longer put weight on his leg. You note swelling and tenderness of the right ankle. The boy describes a throbbing pain during examination.

Does the cortex of the affect bone appear to be disrupted?

A

No, cortex is unaffected

non-ossifying fibroma

117
Q

This is a T1 weighted MRI of a 10-year-old male’s ankle. Note the intact periosteum.

Does this lesion have a short or long zone of transition?
Is it likely aggressive?

A

Short zone of transition
Non-aggressive

non-ossifying fibroma

118
Q

Non-ossifying fibroma occurs in individuals 8-19 years of age.
What is another term for this condition?

A

Fibrous cortical defect

119
Q

The blue arrow points to non-ossifying fibroma/fibrous cortical defect.

What is the most common treatment for these lesions?
What is the typical outcome?

A

Most common treatment is monitor (leave alone)
Typically spontaneously resolves (replaced by normal bone)

if symptomatic: curettage and bone graft

120
Q

In this case of non-ossifying fibroma, the lesion (blue arrow) occupies more than 15% of the bone’s diameter.
What does this increase the risk of?

A

Pathological fracture (orange arrow)

121
Q

Which bones are most affected by non-ossifying fibromas?

A

Metaphyseal regions of tibia/femur, cortically located

122
Q

What syndromes or other conditions may sometimes be associated with non-ossifying fibromas, particularly when they occur in multiples?

A

Multiple fibrous cortical defects:

  • Neurofibromatosis type I
  • Jaffe-Campanacci syndrome (cafe au lait spots)