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
Q

Who is most commonly affected by chondrosarcoma?

A

These are most common in adults over 40, except clear cell and mesenchymal, which affect younger patients

26
Q

Where do chondrosarcomas most commonly affect?

A

Axial skeleton (Most commonly head and neck)

27
Q

Where do clear cell chondrosarcomas most commonly affect?

A

Epiphysis of long tubular bones

28
Q

What are the histological subtypes of chondrosarcoma?

A
  • Conventional: Intramedullary (Central) or juxtacortical (peripheral)
  • Clear cell
  • Mesenchymal
  • Dedifferentiated
29
Q

How are chondrosarcomas graded?

A

They are graded from 1 to 3, depending on cellularity, cytological atypia and mitotic activity

30
Q

Describe the histology of conventional chondrosarcomas?

A

They are bulky tumours forming nodules of grey cartilaginous tissue with gelatinous matrix

They locally invade bone, muscle and fat

31
Q

Describe the histology of de-differentiated chondrosarcomas

A

These are low grade chondrosarcomas with a separate high grade component that does not produce cartilage

32
Q

Describe the histology of clear cell chondrosarcomas

A

Histologically, malignant chondrocytes have abundant clear cytoplasm, associated osteoclastic cells and reactive bone that can suggest osteosarcoma

33
Q

Describe the histology of mesenchymal chondrosarcomas

A

Histologically, these show sheets of well differentiated hyaline appearing cartilage with surrounding small round cells

34
Q

What is the most common method and site of metastasis in chondrosarcoma?

A

Haematogeous spread to the lungs

35
Q

What are osteosarcomas?

A

These are malignant lesions that produce bone, most commonly found in the primary sarcoma of the bone

36
Q

Who is most at risk of developing osteosarcomas?

A

Paediatric group
Elderly (Associated with Paget’s disease)

37
Q

Where do osteosarcomas usually affect?

A

Long bones

38
Q

How are osteosarcomas diagnosed?

A

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
Q

How may osteosarcomas metastasise?

A

Metastatic spread is usually haematogenous but can be lymphatic

10-20% of patients have pulmonary metastases at diagnosis

40
Q

What are Ewing’s sarcomas?

A

They are destructive, rapidly growing and highly malignant tumours of unknown origin that can affect any soft tissue or bony location

41
Q

Who is most at risk of Ewing’s sarcoma?

A

Children and adolescents (Ages 10-20)

42
Q

What are the supposed cells of origin in Ewing’s sarcoma?

A

Primitive mesenchymal cells or neuroectodermal cells

43
Q

Describe the histology of Ewing’s sarcoma

A

They form small round blue cell tumours

44
Q

How will Ewing’s sarcoma present?

A

This will often present with hot, swollen, tender joints or limbs with raised inflammatory markers

They can often mimic infection

45
Q

What are sarcomatoid carcinomas?

A

Sarcomatoid carcinomas are thought to be caused by pluripotential stem cells that become malignant

46
Q

What are fibrosarcomas?

A

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
Q

What are the most common origins of bony metastases?

A

Lungs
Kidneys
Breasts
Prostate

48
Q

What is meant by osteolytic?

A

Tumours that resorb bone

49
Q

What is meant by osteosclerotic?

A

Tumours that cause fibrosis of bone

50
Q

How will osteolytic bone tumours present on X-ray?

A

Radiolucency

51
Q

How will osteosclerotic bone tumours present on X-ray

A

Radio opaque