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Z - Cancer Care Old > Bone Cancer and Sarcoma > Flashcards

Bone Cancer and Sarcoma Flashcards

1
Q

What are sarcomas?

A

Tumours of the body’s supportive connective tissue

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2
Q

What can sarcomas arise from?

A
  • Bone
  • Muscle
  • Cartilage
  • Tendon
  • Fat
  • Synovial tissue
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3
Q

What % of cancers do sarcomas account for?

A

Less than 1%

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4
Q

What kind of sarcomas develop primarily in children and adolescents?

A
  • Ewing’s sarcoma

- Osteosarcoma

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5
Q

What does the occurrence of Ewing’s sarcoma and osteosarcoma appear to correlate with?

A

The growth spurt

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6
Q

Are soft tissue sarcomas common in adults?

A

No, they are rare in adults

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7
Q

What % of childhood cancers do soft tissue sarcomas account for?

A

Approx. 6%

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8
Q

What is the aetiology of bone sarcomas?

A

For the majority of bone sarcomas, no specific aetiology has been established, although predisposing factors have been identified

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9
Q

What group has an increased incidence of osteosarcoma?

A

Children with familial retinoblastoma

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10
Q

What is the genetic abnormality in children with familial retinoblastoma?

A

13q chromosome deletion

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11
Q

What are radiation-associated sarcomas?

A

Sarcomas that develop within a radiation field

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12
Q

When do radiation-associated sarcomas develop?

A

Usually after a latent period of at least 3 years

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13
Q

Of what type are the majority of radiation-induced sarcomas?

A

Osteosarcomas

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14
Q

What is osteosarcoma often associated with in adults?

A
  • Underlying bone abnormality, such as Paget’s disease or fibrous dysplasia
  • Where the bone has been irradiated previously
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15
Q

What genetic association is there with soft tissue sarcomas?

A
  • Neurofibromatosis type 1
  • Hereditary retinoblastoma
  • Li-Fraumeni syndrome
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16
Q

What exposures increases the risk of soft tissue sarcomas?

A
  • Previous radiation therapy
  • Exposure to chemicals such as vinyl chloride monomers
  • Herbicides
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17
Q

Where does Ewing’s sarcoma typically arise from?

A

Axial skeleton

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18
Q

What genetic mutation is Ewing’s sarcoma associated with?

A

t(11;22) chromosomal translocation

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19
Q

How does a t(11;22) chromosomal translocation increase the risk of Ewing’s sarcoma?

A

It juxtaposes the EWS and FL11 genes, producing a hybrid transcript that is able to act as a master regulatory protein

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20
Q

Where do most osteosarcomas occur?

A

In the metaphyseal region of growing long bones, such as the distal femur, proximal tibia, and proximal humerus

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21
Q

What is Ewing’s sarcoma classically described as?

A

Classically a diaphyseal lesion

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22
Q

Does Ewing’s sarcoma always arise in the diaphysis?

A

No, it may arise from any region within an involved long bone

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23
Q

Where does Ewing’s sarcoma commonly arise?

A
  • Flat bones of pelvis

- Scapula

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24
Q

Where are primary bone tumours of any histological subtypes extremely rare?

A
  • Spine

- Sacrum

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25
Q

How do bone sarcomas typically present?

A

Localised pain

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26
Q

What course does the localised pain in bone sarcomas take?

A

Initially is insidious and transient, but worsens progressively

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27
Q

What other presenting features might be present with bone sarcomas?

A
  • Localised soft tissue swelling, with or without associated erythema
  • Joint effusions
  • Decreased range of movement of adjacent joints
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28
Q

What might exacerbate the local symptoms of bone sarcomas?

A

Movement or weight bearing of the involved extremity

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29
Q

Are regional lymph nodes involved in bone sarcomas?

A

They are rarely involved

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30
Q

What constitutional symptoms may be present with bone sarcomas?

A

Constitutional symptoms are rare, however fever, malaise, and weight loss can be seen with Ewing’s sarcoma

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31
Q

How do soft tissue sarcomas usually present?

A

With painless soft tissue swelling

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32
Q

How might soft tissue sarcomas present if located within a body cavity?

A
  • Pain

- Symptoms associated with pressure exerted on nearby structures

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33
Q

How might gastrointestinal stromal tumours present?

A
  • Haematemesis
  • Blood in faeces
  • Anaemia
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34
Q

How might uterine sarcomas present?

A
  • Vaginal bleeding

- Lower abdominal pain

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35
Q

What age does alveolar rhabdomyosarcoma present?

A

10-20

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36
Q

What are the most common sites of alveolar rhabdomyosarcoma?

A

Thigh

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37
Q

What is the primary therapy for alveolar rhabdomyosarcoma?

A

Neoadjuvant chemoradiation and surgery

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38
Q

What is the overall 5 year survival of alveolar rhabdomyosarcoma?

A

60%

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39
Q

What age does angiosarcoma present?

A

50-70

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40
Q

What are the most common sites of angiosarcoma?

A
  • Skin

- Superficial soft tissues

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41
Q

What is the primary therapy of alveolar rhabdomyosarcoma?

A

Wide excision and adjuvant radiation

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42
Q

What is the overall 5 year survival of angiosarcoma?

A

15%

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43
Q

What age does embryonal rhabdomyosarcoma present?

A

0-10

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44
Q

What are the most common sites of embryonal rhabdomyosarcoma?

A
  • Head and neck

- Genitourinary

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45
Q

What is the primary therapy for embryonal rhabdomyosarcoma?

A

Neoadjuvant chemotherapy and surgery

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46
Q

What is the overall 5 year survival for embryonal rhabdomyosarcoma?

A

40%

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47
Q

What age does fibrosarcoma present?

A

20-50

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48
Q

What are the most common sites of fibrosarcoma?

A
  • Thigh
  • Arm
  • Head and neck
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49
Q

What is the primary therapy for fibrosarcoma?

A

Wide excision and adjuvant radiotherapy

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50
Q

What is the 5 year survival for well differentiated fibrosarcoma?

A

90%

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51
Q

What is the 5 year survival for poorly differentiated fibrosarcoma?

A

50%

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52
Q

What age does leiomyosarcoma present?

A

45-65

53
Q

What are the most common sites of leiomyosarcoma?

A
  • Retroperitoneal

- Uterine

54
Q

What is the primary therapy for leiomyosarcoma?

A

Wide excision and adjuvant radiation

55
Q

What is the overall 5 year survival of leiomyosarcoma?

A

40%

56
Q

What age does liposarcoma present?

A

40-60

57
Q

What are the most common sites of liposarcoma?

A
  • Thigh

- Head and neck

58
Q

What is the primary therapy for liposarcoma?

A

Wide excision and adjuvant radiation

59
Q

What is the 5 year survival for myxoid liposarcoma?

A

66%

60
Q

What is the 5 year survival for pleomorphic liposarcoma?

A

10%

61
Q

What age does pleomorphic rhabdomyosarcoma present?

A

40-70

62
Q

What are the most common sites of pleomorphic rhabdomyosarcoma?

A
  • Thigh

- Upper arm

63
Q

What is the primary therapy for pleomorphic rhabdomyosarcoma?

A

Wide excision and adjuvant radiation

64
Q

What is the overall 5 year survival of pleomorphic rhabdomyosarcoma?

A

10%

65
Q

What is the age of onset of synovial sarcoma?

A

20-40

66
Q

What is the most common site of synovial sarcoma?

A

Leg

67
Q

What is the primary therapy for synovial sarcoma?

A

Wide excision and adjuvant radiation

68
Q

What is the overall 5-year survival of synovial sarcoma?

A

40%

69
Q

What is rhabdomyosarcoma?

A

A sarcoma of skeletal muscle

70
Q

What are the symptoms of rhabdomyosarcoma?

A

Visible, enlarging mass

71
Q

What is Kaposi’s sarcoma associated with?

A

HHV8 infection

It is an AIDS-defining illness

72
Q

What are the symptoms of gastrointestinal stromal tumours?

A
  • Haemoptysis
  • Blood in faeces
  • Symptoms of anaemia
73
Q

What is chondrosarcoma?

A

Sarcoma of cartilage tissue

74
Q

Where do chondrosarcomas arise?

A

Bones in pelvis, shoulder, and limbs

75
Q

What are the symptoms of chondrosarcoma?

A
  • Painful bony swellings

- Pathological fractures

76
Q

What is liposarcoma?

A

Sarcoma of fat tissue

77
Q

What are the symptoms of liposarcoma?

A

Enlarging deep-seated mass in soft tissue

78
Q

Where are liposarcomas found?

A
  • Thigh

- Retroperitoneum

79
Q

What is angiosarcoma?

A

Sarcoma of blood vessels

80
Q

What are the symptoms of angiosarcoma?

A

Painful swelling

81
Q

What are gastric leiomyosarcomas?

A

Sarcomas of the smooth muscle cells in the stomach

82
Q

What are the symptoms of gastric leiomyosarcoma?

A
  • Early satiety
  • Weight loss
  • Abdominal pain
83
Q

What is an endometrial stromal sarcoma?

A

Sarcoma of the connective tissue cells in the endometrium

84
Q

What are the symptoms of endometrial stromal sarcoma?

A
  • Passing blood PV

- Lower abdominal pain

85
Q

What is osteosarcoma?

A

Sarcoma of bone

86
Q

What are the symptoms of osteosarcoma?

A
  • Painful bony swelling
  • Overlying erythema
  • Pathological fractures
87
Q

Are bone sarcomas visible on plain x-ray?

A

Many are

88
Q

What is the purpose of MRI in bone sarcomas?

A

It is required to assess the extent of lesions as well as the relationship of tumours to nearby structures such as nerves, blood vessels, and joint

89
Q

What features of bone sarcomas are best demonstrated by MRI?

A
  • Intermedullary tumour extent

- Presence of skip metastases within the bone

90
Q

What might an isotope bone scan be useful for in bone sarcoma?

A

Detecting distant osseous metastases

91
Q

Are there any tumour markers for bone sarcomas?

A

There are no useful tumour markers, but alkaline phosphatase may be raised in some cases

92
Q

Is a biopsy required in bone sarcoma?

A

Yes

93
Q

Who should a biopsy be undertaken by in bone sarcoma?

A

A specialist orthopaedic oncologist

94
Q

Why is it important that the biopsy is taken by a specialised orthopaedic oncologist in bone sarcoma?

A

To avoid seeding of the needle tract, which could compromise future curative therapy

95
Q

What is the investigation of choice for soft tissue sarcomas?

A

MRI

96
Q

What can identify lung metastases in soft tissue sarcomas?

A

Chest x-ray or chest CT

97
Q

Why is it important to identify lung metastases in soft tissue sarcomas?

A

As these tumours will metastasise by blood-borne spread to the lung

98
Q

Is biopsy required in soft tissue sarcomas?

A

Yes

99
Q

What techniques can be used to obtain a biopsy in soft tissue sarcomas?

A
  • Open biopsy

- Image-guided biopsy

100
Q

What samples will patients with Ewing’s sarcoma require?

A
  • Biopsy
  • Bone marrow aspirate
  • Trephine biopsy from distant sites
101
Q

What is the purpose of a trephine biopsy from distant sites in Ewing’s sarcoma?

A

To check for metastasis

102
Q

What blood test findings are often present with Ewing’s sarcoma?

A
  • Anaemia
  • Elevated ESR
  • Elevated CRP
  • Elevated LDH
103
Q

What treatment should bone sarcomas be considered for rather than amputation?

A

Limb-sparing surgery with endoprosthetic replacement of the resected bone

104
Q

What is a good prognostic factor for bone sarcoma when surgery is combined with adjuvant or neoadjuvant chemotherapy?

A

Positive response to chemotherapy

105
Q

What must be ensured when surgery is being used in the management of bone sarcomas?

A

That the entire local tumour is removed, with wide clear margins

106
Q

What % of osteosarcoma surgery is limb sparing?

A

Approx 95%

107
Q

How can muscle be reconstructed after bone sarcoma surgery?

A

Using transfers from other parts of the body

108
Q

What is the goal of using muscle transfers in bone sarcoma surgery?

A

To produce a functioning limb

109
Q

When can radiotherapy be used for bone sarcomas?

A
  • Non-resectable tumours

- Palliation of bone metastases

110
Q

How can patients with metastatic bone sarcomas be managed?

A
  • Palliative chemotherapy
  • Radiotherapy
  • Amputation
111
Q

What is the purpose of the management of metastatic bone sarcomas?

A

Symptom control

112
Q

What management is required for patients with soft tissue sarcomas?

A

Surgery

113
Q

What is the aim of surgery in soft tissue sarcomas?

A

Complete excision of the tumour with clear margins

114
Q

What might be required to achieve complete excision of the tumour with clear margins in soft tissue sarcomas?

A

En bloc dissection with removal of the muscular compartments

115
Q

What is the purpose of en bloc dissection in soft tissue sarcomas?

A

It gives the lowest risk of local recurrence

116
Q

In what % of cases of soft tissue sarcomas is limb amputation necessary?

A

5%

117
Q

What treatment options are of limited use in soft tissue sarcomas?

A

Adjuvant or neoadjuvant chemotherapy or radiotherapy

118
Q

What management do GIST respond to?

A

Tyrosine kinase inhibitors such as imatinib

119
Q

What is the advantage of tyrosine kinase inhibitors in GIST?

A

They may significantly increase the median survival time in patients with disease unresponsive to conventional chemotherapy

120
Q

What does the treatment of Ewing’s sarcoma require?

A

A multi-disciplinary approach

121
Q

What does the treatment of Ewing’s sarcoma usually consist of?

A

Chemotherapy followed by surgical excision and then further chemotherapy

122
Q

What is the 5 year survival rate of localised bone sarcomas?

A

60-70`5

123
Q

What is the 5 year survival rate of metastatic bone sarcoma?

A

10-30%

124
Q

What is the 5 year survival rate of soft tissue sarcomas?

A

70%

125
Q

What is required with survivors of soft tissue sarcomas?

A

Careful monitoring

126
Q

Why is careful monitoring required with survivors of soft tissue sarcomas?

A

As patients with solitary pulmonary metastasis can be cured by surgical resection

127
Q

What is the 5 year survival rate of localised Ewing’s sarcoma?

A

55-60%

128
Q

What is the 5 year survival rate of metastatic Ewing’s sarcoma?

A

10-20%

Z - Cancer Care Old (28 decks)

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