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NYCC MSP > Osteosarcoma & Ewing Sarcoma (DRAFT) > Flashcards

Osteosarcoma & Ewing Sarcoma (DRAFT) Flashcards

1
Q

What are the 4 most common primary malignancies of bone from most to least common?

A

MOCE:
- multiple myeloma
- osteosarcoma
- chondosarcoma
- Ewing sarcoma

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2
Q

Which of the 4 most common primary malignancies of bone occur in older populations?

A

Multiple Myeloma & Chondrosarcoma
(MOCE)

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3
Q

Which of the 4 most common primary malignancies of bone occur in younger populations?

A

Osteosarcoma & Ewing sarcoma
(MOCE)

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4
Q

(Primary/Secondary) malignancies of bone account for 70% of all bone malignancy

A

Secondary

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5
Q

What population is primarily affected by osteosarcomas?

A

Bimodal:
- <20 years (75%; primary)
- Older adults with predisposing conditions (secondary)

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6
Q

What is the most common primary bone tumor in children?

A

Primary osteosarcoma

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7
Q

What conditions can predispose older adults to developing a secondary osteosarcoma?

A
  • Pagets
  • Hx of radiation therapy (20 yr latency period)
  • Bone infarct
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8
Q

What is the preferential location of osteosarcoma?

A
  • Metaphysical region of long bones -> extends into diaphysis
  • knee (50%)
  • secondary osteosarcoma common in flat bones
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9
Q

What is the etiology of osteosarcoma?

A

Mutations in tumor suppressor genes & oncogenes

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10
Q

Name 5 genes that could be mutated to cause osteosarcoma

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

What type of gene is RB?

A

Tumor suppressor gene

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12
Q

What is the function of RB?

A

Negative regulator of cell cycle (slows/stops)

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13
Q

What type of gene is TP53?

A

Tumor suppressor gene

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14
Q

What is the function of TP53?

A

Encodes P53

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15
Q

What is the function of P53?

A

Promote DNA repair and apoptosis of damaged cells

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16
Q

What is the function of the INK4a gene?

A

Encodes p16 and p14 (tumor suppressors)

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17
Q

What is the function of MDM2 and CDK4?

A

Inhibit p53 and RB function
(Allows damaged cells to live)

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18
Q

What genes are overexpressed in many osteosarcomas?

A

MDM2 and CDK4

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19
Q

What are the gross characteristics of an osteosarcoma?

A
  • progressively enlarging soft tissue mass beyond bone
  • mixed blastic (ossification) & lytic (cortex destruction) mass
  • irregular margins
  • may lift/penetrate periosteum (aggressive Rxn)
  • hemorrhagic marrow
  • invasive (may metastasize)
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20
Q

Where do osteosarcomas frequently metastasize to?

A

Lungs, brain, other bones

21
Q

What is the term for large soft tissue masses in the lung due to metastasis of an osteosarcoma?

A

Cannon ball mets

22
Q

How would a patient with an osteosarcoma present clinically?

A
  • bone pain & swelling around site of tumor
  • possible palpable tumor (eg. Popliteal fossa)
  • patho Fx
  • Abnormal labs
    (may be tired/winded if mets to lung)
23
Q

What lab findings would be seen in a patient with an osteosarcoma?

A
  • ^ESR
  • ^CRP
  • ^alkaline phosphatase
24
Q

What are the radiographic characteristics of an osteosarcoma?

A
  • patho Fx
  • long/wide ZoT
  • radiopaque mass and/or radiolucency
  • aggressive periosteal reaction
  • cortex disruption
25
Q

What radiographic characteristic of an osteosarcoma indicates soft tissue mass extending beyond bone?

A

Aggressive periosteal reaction (laminated, spiculated or Codman’s triangle)

26
Q

Which pattern of destruction is linked to a more aggressive type of osteosarcoma?
A) mixed
B) blastic
C) lytic

A

lytic
(more aggressive builds less bone)

27
Q

What general pattern of destruction may be seen in an osteosarcoma?

A

Can be blastic or lytic or both

28
Q

What is the most common primary malignancy of bone?

A

Multiple myeloma

29
Q

What is the most common primary malignancy of bone in kids?

A

osteosarcoma

30
Q

What is the second most common primary malignancy of bone?

A

Osteosarcoma

31
Q

What is the second most common primary malignancy of bone in elderly patients?

A

Chondrosarcoma

32
Q

What is the second most common primary malignancy of bone in kids?

A

Ewing sarcoma

33
Q

What is the most common malignancy of bone?

A

metastatic disease

34
Q

What genes are turned off or deleted in many osteosarcomas?

A
  • RB
  • TP53
  • INK4a
35
Q

Which primary malignancies of bones prefer to metastasize to other bones?

A

Ewing sarcoma
Osteosarcoma

36
Q

Most osteosarcomas are (blastic/lytic/mixed)

A

blastic

37
Q

An osteosarcoma presents on an x-ray as a densely blastic mass. Is this more or less aggressive than an osteosarcoma that appears purely lytic?

A

less

38
Q

If a bone tumor presents radiographically with a soft tissue mass extending beyond the bone, is it more likely to be primary or secondary?

A

primary
(mets to bone rarely have soft tissue mass beyond bone)

39
Q

What is the common treatment for an osteosarcoma?

A

amputation + chemo/radiation therapy

40
Q

What age group is primarily affected by Ewing sarcomas?

A

<20 years

41
Q

What is the etiology of Ewing sarcoma?

A
  • poorly understood
  • 90% show translocation between chromosomes 11 & 22
42
Q

What is an Ewing sarcoma comprised of histologically?

A

neuroectodermal cells:
- closely packed, small, round cells w/out obvious differentiation
- arranged in uniform sheets
- cells have little cytoplasm

43
Q

What is the preferential location of Ewing sarcoma?

A
  • arises in medullary cavity and grows outward
  • femur, other long bones & pelvis
  • mid-diaphysis of long bones
44
Q

What is the most common primary malignancy of bone to metastasize to other bones?

A

Ewing sarcoma

45
Q

How would a patient with Ewing sarcoma present clinically?

A
  • painful enlarging masses (deep boring bone pain)
  • tender, warm, swollen around site
  • may mimic infection (fever, ^ESR, leukocytosis)
  • unexpected weight loss
  • weakness
  • anemia
46
Q

What is the treatment for Ewing sarcoma?

A

chemo/radiation therapy & limb-sparing surgery

47
Q

What are the radiographic characteristics of Ewing sarcoma?

A
  • permeative/motheaten lytic destruction
  • long ZoT
  • cortical disruption
  • laminated/spiculated periosteal Rxn
  • may extend to soft tissues
    (can look like osteomyelitis –> send to ER)
48
Q

What may be on your differential diagnosis list for a child with signs of an aggressive pathology?

A
  • osteosarcoma
  • Ewing sarcoma
  • lymphoma
  • osteomyelitis

NYCC MSP (22 decks)

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