Bone and Soft Tissue Tumours Flashcards

1
Q

What is a sarcoma? How does it spread?

A

Malignant tumours arising from connective or other non-epithelial tissues
Spread along fascial planes
Haemtogenous spread to lungs
Rarely spread to regional lymph nodes - rhabdomyosarcomas, epithelioid sarcomas and synovial sarcomas

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2
Q

What is more common, benign or malignant tumours of the skeleton?

A

Benign tumours of skeleton are common, malignant tumours of skeleton are rare

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3
Q

How many malignant bone tumours are there in comparison to lung tumours in males?

A

1 malignant bone tumour : 90 lung tumours

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4
Q

How many malignant bone tumours are there in comparison to breast tumours in females?

A

1 malignant bone tumour : 50 breast tumours

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5
Q

What is a bone tumour in a patient > 50 likely to be?

A

Metastatic

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6
Q

Give examples of bone-forming tumours

A

Benign

  • osteoid osteoma
  • osteoblastoma

Malignant
- osteosarcoma

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7
Q

Give examples of cartilage-forming tumours

A

Benign

  • endochondroma
  • osteochondroma

Malignant
- chondrosarcoma

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8
Q

Give examples of fibrous tissue tumours

A

Benign
- fibroma

Malignant

  • fibrosarcoma
  • malignant fibrous histiocytoma
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9
Q

Give examples of vascular tissue tumours

A

Benign

  • haemangioma
  • aneurysmal bone cyst

Malignant
- angiosarcoma

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10
Q

Give examples of adipose tissue tumours

A

Benign
- lipoma

Malignant
- liposarcoma

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11
Q

Give examples of marrow tissue tumours

A

Malignant

  • Ewing’s sarcoma
  • Lymphoma
  • Myeloma
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12
Q

What is the incidence of osteosarcoma in the UK?

A

3 per million population per year

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13
Q

What is the incidence of chondrosarcoma in the UK?

A

2 per million population per year

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14
Q

What is the incidence of Ewing’s tumour in the UK?

A

1.5 per million population per year

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15
Q

What is the incidence of malignant fibrous histiocytoma in the UK?

A

< 1 per million population per year

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16
Q

What is the commonest primary malignant bone tumour?

A

Osteosarcoma in younger patients

Myeloma in older patients

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17
Q

What is the typical history of a bone/soft tissue tumour?

A

Mass
Abnormal x-rays
Pain if bone tumour
May be painless if soft tissue tumour

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18
Q

What are the features of pain caused by bone tumours?

A
Activity related
Increasing 
Progressive pain at rest
Unrelenting 
Night time pain
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19
Q

When might benign tumours present with activity-related pain?

A

If they are large enough to weaken the affected bone

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20
Q

What are the important examinations in a patient presenting with a bone/soft tissue tumour?

A
General health 
Measure swelling 
Location 
Shape 
Consistency 
Mobility 
Tenderness
Local temperature 
Neuro-vascular deficits
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21
Q

What are the investigations for a bone/soft tissue tumour?

A
Plain radiographs 
Bloods
MRI 
CT 
Isotope bone scan 
Angiography 
Biopsy
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22
Q

What bloods should you do for a patient with a bone/soft tissue tumour?

A
FBC - anaemia 
Blood film 
ESR and CRP 
Ca (would be raised) 
Phosphate 
Alkaline phosphatase 
LFTs
Plasma protein electrophoresis 
PSA 
Exclude infection and leukaemia
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23
Q

What might you see on imaging of a bone/soft tissue tumour?

A
Plain x-rays most useful for bone lesions
Calcification - synovial sarcoma 
Myositis ossificans 
Phleboliths in haemangiomas
Fat density in lipoma
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24
Q

What would be seen on x-ray of an inactive bone/soft tissue tumour?

A

Clear margins
Surrounding rim of reactive bone
Cortical expansion with aggressive benign lesions

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25
Q

What would be seen on x-ray of a malignant bone/soft tissue tumour?

A

Less well-defined zone of transition between lesion and normal bone - permeative growth
Cortical destruction
Periosteal reactive new bone growth when lesion destroys the cortex
- Codman’s triangle
- sunburst pattern
- onion skinning

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26
Q

What are plain radiographs best for viewing?

A

Bony destruction and periosteal reaction

Conventional 2 views;

  • bone destruction
  • bone expansion
  • bone production
  • pathological fracture
  • soft tissue swelling
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27
Q

Why would you do a CXR for a patient with a bone tumour?

A

80% of primary malignant bone tumours metastasise to the chest

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28
Q

What is MRI best for showing?

A

Size, extent and anatomical relationships of lesions

Intraosseous extent of tumour 
Extraosseous soft tissue extent of tumour 
Joint involvement 
Skip lesions 
Epiphyseal extension
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29
Q

What is MRI specific for?

A

Lipoma
Haemangioma
Haematoma
PVNS

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30
Q

What is MRI non-specific for?

A

Benign vs malignant

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31
Q

What is the use of CT in bone/soft tissue tumours?

A

Assessing ossification and calcification
Integrity of cortex
Good for subtle bony cortical destruction, features and calcification
Best for assessing nidus in osteoid osteoma
Staging - primary of lungs

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32
Q

What is the use of isotope bone scans in bone/soft tissue tumours?

A

Staging for skeletal metastasis
Benign also demonstrate increased uptake
Multiple lesions
Frequently negative in myeloma

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33
Q

What is the use of ultrasound in bone/soft tissue tumours?

A

Cystic vs solid soft tissue lesions

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34
Q

What is the use of angiography in bone/soft tissue tumours?

A
Superseded by MRI 
Pseudoaneurysms and AV malformations 
Embolisation of vascular tumours 
Defines vascular characteristics 
Renal secondaries, aneurysmal bone cyst
Allows pre-operative embolisation
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35
Q

When might PET be useful in bone/soft tissue tumours?

A

For investigating response to chemotherapy

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36
Q

What should be done prior to biopsy of a bone/soft tissue tumour?

A
Complete work up 
Bloods 
X-rays of affected limb and chest
MRI 
Bone scan 
CT chest, abdomen and pelvis
Angiography 
Careful evaluation and planning
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37
Q

Describe the process of a bone/soft tissue tumour biopsy

A

Needle, core or open biopsy
Track is contaminated so placement is crucial for later excision
Tourniquet - elevation not exsanguination
Avoid transverse incisions
Avoid major neuromuscular structures
Sample soft tissue extensions of bone tumours
Frozen section
Open biopsy yields greatest tissue but highest complications
Core biopsy > 90% accurate
Excision biopsy for < 5cm subcutaneous lesions in expendable bones

38
Q

How are bone/soft tissue tumours staged?

A
Enneking staging system 
Benign vs malignant 
Histological grade - low/high 
Local extent - site, intra/extra-compartmental 
Metastases 

Aids in treatment decision making and allows meaningful comparisons of treatment methods

39
Q

What are the most significant prognostic factors to guide surgery and adjuvant treatments?

A

Size
Histological grade
Presence/absence of metastases
Soft tissue - depth

40
Q

What are the features of low grade bone/soft tissue tumours?

A
Stage I 
Well-differentiated 
Few mitoses
Moderate cytological atypica
< 25% risk for metastases
41
Q

What are the features of high grade bone/soft tissue tumours?

A

Stage II
Poorly differentiated
High mitotic rate
High cell/matrix ratio

42
Q

What compartments might be affected in bone/soft tissue tumours?

A

Cortical bone
Articular cartilage
Fascial septa
Joint capsules

43
Q

Where do tumours graded IB and IIB extend?

A

Beyond the compartment of origin

44
Q

What is stage III?

A

Any lesion that has metastasised

Regardless of size or histological grade

45
Q

What are the clinical features of myeloma?

A
Pain 
Loss of function 
Swelling 
Pathological fracture 
Joint effusion 
Deformity 
Neurovascular effects
Systemic effects of neoplasia
46
Q

What are the features of pain due to myeloma?

A

Cardinal feature, especially in children
Severity does not correspond with degree of malignancy
Increasing pain indicates impending fracture, especially lower limb
Analgesics eventually ineffective
Not related to exercise
Deep ache
Worse at night is a red flag

47
Q

What are the features of loss of function due to myeloma?

A

Limp
Reduced joint movement
Stiff back, especially in children

48
Q

What are the features of swelling due to myeloma?

A

Generally diffuse in malignancy
Generally near end of long bone
Once reaching notable size, enlargement may be rapid
Warmth over swelling and venous congestion = active
Pressure effects

49
Q

What are the features of pathological fracture due to myeloma?

A

Usually weight-bearing bone

Minimal trauma and history of pain prior to fracture

50
Q

What are the neuromuscular effects of myeloma?

A

Peripheral nerves affected e.g. common peroneal nerve at fibular neck

51
Q

What are the systemic effects of neoplasia?

A
Weight loss 
Anorexia 
Malaise
Fatigue 
Mood change 
Anaemia
52
Q

What are the treatment options for myeloma?

A
Goal is to make patient disease-free 
Chemotherapy 
Surgery 
Radiotherapy 
MDT approach
53
Q

What is the 5 year survival of modern chemotherapy regimens for myeloma?

A

Approaching 70%

54
Q

When is chemotherapy used in bone/soft tissue tumours?

A

Osteosarcoma
High grade bone lesions
High grade soft tissue lesions of childhood
Less well defined for adult soft tissue tumours
In general not used for cartilaginous or low grade lesions
Adjuvant - post-operatively for micro-metastases
Neoadjuvant - before surgical resection, limb salvage easier but no significant difference to survival

55
Q

If chemotherapy is given neo-adjuvantly, when is surgery done and when is chemotherapy re-started?

A

Surgery 3-4 weeks after last cycle

Restarted when wound healed

56
Q

When is chemotherapy most effective?

A

When tumour is small, combinations more effective

57
Q

Wha tis important in achieving maximal response to chemotherapy?

A

Dosage, sequence of drugs and schedule

58
Q

What are the benefits of chemotherapy?

A

Reduces risk of tumour spill at surgery

Treats micro-metastases early

59
Q

When might surgery be needed for bone/soft tissue tumours?

A

Limb salvage
Involvement of neurovascular structures
Pathological fracture
Poorly-performed biopsy

60
Q

What resection and reconstruction might be done for bone/soft tissue tumours?

A
Wide surgical margins 
Marginal with adjuvant or neoadjuvant therapies 
Osteoarticular allograft 
Endoprosthetic reconstruction 
Allograft prosthesis composite 
Rotationoplasty
61
Q

What are the surgical margins for bone/soft tissue tumours?

A

Intralesional - debulking, benign or metastatic
Marginal - pseudocapsule, benign or low grade
Wide - plane through normal tissue, goal for high grade tumours
Radical - all compartments containing tumour removed

62
Q

What are the risks of limb salvage surgery?

A

Extensive surgery

  • infection
  • wound dehiscence
  • flap necrosis
  • blood loss
  • DVT
  • peri-prosthetic fractures
  • loosening, dislocation, non-union of graft-host junction
  • allograft fracture
  • limb length discrepancy
  • late infection
63
Q

What are the issues of amputation vs salvage?

A

Will survival be affected?
How do short and long-term morbidity compare?
Function of salvaged limb compared to prosthesis
Psychological consequences

64
Q

What is the local recurrence of osteosarcoma?

A

5-10%

65
Q

What are the disadvantages of amputation?

A
Technically demanding, non-standard
Infection 
Wound dehiscence 
Chronic pain 
Phantom pain
66
Q

What does sensitivity to radiotherapy depend on?

A

Cell’s position in cell cycle, actively mitotic are more sensitive
Tissue oxygenation, hypoxia is protective of O2 free radicals
Cell’s ability to repair DNA damage
Cell’s inability to undergo apoptosis in response to injury

67
Q

How do more primary bone malignancies response to radiotherapy?

A

Most are relatively radio-resistant

Exceptions;

  • multiple myeloma
  • lymphoma
  • Ewing’s
  • metastatic carcinoma
68
Q

What is radiotherapy used for in soft tissue sarcoma?

A

Used to reduce local recurrence, particularly in marginal resections

69
Q

What are the complications of radiotherapy?

A

Skin complications
Osteonecrosis
Sarcoma (10 years)
Pathological fracture (29% are 5 years)

70
Q

What are the features of hereditary multiple exostoses?

A

Diaphyseal aclasis
Autosomal dominant
Cartilage-capped bony outgrowths typically near physes of long bones
May cause deformity/loss of function/pain
May be sessile or pedunculated
Perichondrial covering and cartilage cap consisting of zones of maturing cartilage similar to growth plate
Benign bone tumours

71
Q

What is the risk of malignant changes in isolated osteochondromata?

A

< 1%

72
Q

What is the risk of malignant change in hereditary multiple exostoses?

A

5%

Especially in large exostoses with thick cartilage cap, axial location or continuing to grow in adulthood

73
Q

All patients with a soft tissue tumour suspected to be malignant should be referred where?

A

To a specialist tumour centre

74
Q

What are suspicious signs of a soft tissue tumour?

A

Deep to deep fascia, tumour of any size
Subcutaneous tumours > 5cm
Rapid growth, hard, craggy, non-tender
Lipoma

75
Q

What are some red flags for swelling?

A

Rapidly growing
Hard, craggy surface, fixed, indistinct margins
Non-tender to palpation but associated with deep ache, especially if pain is worse at night
Recurred after previous excision (severe)

76
Q

How many times more common is a secondary bone tumour compared to a primary bone tumour?

A

Secondary bone tumour 25 times commoner than primary

Bone most common site of secondaries after lung and liver

77
Q

How many women with breast cancer develop secondaries each year?

A

Approx 9000

78
Q

How many new cases of cancer have secondaries each year?

A

20,000

79
Q

What are the common bony sites for metastases?

A

From most common to least

  • vertebrae
  • proximal femur
  • pelvis
  • ribs
  • sternum
  • skull
80
Q

What are the commonest primary cancers which metastasise to bone?

A

Lung - smoker, CXR, sputum cytology
Breast - commonest, examine
Prostate - osteosclerotic secondary, PR, PSA
Kidney - solitary, vascular, IVP and US, angiography and embolise
Thyroid - especially follicular cancer, examine
GI tract - FOB, endoscopy, Ba studies, tumour markers
Melanoma - examine

81
Q

What is the pathogenesis of metastasis?

A

Local invasion of normal stromal cells by primary tumour
Increased local pressure and dissection by tumour cells along lines of least resistance and release of lytic enzymes
Tumour cells invade local lymphatics and/or vessels, may remain or may be dislodged (embolisation)
Tumour emboli normally destroyed by killer cells or vascular turbulence etc.
Few tumour emboli survive to become embedded and adherent to distant capillary bed of preferred organ
Invasion continues and tumour angiogenesis occurs

82
Q

What is the mechanism of bone invasion?

A

Red marrow bones only e.g. vertebrae, ribs, pelvis, skull, proximal femur
Rare distal to knee/elbow

83
Q

What are the clinical features of hypercalcaemia due to bone destruction?

A
Nausea 
Vomiting 
Anorexia 
Lethargy 
Confusion 
Coma
84
Q

What is the early treatment of pathological fracture?

A

Chemotherapy/DXT

85
Q

When is prophylactic internal fixation indicated (pathological fracture)?

A

Lytic lesion 2.5cm+ diameter
50%+ cortical destruction
Increasing pain

86
Q

If embolisation occurs following a pathological fracture, how long should you wait before surgery?

A

48 hours

87
Q

What are the aims of surgical treatment of pathological fracture?

A

Only one long bone at a time

Aim for early painless weight-bearing and mobilisation

88
Q

What is the failure rate of fixation of pathological fracture or lytic lesions?

A

High failure rate

89
Q

What is the failure rate of cemented hip prostheses?

A

Low failure rate

90
Q

What does surgery for spinal metastases usually require?

A

Decompression and stabilisation