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Flashcards in 7- Bone Tumors Deck (76)
1

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

Bone tumors

2

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

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

3

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

osteoid osteoma:

4

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

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

5

Osteosarcoma, Ewing’s sarcoma seen commonly in:

childhood, adolescence

6

Giant cell tumor seen in

 young adults

7

Chondrosarcoma seen in

 elderly

8

Pattern on radiology:

Sclerotic vs Ill-defined

Sclerotic = well defined margins, slow growing, usually benign

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

9

Review for location of bone tumors

Review location of bone tumors

10

Solid, ivory-like pattern is 
generally seen in 

malignant 
bone matrix-forming tumors

11

Rings and arcs are generally seen 
in 

chondroid matrix-forming 
tumors

12

OSTEOID OSTEOMA 

where

size

X-ray apperance

special about it

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

 

13

Osteoblastoma

Where

size

x-ray apperance

special about

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

14

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

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

benign tumor of long bones, usually more then 2 cm

 

15

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

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

16

What do we expect to see in histology of osteoid osteoma

All immaure osteoids

17

Malignant mesenchymal tumor in which cells 
produce osteoid or bone.

Osteosarcoma
 

18

Most common bone sarcoma

Osteosarcoma

• ~2000 new cases per year in US.

 

19

Age distribution of osteosarcoma

sex preference?

Peak incidence?

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

20

Location of osteosarcoma

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

21

Most common spread of osteosarcoma is:

22

Pathogenesis of Osteosarcoma:

3 genetic components

1 disease you see it in

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

23

Describe what to expect in Xray of osteosarcoma

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

24

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

Osteosarcoma

25

Tx of osteosarcoma

• TREATMENT
• Neo-adjuvant chemotherapy and surgical 
resection

26

Histology of osteosarcoma

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

27

Prognosis of osteosarcoma post chemo

65% survial for pts with non-metastatic disease

28

Prognosis of osteosarcoma with en-bloc and chemo follow up

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

29

Most common benign tumor of bone

Osteochondroma

30

Location of osteochondroma

metaphysis of long bone

31

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

Yes, increased to 40% 

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

32

What does a osteochondroma look like

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

33

Benign hyaline cartilage lesion

Located intramedullary


– Enchondroma 
 

34

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

– Periosteal chondroma 

35

Descibe the lesion and what it is

Lytic, lobulated, cortical thinning.

Endochondroma

36

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

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

Expected HE for endochondroma:

lobules of hyaline cartilage, minimal 
atypia.

 

38

Pt comes in with multilple chondromatosis: suspected genetic alteration:

Frequent point mutations in IDH1 or IDH2

39

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

Olliers disease

 

40

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

Maffucci's syndrome

41

 Malignant tumor in which neoplastic cells 
produce a purely cartilaginous matrix

 

Chondrosarcoma

 

42

Age range and location of chondrosarcomas

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

Most common and second most common bone sarcoma

Most common is osteosarcoma

chondrosarcoma second

44

Describe imaging and Dx

Location

Cortical invovlement

soft tissue invovlement

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

46

Histology of chondrosarcoma

Nuclei:

Any changes?

 

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

What two things correlate with behavior of chondrosarcoma

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

48

Grading (1-3) of chondrosarcoma based on

 degree of 
cellularity and atypia

49

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

80-90%

29% (pulmonary metastasis)

50

Variants of chondrosarcoma depend on:

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

51

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

 

Non-ossifying fibrosarcoma

52

Location of non-ossifying fibroma and age to get them

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

53

Descibe this and expected Dx

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

Non-ossifying fibroma

54

• Developmental arrest of bone

FIBROUS DYSPLASIA

55

 Monostotic fibrous dysplasia seen most in what age group and where
 

• Most common, seen in adolescents
• Ribs, mandible and femur

56

Ployostotic fibrous dysplasia most common in which age and what location


• Infancy/childhood
• Crippling deformities, craniofacial involvement 
common

57


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

McCune-Albright syndrome:

58

McCune Albright more common M, F

Other findings assocated with it

More females

sexual precocity, Cushings, acromegaly, 

 

59

CAuse of McCune albright

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

60

X ray findigs in Fibrous dysplasia

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

61

Histology of Fibrous Dysplasia

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

62

Tx for fibrous dysplasia

• TREATMENT:
• Conservative, except polyostotic form

63

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

Ewing Sarcoma

64

Location of Ewing Sarcoma

Diaphysis of long tubular bones, ribs and pelvis

65

Xray apperance of Ewings Sarcoma

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

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

EWing Sarcoma

67

Histologic findings with Ewing sarcoma


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

68

85% of EWS tumors involve this gene mutation

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

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

EWS translocation

70

Tx for EWS

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

71

Age group targeted in Giant Cell tumor

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

72

Location of Giant Cell Tumor

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

73

Histology of Giant Cell Tumor

Multinucleated cells

74

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

Metestatic bone tumors

75

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

70% go to;


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

 

76

Most bone lesions are not primary but come from:

80%- breast, lung, thyroid, prostate and kidney 
(BLT-KP)