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
Describe this pathology of enchondroma
well circumscribed
26
is this benign or malignant
borderline considered to benign, but locally aggressive well demarcated eroded through cortex but hasnt burst through articular surface haemorrhage looks malignant
27
Summarise giant cell tumours
**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
28
Mx of giant cell tumour
excise with limited margin
29
site predisposition for giant cell tumour
made of osteoclast giant cells tumour cells are the stromal cells site - end of long bone, most around knee
30
XR of giant cell tumour
well demarcated expansile lesion can burst through cortex into surrounding tissue
31
histology of giant cell tumour
sheets of osteoclast giant cell
32
what is the most common malignant bone tumour
mets - but rare below elbow and knee
33
what mets to bone in adult
breast prostate lung kidney thyroid
34
what mets to bone in children
neuroblastoma Wilm's tumour osteosarcoma Ewings Rhabdomyosarcoma
35
how do you determine histologically where the mets have come from
stain - eg stain for PSA (prostate) immunohistological markers can tell you where met from
36
3 types of malignant bone tumours
osteosarcoma *(bone forming)* chondrosarcoma *(cartilage forming)* Ewing's sarcoma/PNET (primitive peripheral neuroectodermal tumour) *(undifferentiated mesenchymal tumour)*
37
Site predeliction for osteosarcoma
if older - jaw most - lower femur/upper tibia end of long bones
38
summarise osteosarcoma
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
Px of osteosarcoma
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
classification of osteosarcoma
according to site - intramedullary, intracortical, surface differentiation (grade) multicentricity ie synchronous or metachronus primary or secondary
41
what is codmans triangle
sclerotic and lytic lesion gone into the soft tissue pushed the periosteium up -> forming triangle called Codman's triangle
42
histopath of osteosarcoma
malignant bone mesenchymal stroma malignant cartilage too many cells - osteosarcoma, looser myxoid area, and disordered bone
43
touch preparation for osteosarcoma
malignant cells produce **alkaline phosphtase** (bone cells use this to mineralise bone) stain for this in core biopsy
44
summarise chondrosarcoma
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
px of condrosarcoma
**better than osteosarcoma** depend on grade and size
46
classification of chondrosarcoma
according to site - intramedullary, juxtacortical histologically - conventional (myxoid or hyaline), clear cell (low grade), dedifferentiated, mesenchymal
47
where is this lesion
arm tumour coming off the ulner into the soft tissue
48
describe this
humerus white glistening tumour lifted the periostium some benign here but some malignant chondrosarcoma because white, not as haemorrhagic as osteosarcoma
49
site prediliction from chondrosarcoma
most in pelvis end of long bone
50
histology for chondrosarcoma
cartilaginous tumour malignant cell in matrix atypical chondrocytes (usually purple/blue)
51
Summarise Ewing's sarcoma
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
site prediliction for ewing's sacoma
pelvis diaphysis of long bone
53
histology of ewings sarcoma
small round blue cell tumour
54
what do you have to exclude and how when dx ewings sarcoma
immunostaining Ewings will be +ve for MIC2
55
cytology of Ewing's sarcoma
alk phos -ve stains for CD99 or MIC2
56
genetics of Ewing sarcoma
t(11;22)(q24;q12) maling EWSR1-FLI1 fusion protein can detect protein to make dx
57
definition of soft tissue sarcoma
mesenchymal proliferations which occur in extraskeletal, non-epithelial tissues of the body - excluding meninges and lymphoreticular system
58
summarise soft tissue tumours
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
aetiology of soft tissue tumours
unknown genetic chemic carcinogens physical - asbestos, foreign body virus immunodeficiency
60
type of soft tissue carcinoma
**myxoid** - eg liposarcoma **spindly type** - spindle cell sarcoma - tumour of nerve/muscle **pleomorphic** - dont know where come from **epithelioid** - resemble carcinoma
61
ddx of spindle cell tumour
62
ddx of myxoid tumours
63
ddx of pleomorphic tumour
64
histology of synovial sarcoma
epithelioid area spindle area do immuno
65
diagnostic technique for soft tissue tumour
66
soft tissue sarcoma - tumour specific chr translocations
67
Px factors for soft tissue sarcoma
68
staging of soft tissue sarcoma
69
vascular invasion pleomorphic tumour in blood vessels on way to lungs
70
macroscopic Ewing's tumour