Tumour Flashcards

0
Q

What lesion is improved with ibuprofen?

A

Osteoid osteoma

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1
Q

Describe Mirel’s criteria. What is it used for?

A
Score for prophylactic fixation of pathological fractures
Score: 8 or more indicated prophylactic fixation
- Site
1: upper limb
2: lower limb
3: peritrochanteric
- Lesion: 
1: blastic
2: mixed
3: lytic
- Size of lesion:
1: 2/3
- Pain:
1: mild
2: moderate
3: severe
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2
Q

What is the clinical vignette of a patient with Osteoid osteoma?

A

Young patient with pain totally relieved by NSAIDs.
Pain worse at night
May spontaneously resolve in 2-4 years

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3
Q

What levels are increased in Osteoid osteoma?

A

Cox 1 & 2

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4
Q

Islands of epithelial cells in a spindle-cell stroma +/- nuclear atypia with mitotic figures

A

Adamantinoma

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6
Q

Loose, storiform fibrous background containing spicules of woven bony trabeculae that are lined with layers of osteoblasts

A

Osteofibrous dysplasia

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7
Q

What is typical of an osteoid osteoma radiograph?

A

A nidus of bone

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8
Q

Slowly enlarging, painless soft tissue mass. what are you worried about?

A

Soft-tissue sarcoma

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9
Q

A mutation in which gene causes multiple hereditary exostosis?

A

EXT1 & EXT2

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10
Q

“Stuck on” appearance:

A

parosteal osteosarcoma

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11
Q

A genetic abnormality in the cyclic AMP signaling pathway:

A

Fibrous dysplasia

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12
Q

Life expectancy of bone mets, from Best to Worst

A

The Pit Bulls Keep Laughing:

Thyroid, Prostate, Breast, Kidney, Lung

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13
Q

What percentage of Paget’s disease transforms into osteosarcoma?

A

1%

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14
Q

What are the 5 most common primary sites of metastatic bone lesions?

A

Thyroid, prostate, breast, kidney, lung

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15
Q

Prostate bony mets: Lytic of blastic?

A

Blastic

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16
Q

Name 3 lytic mets:

A

Renal, lung, thyroid

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17
Q

What are the requisite steps of bony metastasis?

A
  • Tumor cell intravasation (entry into blood vessels)
  • Avoidance of immune surveillance
  • Target tissue localization,
  • Induction of angiogenesis
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18
Q

What percentage of enchondromas transform into chondrosarcoma?

A

<1%

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19
Q

What sarcoma’s to go the LNs?

A
RACES: (RACES to the LN)
Rhabdomyosarcoma
Angiosarcoma (vascular)
Clear cell sarcoma
Epithelioid sarcoma
Synovial  sarcoma
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20
Q

Is sending reamings a good option for biopsy? Why or why not?

A

No, because reamings could contaminate adjacent tissue (muscle, fat, additional compartments), risking successful limb salvage if bx comes back + for malignancy (b/c you’ve just spread it).
NO definitive treatment until biopsy is back.

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21
Q

What is the constellation of signs and symptoms of multiple myeloma?

A

CRAB

  • Calcinosis
  • Renal dysfunction (increased Cr)
  • Anemia
  • Bony (lytic) lesions
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22
Q

What are the tumor biopsy principles?

A
  • Longitudinal incision (allows for extension of incision during definitive management)
  • Do not expose neurovascular structures
  • Maintain meticulous hemostasis
  • Approach through affected compartment
  • Can biopsy adjacent soft tissue if its an extension of the bony tumor
  • Insert any drains in line with the wound
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23
Q

What tumors should be biopsied?

A

All

24
Q

What does post-operative radiation achieve?

A

Pain reduction
Slowing of progression
Reduction in tumor burden

25
Q

What laboratory test should be ordered in the setting of osteosarcoma?

A

ALP

- Can be increased 2-3x

26
Q

What is the risk of malignant transformation of multiple hereditary exostosis (MHE)? What does it transform to?

A

5-10% risk of transformation to chondrosarcoma

27
Q

What is Maffuci syndrome?

A

Variant of enchodroma:
Multiple enchondromatosis
Hemangioma/angioma
Lymphangioma
Visceral malignancy (brain - astrocytoma, GI)
High risk of transformation to chondrosarcoma

28
Q

What is the Enneking tumour staging system?

A
Benign:
1: Latent
2: Active
3: Aggressive
Malignant:
I:  Low grade, no mets:
- IA: intracompartmental
- IB: extracompartmental
II: High grade, no mets:
- IIA: intracompartmental
- IIB: extracompartmental
III: Metastatic
- IIIA: intracompartmental
- IIIB: extracompartmental
29
Q

What is the AJCC tumour staging system?

A

American Joint Committee on Cancer System

30
Q

What works better on osteosarcoma? Radiation or chemotherapy?

A

Chemotherapy

- Osteosarcoma is highly resistant to radiation therapy

31
Q

What are the absolute and relative contraindications for limb-salvage surgery?

A
  • Inability to obtain a wide surgical margin
  • Neurovascular involvement
  • Pathologic fracture
32
Q

What is the survival rate for non-metastatic and metastatic osteosarcoma?

A

10 year survival:
Non-metastatic: 60-80%
Metastatic: 20-30%

33
Q

What effect of chemo has been reported to improve survival?

A

> 90% tumor necrosis with neoadjuvant chemotherapy

34
Q

What are the types of osteosarcoma?

A
Intramedullary:
- Conventional: most common
- Telangeictatic
- Low grade
- Small-cell
Surface:
- Parosteal - low-grade
- Periosteal - intermediate grade
- High-grade - high grade
35
Q

Describe conventional osteosarcoma?

A

Intramedullary osteosarcoma
Most common variant (80%)
X-ray: Osteolytic and/or blastic bone lesion with cortical destruction
Histo:
- Malignant mesenchymal cells
- Spindle to polyhedral shape
- Pleomorphic nuclei and occasional mitotic figures
- Evidence to bone or osteoid matrix
Further divided into:
- Osteoblastic, chondroblastic, fibroblastic, depending on matrix

36
Q

Describe Telangiectatic osteosarcoma?

A

Intramedullary osteosarcoma
Eccentric and osteolytic lesion expanding or disrupting the metaphyseal surface
- Lesion may resemble an ABC as it contains multiple blood-filled sinusoids
- High signal intensity on T2- weighted MRI
Histo:
- Multiple dilated hemorrhagic cavities, small amounts of osteoid and high grade osteosarcoma cells found within the septa

37
Q

Describe low-grade intramedullary osteosarcoma

A

Intramedullary osteosarcoma
- Unaggressive appearing lytic, mixed lytic/blastic or blastic process
- Appears fibro-osseous
- Varying amouts of septal ossification and sclerosis
Histo:
- Well-differentiated cells dispersed with women microtrabeculae of bone and fibrous stroma
- Small amounts of osteoid, atypia and mitotic figures

38
Q

Describe small-cell osteosarcoma

A

Intramedullary. Rare
- Lytic areas with variable amounts of sclerosis
- MRI: large circumferential soft-tissue mass
Histo:
- Small, round, malignant cells within an osteoid matrix

39
Q

Describe parosteal osteosarcoma:

A

Surface osteosarcoma
- Densely ossified and lobulated mass sparing IM cavity
- Often posterior on femur
Histo:
- low-grade, well differentiated fibrous stroma with osseous components
- “Pulled steel wool” pattern with presence of bony trabeculae with a parallel orientation

40
Q

Describe periosteal osteosarcoma:

A

Surface juxtacortical tuour
- More aggressive than parosteal
- Lytic, radiolucent mass sparing IM cavity
- Sunburst appearance common
Histo:
- Intermediate grade tumour containing cartilagenous matrix with areas of calcification
- Small amounts of osteoid

41
Q

Describe high grade osteosarcoma:

A
  • Surface osteosarcoma with partial mineralization and tumour extension in surrounding ST
    Histo:
  • High grade spindle cells with atypia and varying amounts of osteoid
42
Q

What is the common origin of the Ewing Sarcoma Family of Tumours?

A

Mesenchymal stem cells capable of differentiation into multiple lineages

43
Q

Describe Ewing’s Sarcoma

A

Ill-defined, mottled or focally moth-eaten destructive intramedullary lesion. Laminated or onionskin peristeal reaction. May have sunburst or codman’s triangle

44
Q

What are the differences between Osteosarcoma & Ewing’s sarcoma?

A

Osteosarcoma: Metaphysis
ES: diaphysis/metadiaphyseal
Osteosarcoma: Codman’s triangle/sunburst more common
Both 2nd decade of life

45
Q

What is the common genetic abnormality in Ewing’s?

A

EWS-ETS (FLI1) fusion gene

11;22 translocation

46
Q

Describe the histopathology of de-differentiated chondrosarcoma:

A

Often not of the same type as the primary chondrsarcoma/osteochondroma (ie it’s something else like osteosarcoma)

47
Q

What part of the osteochondroma becomes malignant in secondary chondrosarcoma?

A

The cartilagenous cap

48
Q

dDx for “Bubbly” or “Soap bubble” appearance on x-ray

A

aneurysmal bone cyst (ABC)
non-ossifying fibrooma (NOF)
unicameral bone cyst (UBC)

49
Q

“metaphyseal eccentric “bubbly” lytic lesion surrounded by sclerotic rim”

A

Non-ossifying fibroma

50
Q

What is MHE?

A

Multiple hereditary exostosis. Multiple osteochondromas

Maliganancy risk: 5-10% with MHE (vs. 1% with solitary osteochondroma)

51
Q

EXT gene

A

multiple hereditary exostosis

52
Q

What is the risk of malignancy for solitary osteochondroma & MHE

A

Risk of transformation into chondrosarc:
1% for solitary
5-10% for MHE

53
Q

Ollier’s & Maffuci:

A

Multiple enchondromas:
Ollier’s: multiple enchondromatosis. 30% malignancy
Maffuci: 100% malignancy from enchondromatosis & angioMa and visceral malignancies (GIs)

54
Q

What is the most malignant form of chondrosarcoma?

A

Dedifferentiated chondrosarcoma

55
Q

What is the 10 year survival rate of dedifferentiated chondrosarcoma?

A

10% at 10 years - it is the most malignant form of chondrosarcoma

56
Q

Risk factors for fracture in bone mets (not mirel’s)

A

Resistant tumour
Widespread disease
Visceral involvement
End stage disease