neoblastic bone disease Flashcards

1
Q

tumour in popliteal fossa- why would this need an above knee amputation

A

want curative intent - cant just do excision, because tumour wraps itself around blood vessels and nerves in that area

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2
Q

epidemiology of bone tumours

A

very rare
can be benign/malignant
much less common than lung ca
primary malignant bone tumours more common in young adults and children
commenst malignant bone tumour is metastatic

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3
Q

site predilecation of bone tumours

A

osteosarcoma - around knee is most common

different tumours have different site and age predilection

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4
Q

clinical presentation of bone tumour dx

A

pain
swelling
deformity
pathological fracture

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5
Q

hx of bone tumour

A

age
site
duration
hx of trauma - can be misleading
multiple lesions - more likely metastatic or involve marrow like lymphoma
associated disease - wouldnt usually dx osteosarcoma in old, but if prev radiotherapy there, or Pagets disease consider it

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6
Q

ix for bone tumour

A

XR
* site, size, margin
* solitary/multiple
* soft tissue extension - feature of malignancy
* associated disease/fracture

biopsy

if suspect - refer quickly to specialist cenytre

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7
Q

where is the lesion

A

femur metaphysis

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8
Q

describe this lesion

A

lytic - destroyed the cortex
extend to soft tissue

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9
Q

biopsy for bone tumour

A

needle biopsy under radiological guidance with Jamshidi needle
+- US of CT guidance

open biopsy for sclerotic or inaccessible lesions only (ie if very bony and cant get needle in)

imprint (cytology) preparation - get dx in hrs about whether have representative tissue and whether benign/malignant

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10
Q

describe this bone biopsy

A

some pre-existing host bone
some changes in marrow
it is malignant
- no normal marrow tissue, trabecular bone is irregular, cartilage is in marrow. So whatever is goung on iss infiltrating bone

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11
Q

benign conditions that mimic bone tumours

A

fibrous dysplasia
metaphyseal fibrois cortical defect/non-ossifying fibroma
reparative giant cell granuloma
ossifying fibroma
simple bone cyst

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12
Q

summarise fibrous dysplasia

A

same bones and age as osteosarcoma

  • > female
  • mono-ostotic > polyostotic
  • 1st 3yr of life
  • site - any bone, ribs, prox femur commonest
  • XR - soap bubble osteolysis

polyostotic disease associated with endocrine problems, and rough border cafe au lait spots (McCune Albright syndrome)

<1% malignant transformation

somatic mutation in guanine-nucleotide binding protein (G-protein) (GNAS mutation chr 20q13)

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13
Q

XR for fibrodysplasia

A

soap bubble osteolysis
>1 bone

whereas osteosarcoma more likely one bone (unless met)

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14
Q

histology of fibrodysplasia

A

marrow replaced by fibrous stroma
rounded and curved trabecular bone

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15
Q

Rx of fibrodysplasia

A

can excise with a very small margin
no chemo

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16
Q

sheperd’s crook deformity in fibrous dysplasia

A

when fibrodysplasia happens in femoral head

bone weak and cystic -> microfractures

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17
Q

benign bone tumours

A

cartilaginous differentiation
* osteochondroma
* enchondroma
* chondroblastoma

bone forming
* osteoid osteoma
* osteoblastoma
* osteoma

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18
Q

osteochondroma commonest sites

A

end of long bone
top of humerus
around knee

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19
Q

pathology of osteochondroma

A

mimic tubular bone
have cartilaginous surface overling cortical and trabecular bone

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20
Q

histology of osteochrondroma

A

cartilage on surface
bone underneath

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21
Q

common sites for enchondroma

A

most in hands, some in feet
much less in long bones

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22
Q

definition of enchondroma

A

proliferation of cartilige within bone

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23
Q

XR of enchondroma

A

here in finger with pathological fracture

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24
Q

Type of lesion in this enchondroma

A

popcorn calcification

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25
Q

Describe this pathology of enchondroma

A

well circumscribed

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26
Q

is this benign or malignant

A

borderline considered to benign, but locally aggressive
well demarcated
eroded through cortex but hasnt burst through articular surface
haemorrhage looks malignant

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27
Q

Summarise giant cell tumours

A

borderline malignancy
site - epiphysis with metaphyseal extension
age - 20-30yrs
>female
- XR - lytic
- histology - osteoclasts on a background of spindle/ovioid cells

locally aggressive, can recur, can met to lungs

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28
Q

Mx of giant cell tumour

A

excise with limited margin

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29
Q

site predisposition for giant cell tumour

A

made of osteoclast giant cells
tumour cells are the stromal cells

site - end of long bone, most around knee

30
Q

XR of giant cell tumour

A

well demarcated expansile lesion
can burst through cortex into surrounding tissue

31
Q

histology of giant cell tumour

A

sheets of osteoclast giant cell

32
Q

what is the most common malignant bone tumour

A

mets - but rare below elbow and knee

33
Q

what mets to bone in adult

A

breast
prostate
lung
kidney
thyroid

34
Q

what mets to bone in children

A

neuroblastoma
Wilm’s tumour
osteosarcoma
Ewings
Rhabdomyosarcoma

35
Q

how do you determine histologically where the mets have come from

A

stain - eg stain for PSA (prostate)
immunohistological markers can tell you where met from

36
Q

3 types of malignant bone tumours

A

osteosarcoma (bone forming)
chondrosarcoma (cartilage forming)
Ewing’s sarcoma/PNET (primitive peripheral neuroectodermal tumour) (undifferentiated mesenchymal tumour)

37
Q

Site predeliction for osteosarcoma

A

if older - jaw
most - lower femur/upper tibia
end of long bones

38
Q

summarise osteosarcoma

A

mainly adolescence

commonest primary bone sarcoma
most around knee

XR - metaphyseal, lytic, permeative, elevated periostium (Codman’s triangle)

histo- malignant mesenchymal cells +- bone and cartilage formation

Rx - chemo, salvage surgery

39
Q

Px of osteosarcoma

A

Px - poor.

depend on:
* how quickly detecte
* whetehr in soft tissu
* whether met
* if more cartilage than bone hard to treat because not vascular so cant get chemo there

40
Q

classification of osteosarcoma

A

according to site - intramedullary, intracortical, surface
differentiation (grade)
multicentricity ie synchronous or metachronus
primary or secondary

41
Q

what is codmans triangle

A

sclerotic and lytic lesion gone into the soft tissue
pushed the periosteium up
-> forming triangle called Codman’s triangle

42
Q

histopath of osteosarcoma

A

malignant bone
mesenchymal stroma
malignant cartilage

too many cells - osteosarcoma, looser myxoid area, and disordered bone

43
Q

touch preparation for osteosarcoma

A

malignant cells produce alkaline phosphtase (bone cells use this to mineralise bone)
stain for this in core biopsy

44
Q

summarise chondrosarcoma

A

malignant cartilage producing tumour
40yrs and older
site - pelvis, axial skeleton, prox femur and tibia

XR - lytic with fluffy calcification

histo - malignant chondrocytes +- chondroid matrix may dedifferentiate to high grade sarcoma

45
Q

px of condrosarcoma

A

better than osteosarcoma
depend on grade and size

46
Q

classification of chondrosarcoma

A

according to site - intramedullary, juxtacortical

histologically - conventional (myxoid or hyaline), clear cell (low grade), dedifferentiated, mesenchymal

47
Q

where is this lesion

A

arm
tumour coming off the ulner into the soft tissue

48
Q

describe this

A

humerus
white glistening tumour
lifted the periostium
some benign here but some malignant
chondrosarcoma because white, not as haemorrhagic as osteosarcoma

49
Q

site prediliction from chondrosarcoma

A

most in pelvis
end of long bone

50
Q

histology for chondrosarcoma

A

cartilaginous tumour
malignant cell in matrix

atypical chondrocytes (usually purple/blue)

51
Q

Summarise Ewing’s sarcoma

A

highly malignant small round cell tumour
usually < 20yrs

site - diaphysis/metaphysis of long bones, pelvis

XR - onion skinning of periosteum, lytic +- sclerosis

histo - sheets of small round cells

Px - 75%

associated with specific chr translocation 11:22 (EWS/Fli1) - dx molecularly

52
Q

site prediliction for ewing’s sacoma

A

pelvis
diaphysis of long bone

53
Q

histology of ewings sarcoma

A

small round blue cell tumour

54
Q

what do you have to exclude and how when dx ewings sarcoma

A

immunostaining
Ewings will be +ve for MIC2

55
Q

cytology of Ewing’s sarcoma

A

alk phos -ve
stains for CD99 or MIC2

56
Q

genetics of Ewing sarcoma

A

t(11;22)(q24;q12) maling EWSR1-FLI1 fusion protein

can detect protein to make dx

57
Q

definition of soft tissue sarcoma

A

mesenchymal proliferations which occur in extraskeletal, non-epithelial tissues of the body - excluding meninges and lymphoreticular system

58
Q

summarise soft tissue tumours

A

site - anywhere, large musches of extremities, chest wall, mediastinum, retroperitonium

any age - mainly older

men more

Ewings and clear cell are more common in afrocaribbean

59
Q

aetiology of soft tissue tumours

A

unknown
genetic
chemic carcinogens
physical - asbestos, foreign body
virus
immunodeficiency

60
Q

type of soft tissue carcinoma

A

myxoid - eg liposarcoma
spindly type - spindle cell sarcoma - tumour of nerve/muscle
pleomorphic - dont know where come from
epithelioid - resemble carcinoma

61
Q

ddx of spindle cell tumour

A
62
Q

ddx of myxoid tumours

A
63
Q

ddx of pleomorphic tumour

A
64
Q

histology of synovial sarcoma

A

epithelioid area
spindle area

do immuno

65
Q

diagnostic technique for soft tissue tumour

A
66
Q

soft tissue sarcoma - tumour specific chr translocations

A
67
Q

Px factors for soft tissue sarcoma

A
68
Q

staging of soft tissue sarcoma

A
69
Q
A

vascular invasion
pleomorphic tumour in blood vessels on way to lungs

70
Q

macroscopic Ewing’s tumour

A