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Y2 MECHANISMS OF DISEASE > Soft Tissue Tumours > Flashcards

Soft Tissue Tumours Flashcards

1
Q

What is meant by a soft tissue tumour?

What structures tend to be implicated?

A

a tumour of non-bony mesenchymal structures

can involve connective tissue, muscle, fat and blood vessels

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2
Q

What are the 2 types of soft tissue tumours?

What age groups tend to be affected?

A

benign tumours are common

malignant tumours are rare

they occur in all age groups

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3
Q

What inherited conditions can predispose people to getting soft tissue tumours?

A
  1. Gardeners
  2. Li-Fraumeni syndrome
  3. neurofibromatosis
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4
Q

What viruses can predispose people to developing soft tissue tumours?

A
  1. HHV8 predisposes to kaposi’s sarcoma
  2. Epstein-Barr virus predisposes to smooth muscle tumours
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5
Q

How can radiotheraphy affect development of soft tissue tumours?

A

it can lead to angiosarcoma development, usually 10 years after treatment

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6
Q

What are the most common sites for malignant soft tissue tumours?

A

The thigh and retroperitoneum

soft tissue tumours can occur at any site in the body

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7
Q

What are the stages in medical management of soft tissue tumours?

A
  1. history
  2. examination
  3. radiology
  4. biopsy
  5. stage
  6. MDT
  7. treat and follow up
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8
Q

What is the common presentation of a soft tissue tumour?

A

most patients present with a lump

lumps are very common, but sarcomas are rare

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9
Q

What are the clinical clues that a ‘lump’ may be something more sinister?

A
  1. painful
  2. deep to deep fascia (not subcutaneous)
  3. > 5 cm in size
  4. recurrent
  5. enlarging
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10
Q

What are the radiological clues that a lump may be something more sinister?

A
  1. > 5cm
  2. deep to deep fascia
  3. heterogenous appearance
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11
Q

What is the only way to determine whether a soft tissue tumour is malignant or benign?

A

By taking a biopsy

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12
Q

What are the typical endings for a benign and malignant tumour?

A

benign - “oma”

malignant - “sarcoma”

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13
Q

What are the general names for the following tumours?

A
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14
Q

What are the 3 main problems with classifying soft tissue tumours?

A
  1. outdated names - synovial sarcoma is not actually in the synovium
  2. some have no normal tissue counterpart so can only be identified by patterns down the microscope
  3. some are named after the person who discovered them
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15
Q

What are the 3 things that might be carried out on a biopsy?

A
  1. histopathology (looking down a microscope)

this involves immunohistochemistry

  1. cytogenetics to look for chromosomal abnormalities
  2. molecular pathology
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16
Q

What is the purpose of immunohistochemistry?

A

it involves looking for certain proteins in cells to determine which cell type is present

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17
Q

What are the pitfalls when making a differential diagnosis of a soft tissue tumour biopsy?

A
  1. it could be lymphoma/leukaemia
  2. metastases - especially melanoma but also carcinoma and germ cell tumour
  3. reactive pseudotumour
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18
Q

What is shown here?

A

lipoma

19
Q

What is shown here?

A

liposarcoma

mature fat globules are visible within the tumour

20
Q

What is shown here?

A

leiomyoma

this looks like normal smooth muscle cells

21
Q

What is shown here?

A

leiomyosarcoma

necrosis is present

22
Q

What is shown here?

A

small round blue cell tumour

23
Q

What is the differential diagnosis of a small round blue cell tumour?

A

sarcoma:

  • Ewing’s sarcoma
  • neuroblastoma
  • rhabdomyosarcoma

carcinoma:

  • small cell carcinoma e.g. lung

germ cell tumour:

  • embryonal carcinoma

lymphoma

24
Q

What is shown here?

How can it be identified by immunohistochemistry?

A

rhabdomyosarcoma

an antibody to desmin is used, which is a part of muscle

25
Q

What are the 4 main features of a malignant soft tissue tumour?

A
  1. metastases
  2. necrosis
  3. mitoses - abnormal numbers or atypical forms
  4. cytological aytipia (variations in size and shape of the nucleus)
26
Q

What feature of malignancy is shown?

A

abnormal mitoses

27
Q

What feature of malignancy is shown?

A

tumour necrosis

28
Q

What are 2 self limiting lesions that may stimulate sarcoma?

A
  1. nodular fasciitis
  2. myositis ossificans
29
Q

What is myositis ossificans?

A

it occurs in young adults

it is a rapidly growing lump on the limb that is often mistaken for sarcoma

30
Q

How do soft tissue tumours tend to be classified?

A

many soft tissue tumours have characteristic chromosomal changes

cytogenetics and molecular biology can be used to look for chromosome abnormality, fusion RNA or protein expression

31
Q

What is the prognosis for a sarcoma?

A

50% of sarcoma patients are alive at 5 years

32
Q

What are the 3 treatment options for sarcomas?

A
  1. surgery by radical excision - this is the main treatment option
  2. radiotherapy
  3. chemotherapy
33
Q

Where do soft tissue sarcomas tend to spread to?

A

The lungs are the first place that the cancer will spread to

Rarely it spreads to the lymph nodes

34
Q

What is the post-operative pathology involved in sarcoma treatment?

A
  1. confirm the diagnosis
  2. grade using the FNCLCC system (1-3)
  3. look for the margins of the sarcoma
35
Q

Why may radiotherapy be used preoperatively?

A
  1. to downstage the size and extent of the tumour

the tumour may be too large or encompassing too many vital structures

shrinking it makes it operable

36
Q

Why may adjuvant radiotherapy be used post-operatively?

A

to prevent local recurrence

37
Q

What are the 3 main complications associated with adjuvant radiotherapy?

A
  1. fibrosis
  2. fracture to the underlying bone
  3. radiation induced tumours (these are usually sarcomas)
38
Q

When is adjuvant chemotherapy used in addition to surgery?

A
  1. pre-surgery to shrink the tumour
  2. in systemic disease when the cancer has spread
  3. paediatric sarcomas
  4. in GIST
39
Q

Why is chemotherapy often used in paediatric sarcomas?

A

small blue cell tumours are chemoresponsive

this means there is no need for surgery

40
Q

What is the typical treatment for gastrointestinal stromal tumours (GIST)?

A

Imatinib

this is a synthetic tyrosine kinase inhibitor

41
Q

What is GIST?

What causes it?

A

it is a tumour of modified smooth muscle of bowel wall

most cells have a cell membrane receptor CD11 (tyrosine kinase receptor)

this controls cell proliferation and differentiation and is constantly turned on in the tumor

42
Q
A
43
Q
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Y2 MECHANISMS OF DISEASE (36 decks)

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