Bone Tumours Flashcards

1
Q

Where and when do primary and secondary ossification centres appear in long bone?

A

Primary - centre of the diaphysis during intrauterine life.

Secondary - each epiphyseal end sometime after birth.

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2
Q

How do bone tumours typically present?

A
  1. Pain
    - Unexplained limb pain >1 month
    - Pain at night
  2. Swelling
  3. Pathological fracture
  4. Incidental finding - most primaries
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3
Q

What are the 3 forewarning signs of malignancy on X-ray?

A
  1. Cortical destruction
  2. Periosteal reaction
    - Fuzzy line outside of cortex
    - Also seen with # or infection
  3. Zone of transition
    - Sclerotic margin = narrow zone
    - Diffuse margin - more aggressive
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4
Q

List 6 clinical indicators of malignancy?

A
  1. Pain (>1 month, at night)
  2. Swelling
  3. Tenderness
  4. Warmth
  5. X-ray changes (3 signs)
  6. Rapid growth of a lesion
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5
Q

What investigations are indicated if bone malignancy is suspected?

A
  1. X-ray
  2. Bone scans
  3. CT
  4. MRI
  5. ESR - raised
  6. ALP - raised
  7. Biopsy
    Refer to specialist centre early
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6
Q

Recite the mnemonic for benign bone tumours

A

ABC FFG OO

Aneurysmal bone cyst
Bone cyst
- Simple, unicameral or solitary
Chondroma
- Aka endrochondroma
Fibrous cortical defect
- Aka nonossifying fibroma
Fibrous dysplasia
Giant cell tumour
Osteochondroma
Osteoid osteoma
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7
Q

Expansile cysts that usually affect under 30s. Patients present with pain. MRI shows multiple fluid levels.

A

Aneurysmal bone cyst (benign tumour)

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8
Q

What is the pathognomonic sign for aneurysmal bone cysts, as seen on MRI?

A

Multiple fluid levels

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9
Q

How are symptomatic aneurysmal bone cysts treated?

A

Curette out cyst and fill with bone graft.

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10
Q

In which bones and in whom do simple bone cysts most commonly occur?

A

Proximal humerus and femur.
Young patients.
Usually asymptomatic.

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11
Q

When might you see the ‘fallen fragment sign’?

A

Specific radiological sign for a unicameral bone cyst with pathological fracture. Part of the thinned cortex ‘falls’ into the fluid-filled cyst.

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12
Q

Differentiate between Ollier’s disease, Maffucci’s syndrome and metachondromatosis.

A
Multiple enchondromas present in 3 disorders:
Ollier disease 
- Sporadic (more common) 
- Unilateral distribution
Maffucci's syndrome
- Sporadic
- Associated with hemangiomas 
Metachondromatosis 
- Autosomal-dominant 
- Both multiple osteochondromas and enchondromas.
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13
Q

What are (endo)chrondromas and which bones are most commonly affected?

A

The most common benign tumours of hyaline cartilage

Commonest site: phalanges. Can affect other long bones.

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14
Q

A 43 year-old man presents with ongoing pain and swelling in his hands and feet. X-ray findings reveal cystic lesions of the phalanges. Which condition is it important to exclude?

A

Low-grade chondrosarcoma - may develop from benign cystic lesions (chondromas)

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15
Q

What is a nonossifying fibroma and how does it typically present?

A

Aka fibrous cortical defect.

  • Benign defect of cortex
  • Common: incidental finding on X-ray in 20% of children
  • Nonpainful
  • Usually affects metaphysis
  • Usually spontaneously regress
  • Rarely seen after age 30
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16
Q

Differentiate between monostotic and polystotic fibrous dysplasias

A

Fibrous dysplasia = areas of bone are replaced by fibrous tissue.

Monostotic = localised

  • Solitary segment affected
  • More common (70-80%)
  • Unknown cause
  • Incidental X-ray finding or presents as pathological # with pain

Polystotic = generalised

  • 20-30%, several bones affected
  • Presentation: progressive deformity (bending, enlargement), pain and pathological fracture
  • X-ray: ground glass or smokey
  • Elephant man?!
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17
Q

What is McCune-Albright syndrome?

A

A rare condition in which polyostotic fibrous dysplasia occurs in association with café au lait spots and (in females) precocious puberty (endocrinopathies).

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18
Q

In whom and in which bone(s) are giant cell tumours most commonly seen?

A

Occur in young adults (always after fusion of growth plate) usually around knee (lower femur or upper tibia) and always abut the articular surface.

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19
Q

Which is the commonest tumour of the bone?

A

Osteochondroma - aka “exostosis”, a cartilage-capped outgrowth. Arise from metaphysis, especially about knee. M>F (3:1)

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20
Q

A patient presents with a bony lump. X-ray shows a cauliflower-shaped projection from bone that is smaller than the size to touch. What is the most likely diagnosis?

A

Osteochondroma - if symptomatic, excise tumour.

Chondrosarcoma - if it changes in size after skeletal maturity.

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21
Q

A 20 year old patient presents with ongoing leg pain which is relieved by aspirin. X-ray shows a small radiolucent area surrounded by dense sclerosis which appears as a hot spot on a bone scan. What is the most likely diagnosis and how would you treat?

A

Osteoid osteoma - a benign tumour (

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22
Q

How does an osteoblastoma differ to an osteoid osteoma?

A

An uncommon primary bone tumour with clinical and histological similarities to osteoid osteoma, but larger (2-6 cm). Usually benign and related to spine.

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23
Q

In whom do osteosarcomas most commonly occur?

A

Males > females (3:1)
Primary: adolescents (under 20)
Secondary: over 50s (due to Paget’s disease)

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24
Q

What is the main symptom of osteosarcoma?

A

Pain, especially nocturnal

+/- local tenderness

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25
What are the radiological features of osteosarcoma?
X-ray: - Metaphyseal, translucent, destructive lesion - Expands through cortex, lifts, periosteum - Codman's triangle - 'Sun-ray' appearance
26
What is Codman's triangle?
Radiographic feature: | Triangular area of new subperiosteal bone, created when a lesion raises the periosteum away from the bone
27
What are 'sun-ray' and 'sunburst' appearances caused by?
Sun-ray appearance occurs with rapidly-growing lesions - Streak of calcification - Periosteum has no time to lay down thin shell of bone - Sharpey's fibers stretch out, perpendicular to the bone When these fibres ossify, they produce a pattern sometimes called "sunburst" periosteal reaction
28
How are malignant bone tumours treated and what is their prognosis?
1. Chemotherapy - except chondrosarcomas 2. Resection - Amputation - Or wide local excision (allograft or prosthesis) Five-year survival - Osteosarcoma: 60% - Chondrosarcoma: 50%
29
Name the rare malignant tumour that arises from bone marrow, usually in young patients.
Ewing's sarcoma - boys > girls
30
True or false: most Ewing's sarcomas occur in diaphysis of long bones
True - especially femur, also tibia and humerus
31
True or false: osteosarcomas tend to be metaphyseal
True
32
A 25 year-old patient presents with a painful pelvic swelling that is warm and tender. X-ray shows an onion-skin appearance. What is the diagnosis?
Ewing's sarcoma - occasionally misdiagnosed as osteomyelitis
33
What causes an 'onion skin' appearance on X-ray?
Several layers of periosteal new bone around destructive lesion - seen in Ewing's sarcoma
34
A 45 year-old patient presents with a painful swelling in pelvis. X-ray shows an expanding radiolucent lesion with flecks of calcification. What is the diagnosis?
Chondrosarcoma - usually affects older patients (40+)
35
Describe the two types of chondroma/chondrosarcoma
1. Subperiosteal chondroma: arises on surface of bone - Sometimes in cartilage-covered cap of osteochondroma 2. Endochondroma: arises within medulla of bone - Either slow-growing malignancy or benign chondroma that becomes malignant
36
True or false: chondrosarcomas tend to be treated with chemotherapy before excision
False - Rx by excision or amputation since they metastasise late
37
Which malignant tumours are more common in younger patients (under 30)?
1. Osteosarcoma | 2. Ewing's sarcoma
38
Which malignant tumours are more common in older patients (over 40)?
1. Chondrosarcoma 2. Myeloma 3. Metastases
39
Which cancers most commonly metastasise to bone?
'Bone Tumours are Rarely Bony Primaries' 1. Breast 2. Thyroid 3. Renal 4. Bronchus 5. Prostate
40
True or false: most metastases are osteoclerotic lesions
False - majority are lytic lesions Exception: prostate - usually osteosclerotic However, breast and thyroid are sometimes osteosclerotic
41
Which bones do secondaries tend to metastasise to?
``` Axial skeleton (red marrow) - Spine, pelvis, ribs, proximal and of long bones ```
42
How do secondary bone tumours tend to present?
1. Local pain | 2. Pathological fracture
43
How are pathological fractures treated?
Internal fixation - as they tend not to heal
44
How do secondary bone tumour tend to be treated?
1. If local - radiotherapy | 2. If multiple - palliative (poor prognosis)
45
True or false: most benign lesions are seen <30 years of age
True
46
True or false: a new bone tumour in the elderly is more likely to be malignant
True
47
What is the best test for diagnosis and staging of bone tumours?
Dx: X-ray Staging: CT or MRI Histology grade - Most important prognostic feature of sarcomas - Essential for staging of most bone tumour types
48
What are the most common benign lesions in the under 20s?
``` ABC FFG OO - Endochondroma - Giant cell tumour + Osteoblastoma + Chondroblastoma + Chondromyxoid fibroma + Eosinophilic granuloma ```
49
What are the most common malignant tumours in the under 20's?
1. Osteosarcoma 2. Ewing's sarcoma 3. Metastatic neuroblastoma 4. Leukaemic involvement In younger patients think osteosarcoma or Ewing's sarcoma!
50
What are the most common benign lesions in 21-40 year olds?
1. Endochondroma | 2. Giant cell tumour
51
What are the most common malignant tumours in 21-40 year olds?
Chondrosarcoma In older patients think chondrosarcoma, myeloma and mets!
52
What is the most common benign lesion in the over 40s and in which bones does it commonly arise?
Osteoma - facial bones, skull
53
What are the most common malignant lesions in the over 40s?
1. Metastatic tumours 2. Myeloma 3. Leukaemic involvement 4. Chondrosarcoma 5. Osteosarcoma (Paget's associated) 6. Malignant fibrous histiocytoma 7. Chordoma In older patients think chondrosarcoma, myeloma and mets!
54
True or false: most secondary bone tumours have favoured sites within long bone
False - this is true of primaries
55
Diaphyseal cortical lesion
Osteoid osteoma
56
Diaphyseal intramedullary lesion
1. *Fibrous dysplasia* 2. *Endochondroma* 3. Ewing's sarcoma 4. Lymphoma 5. Myeloma
57
Metaphyseal cortical lesion
1. *Non-ossifying fibroma* | 2. Osteoid osteoma
58
Metaphyseal intramedullary lesions
1. *Osteosarcoma* 2. Chondrosarcoma 3. Fibrosarcoma 4. Osteoblastoma 5. Endochondroma 6. Fibrous dysplasia 7. Simple bone cyst 8. Aneurysmal bone cyst
59
Epiphyseal lesions
1. *Chondroblastoma* - rare, open growth plates | 2. *Giant cell tumour* - closed growth plates
60
Metaphyseal exostosis
Osteochondroma
61
What are the commonest sites for an osteosarcoma?
Primary: locally invasive (metaphysis) - Around knee (distal femur/proximal tibia) - Proximal humerus Secondary: femur, humerus, pelvis
62
Where do osteosarcomas commonly metastasise to?
Often metastasises to lung via blood
63
Where do chondrosarcomas commonly arise and where do they spread to?
Common sites: central portions of skeleton: - Pelvis, proximal femur, ribs, sternum, shoulder girdle Spreads to lungs and skeleton
64
What is a giant cell tumour?
Uncommon, benign bone tumour. Contains a profusion of multinucleate osteoclast type giant cells. Usually benign but may become locally aggressive and metastasise. Arises during 5th decade. Female predominance
65
In which bones are osteoblastomas commonly found?
Vertebral column
66
What are Homer-Wright rosettes?
Small round blue cells arranged in rosettes with a neurofibrillar centre - pathobiologic marker for Ewing's sarcoma
67
What is a chordoma?
A rare slow-growing neoplasm thought to arise from cellular remnants of the notochord. Chordomas can arise from bone in the skull base and anywhere along the spine (neuraxis). Commonest locations: 1. Cranially at the clivus 2. Sacrum