7- Bone Tumors Flashcards

1
Q

Relatively rare group of tumors in comparison with carcinomas and hematopoietic tumors • Malignant tumors make up ~0.2% of all types of cancer

A

Bone tumors

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2
Q

What are some clinical presentations we would expect to see in bone tumors?

A

Usually nonspecific, may present: • Pain (any tumor;) • Mass (parosteal OS: painless, hard growing mass in popliteal fossa) • Pathologic fracture • Asymptomatic

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3
Q

tumors causes severe pain, worse at night, relieved by aspirin

A

osteoid osteoma:

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4
Q

What do we need to consider when Dx for bone tumors?

A

Age • Sex • Skeletal localization • Specific bone • Specific area of bone 1. Medullary cavity, cortex, juxtacortical 2. Epiphysis, metaphysis, diaphysis • Radiographic appearance

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5
Q

Osteosarcoma, Ewing’s sarcoma seen commonly in:

A

childhood, adolescence

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6
Q

Giant cell tumor seen in

A

 young adults

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7
Q

Chondrosarcoma seen in

A

 elderly

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8
Q

Pattern on radiology:

Sclerotic vs Ill-defined

A

Sclerotic = well defined margins, slow growing, usually benign

Ill-defined = fuzzy border, fast growing and usually metestatic

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9
Q

Review for location of bone tumors

A

Review location of bone tumors

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10
Q

Solid, ivory-like pattern is
generally seen in

A

malignant
bone matrix-forming tumors

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11
Q

Rings and arcs are generally seen
in

A

chondroid matrix-forming
tumors

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12
Q

OSTEOID OSTEOMA

where

size

X-ray apperance

special about it

A

•Long bones, femur &
tibia
•< 2 cm
•Night pain
•Responds to aspirin
•Radiolucent lesion within
sclerotic cortex

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13
Q

Osteoblastoma

Where

size

x-ray apperance

special about

A

Vertebrae or long bone
metaphysis
•> 2 cm
•Painful
•Not responsive to aspirin
•Expansile radio-lucency
with mottling

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14
Q

Pt come in with really bad night pain in her leg, the only thing that helps is taking aspirin. What do you expect to see on xray

A

indicitave of osteoid osteoma; will see radiolucent lesion within the Sclerotic cortex

benign tumor of long bones, usually more then 2 cm

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15
Q

Mottled appearance larger then 2 cm. Painful and aspirin doesn’t help

A

Osteoblastoma; seen in long bone metaphysis and more then 2 cm

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16
Q

What do we expect to see in histology of osteoid osteoma

A

All immaure osteoids

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17
Q

Malignant mesenchymal tumor in which cells
produce osteoid or bone.

A

Osteosarcoma

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18
Q

Most common bone sarcoma

A

Osteosarcoma

• ~2000 new cases per year in US.

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19
Q

Age distribution of osteosarcoma

sex preference?

Peak incidence?

A
  • Bimodal age distribution, M>F
  • Mean age: 15 (60% 10-20)
  • 2nd peak: 55-80
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20
Q

Location of osteosarcoma

A

Metaphysis of long bones
• Femur, tibia, humerus (56%); flat
bones, spine (older patients)
• May be polyostotic (not common)
• Hematogenous spread to lungs is
common

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21
Q

Most common spread of osteosarcoma is:

A

Hematogenous spread to lungs is
common

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22
Q

Pathogenesis of Osteosarcoma:

3 genetic components

1 disease you see it in

A

Inherited mutant allele of RB gene
• Hereditary RB: marked increase (1000X) in OS
• Mutation of p53 suppressor gene
• Li-Fraumeni: bone and soft tissue sarcomas, early
onset breast cancer, brain tumors, leukemia
• Overexpression of MDM2 (5-10%); INK4 and
p16
• Sites of bone growth/disease (i.e. Paget dz.)
• Prior irradiation

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23
Q

Describe what to expect in Xray of osteosarcoma

A
  • Poorly delineated
  • Bone destruction
  • Cortical disruption
  • Bone matrix
  • Soft tissue extension
  • Codman’s triangle
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24
Q

Infiltrative tumor… extends to soft tissues. Has malignant cells producing osteoid

A

Osteosarcoma

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25
Q

Tx of osteosarcoma

A

• TREATMENT
• Neo-adjuvant chemotherapy and surgical
resection

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26
Q

Histology of osteosarcoma

A

Bone formation and in higher power you see nuclei and convoluted and variable size, see tri polar mitosis

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27
Q

Prognosis of osteosarcoma post chemo

A

65% survial for pts with non-metastatic disease

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28
Q

Prognosis of osteosarcoma with en-bloc and chemo follow up

A

En-bloc resection following chemotherapy: >90%
necrosis  near 90% survival

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29
Q

Most common benign tumor of bone

A

Osteochondroma

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30
Q

Location of osteochondroma

A

metaphysis of long bone

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31
Q

PT has multiple osteochondromas. Do we worry about maligant transformation? Whats the linked etiology

A

Yes, increased to 40%

Autosomal dominant most commly secondary to EXT-1 or a 8q24 muation

32
Q

What does a osteochondroma look like

A

The cartilage, bone and marrow all grow out together in a little outpouching

33
Q

Benign hyaline cartilage lesion

Located intramedullary

A

– Enchondroma

34
Q

juxtacortical chondroma is located on cortical surface under periosteum. Called:

A

– Periosteal chondroma

35
Q

Descibe the lesion and what it is

A

Lytic, lobulated, cortical thinning.

Endochondroma

36
Q

Pt comes in to get a Bone density scan… you notice some irregularites in her hand so you take an xray and see this:

Recommended tx

A

Endochondroma, present in hand, feet, appendicular skeleton… IG incidental finding

TX

none, unless lesion shows changes:
1. Symptomatic (onset of acute pain is felt to be
circumstantial evidence that lesion is malignant)
2. Evidence of recent growth after skeletal maturity

37
Q

Expected HE for endochondroma:

A

lobules of hyaline cartilage, minimal
atypia.

38
Q

Pt comes in with multilple chondromatosis: suspected genetic alteration:

A

Frequent point mutations in IDH1 or IDH2

39
Q

– Multiple enchondromata
– Tend to have regional distribution
– ± severe skeletal malformation

A

Olliers disease

40
Q

– Multiple enchondromata + angiomata
– Severe skeletal malformation
– Higher incidence of malignant transformation

A

Maffucci’s syndrome

41
Q

Malignant tumor in which neoplastic cells
produce a purely cartilaginous matrix

A

Chondrosarcoma

42
Q

Age range and location of chondrosarcomas

A

Wide range of ages, mainly older adults
• Mostly above 40-50 years
• Peak during 6th- 7th decades
• Central skeleton: pelvis and ribs (45%); humerus,
femur (metaphysis, diaphysis)

43
Q

Most common and second most common bone sarcoma

A

Most common is osteosarcoma

chondrosarcoma second

44
Q

Describe imaging and Dx

Location

Cortical invovlement

soft tissue invovlement

A

Chondrosarcoma = popcorn like

Medullary location
• Frequently present calcifications, which tend
to be lost in grade 3 tumors
• Cortical erosion or destruction
• Occasional soft tissue extension

45
Q
A
46
Q

Histology of chondrosarcoma

Nuclei:

Any changes?

A

Generally more cellular and nuclei more
pleomorphic than in enchondromas.
• Binucleation is frequent, but does not suffice
for malignant diagnosis.
• Myxoid change of chondroid matrix.

47
Q

What two things correlate with behavior of chondrosarcoma

A

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

48
Q

Grading (1-3) of chondrosarcoma based on

A

degree of
cellularity and atypia

49
Q

5 year survival outcomes for chondrosarcoma
• Grades 1,2:
• Grade 3:

A

80-90%

29% (pulmonary metastasis)

50
Q

Variants of chondrosarcoma depend on:

A

de-differntiaton, myxoid, clear cells, mesenchymal, juxtacortical

51
Q

Common developmental cortical defect
• Most common space-occupying lesion of bone: 1
of every 4 individuals
• Multifocal in 25% of cases

A

Non-ossifying fibrosarcoma

52
Q

Location of non-ossifying fibroma and age to get them

A

Tibia, femur (metaphysis); 1st – 3rd decades.
• Eccentric, lytic, peripheral sclerosis
• Incidental finding or pathologic fracture

53
Q

Descibe this and expected Dx

A

lots of spindle cells, no ryhyme or reason. Looks like Starry night

Non-ossifying fibroma

54
Q

• Developmental arrest of bone

A

FIBROUS DYSPLASIA

55
Q

Monostotic fibrous dysplasia seen most in what age group and where

A
  • Most common, seen in adolescents
  • Ribs, mandible and femur
56
Q

Ployostotic fibrous dysplasia most common in which age and what location

A

• Infancy/childhood
• Crippling deformities, craniofacial involvement
common

57
Q

• Polyostotic FD with endocrinopathies and café-
au-lait spots

A

McCune-Albright syndrome:

58
Q

McCune Albright more common M, F

Other findings assocated with it

A

More females

sexual precocity, Cushings, acromegaly,

59
Q

CAuse of McCune albright

A

Germline mutation of GNAS that causes excess cAMP leading to endocrine gland hyperfunction

60
Q

X ray findigs in Fibrous dysplasia

A
  • Expansile
  • Circumscribed
  • Thinned cortex
  • “Ground glass”
  • May be multiple
61
Q

Histology of Fibrous Dysplasia

A

PATHOLOGY:
• Haphazard, curvilinear, randomly oriented
woven bone trabeculae (“Chinese
characters”), surrounded by fibroblastic
stroma
• No significant osteoblastic rimming

62
Q

Tx for fibrous dysplasia

A
  • TREATMENT:
  • Conservative, except polyostotic form
63
Q

Second most common malignant bone tumor
in childhood
• 16% of primary bone malignancies
• Adolescents, young adults; M>F
• Present as painful, often enlarging mass

A

Ewing Sarcoma

64
Q

Location of Ewing Sarcoma

A

Diaphysis of long tubular bones, ribs and pelvis

65
Q

Xray apperance of Ewings Sarcoma

A

X-RAY:
• Destructive moth-eaten, permeative
medullary lesion with large soft
tissue mass
• “Onion-skin” pattern of periosteal
reaction in response to rapid growth

66
Q

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

A

EWing Sarcoma

67
Q

Histologic findings with Ewing sarcoma

A

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

68
Q

85% of EWS tumors involve this gene mutation

A

EWS involved in >95% of ES/PNET
• t(11;22) present in 85% of the tumors
• EWS on 22q fused with FLI-1 transcription factor on 11q

69
Q

on 22q fused with FLI-1 transcription factor on 11q

A

EWS translocation

70
Q

Tx for EWS

A

Chemotherapy and surgery
• Radiation therapy may be added
• Stage I- 5 year survival 70% with chemo/ RT

71
Q

Age group targeted in Giant Cell tumor

A
Young adults (20-40 years), older 
adolescents (skeletally matured); F\>M
72
Q

Location of Giant Cell Tumor

A

• Epiphyseal location
• Knee, proximal humerus, radius
• Most are benign, locally aggressive
• May destroy cortex of bone and extend
into soft tissue

73
Q

Histology of Giant Cell Tumor

A

Multinucleated cells

74
Q

Most common malignant bone tumor, especially
in adults (20X more frequent than primary bone
tumors)
• Mostly multiple

A

Metestatic bone tumors

75
Q

Solitary lesions may mimic a primary bone tumor
and precede discovery of its source

70% go to;

A

– 70% go to axial skeleton (skull, ribs, vertebral column, sacrum)
– Mostly lytic
– May be blastic (bone-forming): breast, prostate

76
Q

Most bone lesions are not primary but come from:

A

80%- breast, lung, thyroid, prostate and kidney

(BLT-KP)