Osteosarcoma & Ewing Sarcoma Flashcards

(49 cards)

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

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

A

Multiple Myeloma & Chondrosarcoma
(MOCE)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

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

A

Osteosarcoma & Ewing sarcoma
(MOCE)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

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

A

Secondary

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What population is primarily affected by osteosarcomas?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the most common primary bone tumor in children?

A

Primary osteosarcoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the preferential location of osteosarcoma?

A
  • Metaphyseal region of long bones -> extends into diaphysis
  • knee (50%)
  • secondary osteosarcoma common in flat bones
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the etiology of osteosarcoma?

A

Mutations in tumor suppressor genes & oncogenes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Name 5 genes that could be mutated to cause osteosarcoma

A
  • RB
  • TP53
  • INK4a
  • MDM2
  • CDK4
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What type of gene is RB?

A

Tumor suppressor gene

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the function of RB?

A

Negative regulator of cell cycle (slows/stops)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What type of gene is TP53?

A

Tumor suppressor gene

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the function of TP53?

A

Encodes P53

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the function of P53?

A

Promote DNA repair and apoptosis of damaged cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the function of the INK4a gene?

A

Encodes p16 and p14 (tumor suppressors)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the function of MDM2 and CDK4?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What genes are overexpressed in many osteosarcomas?

A

MDM2 and CDK4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
  • cortical destruction
25
What radiographic characteristic of an osteosarcoma indicates soft tissue mass extending beyond bone?
Aggressive periosteal reaction (laminated, spiculated or Codman's triangle)
26
Which pattern of destruction is linked to a more aggressive type of osteosarcoma? A) mixed B) blastic C) lytic
lytic (more aggressive builds less bone)
27
What general pattern of destruction may be seen in an osteosarcoma?
Can be blastic or lytic or both
28
What is the most common primary malignancy of bone?
Multiple myeloma
29
What is the most common primary malignancy of bone in kids?
osteosarcoma
30
What is the second most common primary malignancy of bone?
Osteosarcoma
31
What is the second most common primary malignancy of bone in elderly patients?
Chondrosarcoma
32
What is the second most common primary malignancy of bone in kids?
Ewing sarcoma
33
What is the most common malignancy of bone?
metastatic disease
34
What genes are **turned off** or deleted in many osteosarcomas?
- RB - TP53 - INK4a
35
Which primary malignancies of bones prefer to metastasize to other bones?
1 MC: Ewing sarcoma 2 MC: Osteosarcoma
36
Most osteosarcomas are (blastic/lytic/mixed)
blastic
37
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?
less
38
If a bone tumor presents radiographically with a soft tissue mass extending beyond the bone, is it more likely to be primary or secondary?
primary (mets to bone rarely have soft tissue mass beyond bone)
39
What is the common treatment for an osteosarcoma?
- amputation + chemo/radiation therapy - limb salvage surgery
40
What age group is primarily affected by Ewing sarcomas?
<20 years
41
What is the etiology of Ewing sarcoma?
- poorly understood - 90% show translocation between chromosomes 11 & 22
42
What is an Ewing sarcoma comprised of histologically?
neuroectodermal cells: - closely packed, small, round cells w/out obvious differentiation - arranged in uniform sheets - cells have little cytoplasm, w/ huge nuclei
43
What is the preferential location of Ewing sarcoma?
- arises in medullary cavity and grows outward - femur, other long bones & pelvis - mid-diaphysis of long bones
44
What is the most common primary malignancy of bone to metastasize to other bones?
Ewing sarcoma
45
How would a patient with Ewing sarcoma present clinically?
- painful enlarging masses (deep boring bone pain) - tender, warm, swollen around site - may mimic infection (fever, ^ESR, leukocytosis) - unexpected weight loss - weakness - anemia
46
What is the treatment for Ewing sarcoma?
chemo/radiation therapy & limb-sparing surgery
47
What are the radiographic characteristics of Ewing sarcoma?
- permeative/motheaten lytic destruction - long ZoT - cortical disruption - laminated/spiculated/Codman's periosteal Rxn - may extend to soft tissues (can look like osteomyelitis --> send to ER)
48
What bone pathologies would you include in your differential diagnosis if you see aggressive bone destruction radiographically in a patient under 25 years old?
OLEO: - osteosarcoma (aggressive kid tumor) - Ewing sarcoma (aggressive kid tumor) - lymphoma (any age) - osteomyelitis (appears same; more common in kids)
49
What benign and malignant tumors of bone may be on your differential diagnosis for a lesion in the diaphysis?
- enchondroma - fibrous dysplasia - ewing sarcoma - chondrosarcoma