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MSS > Bone Tumors > Flashcards

Bone Tumors Flashcards

1
Q

Are bone tumors common?

A

Not in comparison to carcinomas and hematopoietic tumors

Malignant bone tumors comprise (0.2%) of all types of cancer

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2
Q

How do bone tumors commonly present?

A

Usually non-specific:

Pain

Mass

Pathologic fracture

Asymptomatic

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3
Q

What are some diagnostic factors to think about with bone tumors?

A

Age

Sex

Skeletal localization: specific bone, specific area of bone

Radiographic appearance

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4
Q

What age do Osteosarcoma, Ewing’s sarcoma generally affect?

A

Childhood, adolescence

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5
Q

What ages do giant cell tumors generally affect?

A

Young adults

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6
Q

What ages do Chondrosarcomas generally affect?

A

Elderly

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7
Q

What is a slcerotic margin an indication of?

A

Benign, slowly-growing neoplasm

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8
Q

What is an ill-defined margin indicative of?

A

Malignant, rapidly-growing neoplasm

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9
Q

What does it mean to see solid, ivory-like patterns on radiographs of bone?

A

Generally seen in malignant bone matrix-forming tumors

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10
Q

What does it mean to see rings and arcs on radiographs of bone?

A

Generally seen in chondroid matrix-forming tumors

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11
Q

What is the most common primary bone tumor?

A

Osteochondroma

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12
Q

Benign bone-forming tumors

A

Osteoid Osteoma & Osteoblastoma

Histologically very similar

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13
Q

Located in long bones, femur & tibia

<2cm

Night pain

Responds to aspirin

Radiolucent lesion within sclerotic cortex

A

Osteoid Osteoma

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14
Q

Located in vertebrae or long bone metaphysis

>2cm

Painful

Not responsive to aspirin

Expansile radio-lucency with mottling

A

Osteoblastoma

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15
Q

Malignant mesenchymal tumor in which cells produce osteoid or bone

35% of bone sarcomas

A

Osteosarcoma

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16
Q

What population does osteosarcoma generally affect?

A

M>F

Mean age: 15 (60% 10-20)

2nd peak: 55-80

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17
Q

Where does osteosarcoma usually reside?

A

Metaphysis of long bones (femur, tibia, humerous; flat bones, spine - older pts)

May be polyostotic – multiple bone sites (not common)

Hematogenous spread to lungs is common

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18
Q

Where does osteosarcoma commonly spread hematogenously?

A

To the lungs

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19
Q

What are some characteristics that may predispose someone to osteosarcoma?

A
  • Inherited mutant allele of RB gene: marked increased (1000X) in OS
  • Mutation of p53 suppressor gene: Li-Fraumeni
  • Overexpression of MDM2 (5-10%); INK4 and p16
  • Sites of bone growth/disease (Paget’s)
  • Prior irradiation
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20
Q

What are some radiographic findings that are commonly indicative of osteosarcoma?

A
  • Poorly delineated
  • Bone destruction
  • Cortical disruption
  • Bone matrix
  • Soft tissue extention
  • Codman’s triange: radiographic sign, rapidly growing mass with periosteum trying to grow around it
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21
Q

What is found pathologically with osteosarcomas?

A

Infiltrative tumor, extending into soft tissue

Malignant cells producing osteoid

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22
Q

What is the treatment for osteosarcoma?

A

Neo-adjuvant chemotherapy and surgical resection (amputation)

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23
Q

What was the prognosis for Osteosarcoma prior to 1970? What is it now, after the advent of chemotherapy?

A

Prior to 1970: 5 year survival 20%; most relapsed in lung within 6 months after primary amputation; mets to lung pleura, other bones, CNS

Post chemotherapy: 60-65% 3-5 year survival for patients with non-metastatic disease; En-bloc resection following chemotherapy: >90% necrosis – near 90% survival

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24
Q

Most common benign tumor of bone

A

Osteochondroma

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25
Q

Where are osteochondromas commonly located in bones?

A

Metaphysis of long bones

26
Q

Is transformation of osteochondroma to malignancy common?

A

No – it’s rare (<1%) but increased risk in hereditary multiple exostoses –> Autosomal dominant, commonly secondary to mutations in EXT-1 (8q24)

27
Q

Benign hyaline cartilage lesion (2 kinds)

A

Enchondroma = intramedullary chondroma

Periosteal chondroma = juxtacortical chondroma (located on the cortical surface under the periosteum)

28
Q

How do enchondromas usually present?

A

Usually asymptomatic, incidental finding.

Appendicular skeleton; small bones of hands and feet.

29
Q

How do enchondromas appear on x-ray? In microanalysis?

A

X-ray: lytic, lobulated, cortical thinning

Micro: lobules of hyaline cartilage, minimal atypia

30
Q

How are enchondromas treated?

A

Nothing! Unless lesion shows changes:

  1. become symptomatic
  2. evidence of recent growth after skeletal maturity
31
Q

What can lead to Multiple Chondromatosis?

A

Frequent point mutations in IDH1 or IDH2

Ollier’s disease: multiple enchondromata, tend to have regional distribution, could have severe skeletal malformation

Maffucci’s syndrom: multiple enchondromata + angiomata, severe skeletal malformation, higher incidence of malignant transformation

32
Q

Malignant tumor in which neoplastic cells produce a purely carilaginous matrix

2nd most common bone sarcoma (~26%)

A

Chondrosarcoma

33
Q

What population do Chondrosarcomas commonly affect?

A

Wide range of ages, mainly older adults (mostly 40-50 years)

Peak at 6-7th decades

34
Q

Where do chondrosarcomas generally occur?

A

Pelvis and ribs (45%); humerus, femur (metaphysis, diaphysis)

35
Q

What do chondrosarcomas look like on radiographs?

A

Medullary location

Frequently present calcifications, which tend to be lost in grade 3 tumors

Cortical erosion or destruction

Occasional soft tissue extension

“Popcorn-like appearance”

36
Q

How do chondrosarcomas appear pathologically?

A

More cellular and nuclei more pleomorphic than enchondromas

Binucleation is frequent but does not suffice for malignant diagnosis

Myxoid change of chondroid matrix

37
Q

How is grading dictated in chondrosarcomas?

A

Size (< or > 10 cm) and grade correlate with behavior

Grading (1-3) based on degree of cellularity and atypia

38
Q

What is the prognosis for chondrosarcoma?

A

Grades 1,2: 80-90%

Grade 3: 29% (pulmonary metastasis)

Variants: de-differentiated, myxoid, clear cell, mesenchymal, juxtacortical

39
Q

Common developmental cortical defect

Most common space-occupying lesion of bone: 1 of every 4 individuals

A

Non-ossifying fibroma

40
Q

What are some common locations for non-ossifying fibromas?

A

Tibia, femur (metaphysis)

41
Q

When do non-ossifying fibromas occur in life?

A

1st-3rd decade

42
Q

What is the histological appearance called of non-ossifying fibromas?

A

“Starry-sky”

43
Q

Developmental arrest of bone

Can be monostotic (most common, in adolescents, ribs, mandible and femur)

Or Polyostotic (in infancy/childhood, crippling deformities, cranio facial involvement common)

A

Fibrous dysplasia

44
Q

What is McCune-Albright syndrome? More common in F or M?

A

Polyostotic fibrous dysplasia with endocrinopathies and cafe-au-lait spots

F>M

Sexual precocity, acromegaly, Cushing syndrome

45
Q

Genetically, how does McCune-Albright occur?

A

Activating germline mutations of GNAS (GTP-binding protein) results in excess cAMP leading to endocrine gland hyperfunction

46
Q

How does fibrous dysplasia appear on x-ray?

A
  • Expansile
  • Circumscribed
  • Thinned cortex
  • “ground glass”
  • May be multiple
47
Q

What does the pathology of fibrous dysplasia look like?

A

Haphazard, curvilinear, randomly oriented woven bone trabeculae (“chinese characters”), surrounded by fibroblastic stroma

No significant osteoblastic rimming

48
Q

How do you treat fibrous dysplasia?

A

Conservative, except polyostotic form

49
Q

Second most common malignant bone tumor in childhood

Adolescents, young adults; M>F

Present as painful, often enlarging mass

A

Ewing Sarcoma/ PNET

50
Q

What part of bone does Ewing Sarcoma affect?

A

Diaphysis of long tumular bones, ribs, pelvis

51
Q

How does Ewing Sarcoma appear on X-ray?

A

Destructive moth-eaten, permeative medullary lesion with large soft tissue mass

“Onion-skin” pattern of periosteal reaction in response to rapid growth

52
Q

How does Ewing Sarcoma present pathologically?

A
  • Sheets of primitive small round blue cells with neural phenotype
  • Membranous CD99
  • Contain abundant glycogen
  • Hemorrhage and necrosis common
53
Q

What genetic translocation is associated with Ewing Sarcoma?

A

t(11;22): EWS on 22q fused wtih FLI-1 transcription factor on 11

Present in 85% of tumors

54
Q

What is the treatment for Ewing Sarcoma?

A

Chemotherapy and surgery

Radiation therapy may be added

Stage 1: 5 year survival 70% with chemo/RT

55
Q

What population do giant cell tumors of bone commonly affect?

A

Young adults (20-40 years)

Older adolescents (skeletally matured)

F>M

56
Q

Where do giant cell tumors generally presents?

A

Epiphyseal location

Knee, proximal humerus, radius

57
Q

Are giant cell tumors of bone generally malignant or benign?

A

Most are benign, locally aggressive

May destroy cortex of bone and extend into soft tissue

58
Q

Are primary or metastatic bone tumors more likely?

A

Malignant bone tumors are 20x more frequent than primary bone tumors – especially in adults

59
Q

What are the main types of cancers that are known to cause metastatic bone tumors?

A

BLT-KP

Breast, Lung, Thyroid, Kidney, Prostate

60
Q
A

MSS (24 decks)

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