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
What would be seen on x-ray of a malignant bone/soft tissue tumour?
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
26
What are plain radiographs best for viewing?
Bony destruction and periosteal reaction Conventional 2 views; - bone destruction - bone expansion - bone production - pathological fracture - soft tissue swelling
27
Why would you do a CXR for a patient with a bone tumour?
80% of primary malignant bone tumours metastasise to the chest
28
What is MRI best for showing?
Size, extent and anatomical relationships of lesions ``` Intraosseous extent of tumour Extraosseous soft tissue extent of tumour Joint involvement Skip lesions Epiphyseal extension ```
29
What is MRI specific for?
Lipoma Haemangioma Haematoma PVNS
30
What is MRI non-specific for?
Benign vs malignant
31
What is the use of CT in bone/soft tissue tumours?
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
32
What is the use of isotope bone scans in bone/soft tissue tumours?
Staging for skeletal metastasis Benign also demonstrate increased uptake Multiple lesions Frequently negative in myeloma
33
What is the use of ultrasound in bone/soft tissue tumours?
Cystic vs solid soft tissue lesions
34
What is the use of angiography in bone/soft tissue tumours?
``` Superseded by MRI Pseudoaneurysms and AV malformations Embolisation of vascular tumours Defines vascular characteristics Renal secondaries, aneurysmal bone cyst Allows pre-operative embolisation ```
35
When might PET be useful in bone/soft tissue tumours?
For investigating response to chemotherapy
36
What should be done prior to biopsy of a bone/soft tissue tumour?
``` Complete work up Bloods X-rays of affected limb and chest MRI Bone scan CT chest, abdomen and pelvis Angiography Careful evaluation and planning ```
37
Describe the process of a bone/soft tissue tumour biopsy
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
How are bone/soft tissue tumours staged?
``` 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
What are the most significant prognostic factors to guide surgery and adjuvant treatments?
Size Histological grade Presence/absence of metastases Soft tissue - depth
40
What are the features of low grade bone/soft tissue tumours?
``` Stage I Well-differentiated Few mitoses Moderate cytological atypica < 25% risk for metastases ```
41
What are the features of high grade bone/soft tissue tumours?
Stage II Poorly differentiated High mitotic rate High cell/matrix ratio
42
What compartments might be affected in bone/soft tissue tumours?
Cortical bone Articular cartilage Fascial septa Joint capsules
43
Where do tumours graded IB and IIB extend?
Beyond the compartment of origin
44
What is stage III?
Any lesion that has metastasised | Regardless of size or histological grade
45
What are the clinical features of myeloma?
``` Pain Loss of function Swelling Pathological fracture Joint effusion Deformity Neurovascular effects Systemic effects of neoplasia ```
46
What are the features of pain due to myeloma?
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
What are the features of loss of function due to myeloma?
Limp Reduced joint movement Stiff back, especially in children
48
What are the features of swelling due to myeloma?
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
What are the features of pathological fracture due to myeloma?
Usually weight-bearing bone | Minimal trauma and history of pain prior to fracture
50
What are the neuromuscular effects of myeloma?
Peripheral nerves affected e.g. common peroneal nerve at fibular neck
51
What are the systemic effects of neoplasia?
``` Weight loss Anorexia Malaise Fatigue Mood change Anaemia ```
52
What are the treatment options for myeloma?
``` Goal is to make patient disease-free Chemotherapy Surgery Radiotherapy MDT approach ```
53
What is the 5 year survival of modern chemotherapy regimens for myeloma?
Approaching 70%
54
When is chemotherapy used in bone/soft tissue tumours?
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
If chemotherapy is given neo-adjuvantly, when is surgery done and when is chemotherapy re-started?
Surgery 3-4 weeks after last cycle | Restarted when wound healed
56
When is chemotherapy most effective?
When tumour is small, combinations more effective
57
Wha tis important in achieving maximal response to chemotherapy?
Dosage, sequence of drugs and schedule
58
What are the benefits of chemotherapy?
Reduces risk of tumour spill at surgery | Treats micro-metastases early
59
When might surgery be needed for bone/soft tissue tumours?
Limb salvage Involvement of neurovascular structures Pathological fracture Poorly-performed biopsy
60
What resection and reconstruction might be done for bone/soft tissue tumours?
``` Wide surgical margins Marginal with adjuvant or neoadjuvant therapies Osteoarticular allograft Endoprosthetic reconstruction Allograft prosthesis composite Rotationoplasty ```
61
What are the surgical margins for bone/soft tissue tumours?
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
What are the risks of limb salvage surgery?
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
What are the issues of amputation vs salvage?
Will survival be affected? How do short and long-term morbidity compare? Function of salvaged limb compared to prosthesis Psychological consequences
64
What is the local recurrence of osteosarcoma?
5-10%
65
What are the disadvantages of amputation?
``` Technically demanding, non-standard Infection Wound dehiscence Chronic pain Phantom pain ```
66
What does sensitivity to radiotherapy depend on?
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
How do more primary bone malignancies response to radiotherapy?
Most are relatively radio-resistant Exceptions; - multiple myeloma - lymphoma - Ewing's - metastatic carcinoma
68
What is radiotherapy used for in soft tissue sarcoma?
Used to reduce local recurrence, particularly in marginal resections
69
What are the complications of radiotherapy?
Skin complications Osteonecrosis Sarcoma (10 years) Pathological fracture (29% are 5 years)
70
What are the features of hereditary multiple exostoses?
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
What is the risk of malignant changes in isolated osteochondromata?
< 1%
72
What is the risk of malignant change in hereditary multiple exostoses?
5% | Especially in large exostoses with thick cartilage cap, axial location or continuing to grow in adulthood
73
All patients with a soft tissue tumour suspected to be malignant should be referred where?
To a specialist tumour centre
74
What are suspicious signs of a soft tissue tumour?
Deep to deep fascia, tumour of any size Subcutaneous tumours > 5cm Rapid growth, hard, craggy, non-tender Lipoma
75
What are some red flags for swelling?
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
How many times more common is a secondary bone tumour compared to a primary bone tumour?
Secondary bone tumour 25 times commoner than primary Bone most common site of secondaries after lung and liver
77
How many women with breast cancer develop secondaries each year?
Approx 9000
78
How many new cases of cancer have secondaries each year?
20,000
79
What are the common bony sites for metastases?
From most common to least - vertebrae - proximal femur - pelvis - ribs - sternum - skull
80
What are the commonest primary cancers which metastasise to bone?
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
What is the pathogenesis of metastasis?
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
What is the mechanism of bone invasion?
Red marrow bones only e.g. vertebrae, ribs, pelvis, skull, proximal femur Rare distal to knee/elbow
83
What are the clinical features of hypercalcaemia due to bone destruction?
``` Nausea Vomiting Anorexia Lethargy Confusion Coma ```
84
What is the early treatment of pathological fracture?
Chemotherapy/DXT
85
When is prophylactic internal fixation indicated (pathological fracture)?
Lytic lesion 2.5cm+ diameter 50%+ cortical destruction Increasing pain
86
If embolisation occurs following a pathological fracture, how long should you wait before surgery?
48 hours
87
What are the aims of surgical treatment of pathological fracture?
Only one long bone at a time | Aim for early painless weight-bearing and mobilisation
88
What is the failure rate of fixation of pathological fracture or lytic lesions?
High failure rate
89
What is the failure rate of cemented hip prostheses?
Low failure rate
90
What does surgery for spinal metastases usually require?
Decompression and stabilisation