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SARCOMAS Flashcards

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1
Q

What are the 2 categories of sarcomas?

A

Bone sarcomas and soft tissue sarcomas

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2
Q

What are the types of malignant bone tumours?

A

Osteosarcoma
Chondrosarcoma
Ewing’s sarcoma

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3
Q

What are the types of soft tissue sarcomas?

A

Liposarcoma
Rhabdomyosarcoma (striated muscle origin)
Synovial sarcoma
Fibrosarcoma
Angiosarcoma
Leiomyosarcoma (smooth muscle origin)

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4
Q

What is the most common type of primary bone cancer in adults?

A

Chondrosarcoma

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5
Q

What is the most common type of primary bone cancer in children and young adults?

A

Osteosarcoma

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6
Q

What is the most common primary maligannt bone tumour overall?

A

Osteosarcoma

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7
Q

What are the types of benign bone tumours?

A

Osteoma
Osteochondroma
Giant cell tumour

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8
Q

What is an osteoma?

A

A small, benign overgrowth of bone that most typically occurs on the skull

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9
Q

What syndrome are osteomas associated with?

A

Gardner’s syndrome (a variant of familial adenomatous polyposis)

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10
Q

What is an osteoid osteoma?

A

A small osteogenic benign tumour often seen in patients under the age of 30
Arise from osteoblasts
Often occurs in long bones in the diaphysis or metaphysis
Smaller than osteomas ans characterised by the presence of a central core of abnormal bone tissue called osteoid

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11
Q

Presentation of osteoid osteomas?

A

Constant and progressive pain that is worse at night
Relieved by NSAIDs (this is a diagnostic hallmark due to increased levels of COX enzymes and prostaglandins in the lesion)

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12
Q

What is the most common benign bone tumour?

A

An osteochondroma

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13
Q

Who do osteochondromas most commonly affect?

A

Males
<20

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14
Q

What is thought to cause osteochondromas?

A

Mutations in exostosin genes which encode exostosin proteins which help to synthesise heparin sulphate. This is a protein that regulates the growth of the growth plate

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15
Q

What is an osteochondroma?

A

A benign bony tumour that starts near the growth plate and it results in an exostosis (lateral bony projection)

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16
Q

Where do osteochondromas most commonly occur?

A

In the metaphysis of long bones - most commonly the distal femur and proximal tibia
Pelvis
Scapula

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17
Q

What is a giant cell tumour?

A

A benign but locally aggressive tumour of multi nucleated giant cells (osteoclasts) within a fibrous stroma

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18
Q

Where do giant cell tumours most typically develop?

A

In the epiphysis of the long bones e.g. distal femur and proximal tibia

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19
Q

Peak incidence for age of giant cell tumours?

A

20-40

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20
Q

XR sign for giant cell tumours?

A

Double bubble or soap bubble appearance - this is because it producing lytic lesions

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21
Q

What are the 2 benign bone tumours that arise from osteoblasts?

A

Osteoblastomas
Osteoid osteomas

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22
Q

What is a nidus?

A

A disorganised mix of small blood vessels, trabeculae and osteoid

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23
Q

Which bone tumours form a nidus?

A

Osteoblastomas
Osteoid osteomas

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24
Q

Whats the difference between the nidus in Osteoblastomas and osteoid osteomas?

Study These Flashcards
A

Large >1.5cm in Osteoblastomas
Small <1.5cm in osteoid osteomas and are often surrounded by a ring of sclerotic bone tissue which produces prostaglandins

25
Where do Osteoblastomas tend to affect?
The axial skeletal e.g. spine and mandible
26
Who do chondrosarcomas most commonly occur in?
30-60 year olds More commonly men
27
What is the difference between primary and secondary chondrosarcomas?
Primary - typically develop in the centre of metaphysis and diaphysis of long bones Secondary - develop in periphery of flat bones e.g. scapula and sacrum
28
What can chondrosarcomas sometimes arise from?
Pre-existing osteochondromas that transform
29
What are chondrosarcomas?
Malignant bone tumours arising from chondriocytes
30
What do chondrosarcomas appear as on XR?
Radiolucent area with central flecks of calcification within the medullary cavity Often described as “moth-eaten cloth”
31
What is an osteosarcoma?
A malignant bone tumour arising from osteoblasts
32
Who do osteosarcomas most commonly occur in?
Children and adolescents Can occur in older adults over 60 as a complication
33
Risk factors for osteosarcoma in older adults?
Paget’s disease Pre-existing bone lesions Radiation exposure
34
Where do osteosarcomas typically affect?
Metaphysis of long bones where there is rapid bone growth e.g. femur, proximal tibia and proximal humerus
35
Risk factor for osteosarcoma in children?
Retinoblastoma - as pRB is implicated in osteosarcoma
36
XR findings of osteosarcoma?
Codman’s triangle Sunburst pattern
37
What is Codman’s triangle?
the radiographic appearance of the rim of new subperiosteal bone which forms when a lesion such as a tumour lifts the periosteum away from the bone Seen in osteosarcoma, ewings sarcoma and subperiosteal abscesses
38
What is a sunburst pattern on XR seen in osteosarcoma?
This occurs when lesions grow too fast ans the margins are poorly defined with an aggressive periosteal reaction
39
Who are Ewing’s sarcomas most common in?
Children and adolescents
40
What do Ewing’s sarcomas arise from?
The cell origin of the sarcoma is unknown but the current theory points to mesenchymal stem cells with the chromosomal translocation t(11;22)(q24;q12) resulting in the EWSR1-FLI1 fusion protein.
41
Where do Ewing’s sarcoma most commonly arise from?
Most frequently in the long bones and pelvis
42
XR findings of Ewing’s sarcoma?
An area of bone destruction Onion-peel pattern
43
What is the onion skin appearance seen in Ewing’s sarcoma?
When the growth develops in a concentric pattern around the outside of the bone like layers in an onion
44
How do bone cancers present?
Bone pain Bone swelling Pathological fractures Fever, night sweats, fatigue - more common with maligannt tmors
45
Referral criteria for suspected bone cancer?
Very urgent direct access XR within 48 for children presenting with unexplained bone pain or swelling. If this suggests a possible sarcoma they need very urgent specialist assessment within 48 hours If an XR suggests the possibility of a bone sarcoma in an adult then 2WW
46
Imaging for suspected bone cancer?
Plain XR CT and MRI of bone MRI of whole body Biopsy
47
Why is a whole body MRI so important following a diagnosis of osteosarcoma?
As these often cause skip lesions (Also checks for distant metastasis and stages the tumour as with the other types of bone cancers)
48
Which primary bone cancer has the worst prognosis?
Osteosarcoma - 40% survive 5 years
49
Characteristic presentation of osteochondroma?
A painless palpable mass or bony protuberance near a joint
50
Characteristic presentation of a giant cell tumour?
Localised pain and swelling, commonly at the end of a long bone
51
What is an endochrondroma?
When chondromas occur in the bones of the hands and in the diaphysis/metaphyss of the bone
52
Characteristic presentation of osteosarcoma?
Aggressive and progressive pain Pain at night
53
Characteristic presentation of chondrosarcoma?
Slow growing with dull pain and swelling
54
Characteristic presentation of Ewing’s sarcoma?
Throbbing pain and swelling Most commonly tibia, fibula, clavicle or humerus May be generally unwell with pyrexia, fatigue and weight loss
55
What is a simple bone cyst?
Fluid-filled holes in bones that commonly agent the diaphysis of long bones Affect children and adolescents
56
What is the problem with a simple bone cyst?
They can occasionally cause pathological fractures
57
How do we manage a simple bone cyst?
Some are asymptomatic and small enough to spontaneously heal as the pt reaches skeletal maturity In large cysts decompression is important e.g. curettage, grafting, percutaneous aspiration
58
What are anerysmal bone cysts?
Benign tumours in the bone Affect children more frequently They are hollow and filled with cysts making them fragile and prone to fracture Associated with malignant transformation

MUSCULOSKELETAL (13 decks)

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