SARCOMAS Flashcards

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?

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
Q

Where do Osteoblastomas tend to affect?

A

The axial skeletal e.g. spine and mandible

26
Q

Who do chondrosarcomas most commonly occur in?

A

30-60 year olds
More commonly men

27
Q

What is the difference between primary and secondary chondrosarcomas?

A

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
Q

What can chondrosarcomas sometimes arise from?

A

Pre-existing osteochondromas that transform

29
Q

What are chondrosarcomas?

A

Malignant bone tumours arising from chondriocytes

30
Q

What do chondrosarcomas appear as on XR?

A

Radiolucent area with central flecks of calcification within the medullary cavity
Often described as “moth-eaten cloth”

31
Q

What is an osteosarcoma?

A

A malignant bone tumour arising from osteoblasts

32
Q

Who do osteosarcomas most commonly occur in?

A

Children and adolescents

Can occur in older adults over 60 as a complication

33
Q

Risk factors for osteosarcoma in older adults?

A

Paget’s disease
Pre-existing bone lesions
Radiation exposure

34
Q

Where do osteosarcomas typically affect?

A

Metaphysis of long bones where there is rapid bone growth e.g. femur, proximal tibia and proximal humerus

35
Q

Risk factor for osteosarcoma in children?

A

Retinoblastoma - as pRB is implicated in osteosarcoma

36
Q

XR findings of osteosarcoma?

A

Codman’s triangle
Sunburst pattern

37
Q

What is Codman’s triangle?

A

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
Q

What is a sunburst pattern on XR seen in osteosarcoma?

A

This occurs when lesions grow too fast ans the margins are poorly defined with an aggressive periosteal reaction

39
Q

Who are Ewing’s sarcomas most common in?

A

Children and adolescents

40
Q

What do Ewing’s sarcomas arise from?

A

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
Q

Where do Ewing’s sarcoma most commonly arise from?

A

Most frequently in the long bones and pelvis

42
Q

XR findings of Ewing’s sarcoma?

A

An area of bone destruction
Onion-peel pattern

43
Q

What is the onion skin appearance seen in Ewing’s sarcoma?

A

When the growth develops in a concentric pattern around the outside of the bone like layers in an onion

44
Q

How do bone cancers present?

A

Bone pain
Bone swelling
Pathological fractures

Fever, night sweats, fatigue - more common with maligannt tmors

45
Q

Referral criteria for suspected bone cancer?

A

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
Q

Imaging for suspected bone cancer?

A

Plain XR
CT and MRI of bone
MRI of whole body
Biopsy

47
Q

Why is a whole body MRI so important following a diagnosis of osteosarcoma?

A

As these often cause skip lesions
(Also checks for distant metastasis and stages the tumour as with the other types of bone cancers)

48
Q

Which primary bone cancer has the worst prognosis?

A

Osteosarcoma - 40% survive 5 years

49
Q

Characteristic presentation of osteochondroma?

A

A painless palpable mass or bony protuberance near a joint

50
Q

Characteristic presentation of a giant cell tumour?

A

Localised pain and swelling, commonly at the end of a long bone

51
Q

What is an endochrondroma?

A

When chondromas occur in the bones of the hands and in the diaphysis/metaphyss of the bone

52
Q

Characteristic presentation of osteosarcoma?

A

Aggressive and progressive pain
Pain at night

53
Q

Characteristic presentation of chondrosarcoma?

A

Slow growing with dull pain and swelling

54
Q

Characteristic presentation of Ewing’s sarcoma?

A

Throbbing pain and swelling
Most commonly tibia, fibula, clavicle or humerus
May be generally unwell with pyrexia, fatigue and weight loss

55
Q

What is a simple bone cyst?

A

Fluid-filled holes in bones that commonly agent the diaphysis of long bones
Affect children and adolescents

56
Q

What is the problem with a simple bone cyst?

A

They can occasionally cause pathological fractures

57
Q

How do we manage a simple bone cyst?

A

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
Q

What are anerysmal bone cysts?

A

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