Malignant MSK tumours Flashcards

1
Q

What is a malignant tumour of fat known as?

A

Liposarcoma

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2
Q

What is a malignant tumour of blood vessels known as?

A

Angiosarcoma

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3
Q

What is a malignant tumour of smooth muscle known as?

A

Leiomyosarcoma

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4
Q

What is a malignant tumour of skeletal or cardiac muscle known as?

A

Rhabdomyosarcoma

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5
Q

What is a malignant tumour of cartilage known as?

A

Chondrosarcoma

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6
Q

What is a malignant tumour of bone known as?

A

Osteosarcoma

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7
Q

Who is most at risk of developing liposarcoma?

A

Ages 50 - 60

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8
Q

What are the 3 histological sub-types of liposarcoma?

A
  • Well-differentiated
  • Myxoid
  • Pleomorphic
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9
Q

Where do liposarcomas most commonly form?

A

Deep soft tissue of extremities or retroperitoneum

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10
Q

Who is most at risk of developing leiomyosarcoma?

A

Females

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11
Q

Where do leiomyosarcomas most commonly form?

A

Deep soft tissues of the extremities and retroperitoneum
Great vessels (e.g. IVC)

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12
Q

What are some complications of leiomyosarcoma?

A

These can be lethal due to local invasion and metastasis, most commonly via haematogenous spread to the lungs

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13
Q

What are the 3 categories of rhabdomyosarcoma?

A
  • Embryonal
  • Alveolar
  • Pleomorphic
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14
Q

How should rhabdomyomas be treated?

A

They are aggressive tumours so require surgery and adjuvant therapy

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15
Q

When do embryonal rhabdomyosarcomas occur?

A

During early childhood

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16
Q

What are some structures affected by embryonal rhabdomyosarcoma?

A
  • Genital tract (Botyroides)
  • GU tract
  • Head and Neck (Periorbital)
  • Common bile duct
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17
Q

What are botyroides?

A

Embryonic rhabdomyosarcomas of the genital tract

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18
Q

Describe the histology of boytryoides?

A

Botyroides forms have cambium layers, in which there is condensation of the tumour cells below the mucosal surface

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19
Q

Who is most commonly affected by alveolar rhabdomyosarcomas?

A

Young adults

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20
Q

What are the most common sites affected by alveolar rhabdomyosarcoma?

A

Head and neck

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21
Q

What gene mutations are associated with alveolar rhabdomyosarcoma?

A

FOXO1 (PAX 3 or 7 mutations)

22
Q

Who is most commonly affected by pleomorphic rhabdomyosarcoma?

A

Older age groups

23
Q

What will immunohistochemistry show in pleomorphic rhabdomyosarcoma?

A

MYOD1
Myogenin

24
Q

What are chondrosarcomas?

A

These are a malignant tumour that produces cartilage

25
Who is most commonly affected by chondrosarcoma?
These are most common in adults over 40, except clear cell and mesenchymal, which affect younger patients
26
Where do chondrosarcomas most commonly affect?
Axial skeleton (Most commonly head and neck)
27
Where do clear cell chondrosarcomas most commonly affect?
Epiphysis of long tubular bones
28
What are the histological subtypes of chondrosarcoma?
- Conventional: Intramedullary (Central) or juxtacortical (peripheral) - Clear cell - Mesenchymal - Dedifferentiated
29
How are chondrosarcomas graded?
They are graded from 1 to 3, depending on cellularity, cytological atypia and mitotic activity
30
Describe the histology of conventional chondrosarcomas?
They are bulky tumours forming nodules of grey cartilaginous tissue with gelatinous matrix They locally invade bone, muscle and fat
31
Describe the histology of de-differentiated chondrosarcomas
These are low grade chondrosarcomas with a separate high grade component that does not produce cartilage
32
Describe the histology of clear cell chondrosarcomas
Histologically, malignant chondrocytes have abundant clear cytoplasm, associated osteoclastic cells and reactive bone that can suggest osteosarcoma
33
Describe the histology of mesenchymal chondrosarcomas
Histologically, these show sheets of well differentiated hyaline appearing cartilage with surrounding small round cells
34
What is the most common method and site of metastasis in chondrosarcoma?
Haematogeous spread to the lungs
35
What are osteosarcomas?
These are malignant lesions that produce bone, most commonly found in the primary sarcoma of the bone
36
Who is most at risk of developing osteosarcomas?
Paediatric group Elderly (Associated with Paget's disease)
37
Where do osteosarcomas usually affect?
Long bones
38
How are osteosarcomas diagnosed?
They are diagnosed using radiography, showing signs such as Codman’s triangle, in which the reactive bone lifts the periosteum up, forming a triangle Any malignant tumour that produces osteoid is an osteosarcoma until proven otherwise
39
How may osteosarcomas metastasise?
Metastatic spread is usually haematogenous but can be lymphatic 10-20% of patients have pulmonary metastases at diagnosis
40
What are Ewing's sarcomas?
They are destructive, rapidly growing and highly malignant tumours of unknown origin that can affect any soft tissue or bony location
41
Who is most at risk of Ewing's sarcoma?
Children and adolescents (Ages 10-20)
42
What are the supposed cells of origin in Ewing's sarcoma?
Primitive mesenchymal cells or neuroectodermal cells
43
Describe the histology of Ewing's sarcoma
They form small round blue cell tumours
44
How will Ewing's sarcoma present?
This will often present with hot, swollen, tender joints or limbs with raised inflammatory markers They can often mimic infection
45
What are sarcomatoid carcinomas?
Sarcomatoid carcinomas are thought to be caused by pluripotential stem cells that become malignant
46
What are fibrosarcomas?
These are fibrous malignant primary bone tumours which tend to occur in abnormal bone e.g. bone infarct, post radiation Fibrosarcoma tends to affect adolescents/YAs
47
What are the most common origins of bony metastases?
Lungs Kidneys Breasts Prostate
48
What is meant by osteolytic?
Tumours that resorb bone
49
What is meant by osteosclerotic?
Tumours that cause fibrosis of bone
50
How will osteolytic bone tumours present on X-ray?
Radiolucency
51
How will osteosclerotic bone tumours present on X-ray
Radio opaque