Pathology Of Soft Tissue Tumours Flashcards

1
Q

What are soft tissue tumours

A

Tumours forming mesenchymal tissue

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2
Q

What are mesenchymal tissue examples

A
Fat
Muscle 
Connective tissue 
Blood vessel
Nerve sheath
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3
Q

What are tumours that do not form recognisable tissue called

A

Undifferentiated tumours

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4
Q

What are the common sites for soft tissue tumours

A

Skin
Subcutis
Deep soft tissue
Organ

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5
Q

What can the classification of soft tissue tumours be by

A

Diffiferentiation

By behaviour

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6
Q

What is the classification of soft tissue tumours by differentiation

A

If the tumour is:

  • adipocytic
  • fibroblastic
  • msooth or skeletal muscle
  • perivascular
  • neural
  • GIST
  • chondro-osseous
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7
Q

What is the classification of soft tissue tumours in behaviour like

A
  • benign
  • intermediate
  • malignant
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8
Q

What is a intermediate soft tissue tumour

A

It is a tumour that is locally aggresssive by rarerly metasising

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9
Q

What are the problems with soft tissue tumour classification

A
  • many soft tissue are of uncertain linegae
  • difficult to predict behaviour
  • soft tissue arise by genetic changes and not from a differentiated cell
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10
Q

What are the causual associations of soft tissue tumours

A
Hereditary predisposition e.g li fraumeni syndrome 
Viral infections 
Irradiation, solar 
Irradiation, therapeutic
Chemical agents 
Lymphoedema
Acquired molecular abnormalities
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11
Q

What are the 3 groups of acquired molecular abnormalities

A
  1. Translocations
  2. Non translocation- usually affecting single genes
  3. Complex unbalanced karyoptype
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12
Q

What are chromosomal translocations

A

When 2 proportions of chromosome break off and swap over to form a fusion gene

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13
Q

What does the fusion gene form

A

Oncogene

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14
Q

What are non translocations abnormalities

A

Somatic mutations

Copy number abnormalities: gene amplification and gene deletion

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15
Q

What is the most common soft tissue tumour

A

Lipoma (adipocytic tumour)

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16
Q

What does a lipoma represent

A

Slow growing benign fat

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17
Q

What is a malignant fat soft tissue tumour called

A

Liposarcoma

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18
Q

What are the 2 subtypes of liposarcoma

A

Well differentiated liposarcoma

Myxoid liposarcoma

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19
Q

What is a well differentiated liposarcoma look like

A

Fat, scattered cells with enlarged darkly stained nuclei

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20
Q

What is the genetic mechanism of well differentiated liposarcoma

A

Amplification of the long arm chromosome 12 which contains MDM2 and CDK4 which is involved in the cell cycle

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21
Q

What is the mechanism of myxoid liposarcoma

A

Translocation with fusiomn of DDIT3 and FUS or EWSR1 gene

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22
Q

What is the histological appearance of myoxid liposarcoma

A

Stroma with loos pale secretions and vasculated cytoplasm

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23
Q

What is the common location of liposarcoma

A

Lower extremities

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24
Q

What is an example of a benign smooth muscle tumour

A

Leiomyoma

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25
Q

What is a common location for leiomyoma

A

Uterus and fibroids

26
Q

What is a malignant smooth muscle tumour called

A

Leiomyosarcoma

27
Q

Where does leiomyosarcoma occur

A

Uterus

Extra uterine sites e.g limbs, retroperitoneum, large vessels

28
Q

What are the causes of leiomyosarcoma

A

Oestrogen
Irradiation
Immune supression: EBV related

29
Q

What are the sites that a leiomyosarcoma can occur

A
Large blood vessel
Soft tissue
GIT
Genital tract: uterus 
Urinary tract: kidney, bladder and prostate
30
Q

What are the histological featuers of leiomyosarcoma

A

Abnormal mitosis

Consists of smooth muscle cells

31
Q

What are the cells of the nerve sheath

A

Schwann cells

Perineural cells

32
Q

What does schwann cells produce

A

Myelin to facilitate the impulse

33
Q

What does the perineural cells od

A

Form the layer around the nerve

34
Q

Which immunohistochemistry is positive for schwann cells

A

S100 protein

35
Q

Which immunohistochemistry will be posisitve for perineurial cells

A

Epithelial membrane antigen

36
Q

What is the benign tumour that forms schwann cells called

A

Schawannoma

37
Q

Where do schwannoma occur

A

Any peripheral nerves of the body

38
Q

How does the tumour arise i.e what doe sit look like in the nerve

A

A capsule with a fibrous layer around it

39
Q

Histologically what are the 2 patterns of schwannoma

A
  1. Antoni a

2. Antoni b

40
Q

What is antoni a

A

Pink intercellular material of a celullar area

41
Q

What is antoni b

A

A loose cellular arrangement

42
Q

What is a malignant nerve sheath tumour called

A

Malignant peripheral nerve sheath tumour

43
Q

Where do malignant peripheral nerve sheath tumours arise from

A

Nerve
Trunk
Existing neurofibroma

44
Q

What does immunhistochemistry of malignant peripheral nerve sheath tumour show

A

Patchy positive s100 protein

45
Q

Name a malignant vascular tumour

A

Angiosarcoma

46
Q

Where can angiosarcoma occur

A

In the skin due to long sun exposure

47
Q

What is kaposi sarcoma

A

Tumour of the blood vessel that is locally destructive with intermediate behaviour

48
Q

What are kaposi sarcoma associated with

A

HHV8 infection

49
Q

What is an example of small round cell tumour

A

Ewing sarcoma

50
Q

Where does ewing sarcoma occur

A

Bone or soft tissue sites

51
Q

What are the types of genetic changes that occur in ewing sarcoma

A

Translocation with EWSR1 fused with FLI1 or ERG

52
Q

What are the approach to diagnosis in tumours

A
  1. Clinical features
  2. Gross findings with naked eye
  3. History
  4. Applying ancillary techniques such as immunohistochemistry or molecular genetics or electron microscopy
53
Q

When we take a tissue via biopsy how do we fix it

A

With formalin

54
Q

What is the first type of stain used

A

Haemotoxyclin and eosine (H and E)

55
Q

What are the better techniques that allow us to help define tumour types

A

Immunohistochemistry
FISH
RT-PCR
Next generation sequencing

56
Q

What is immunohistochemistry

A

Using fluroscent tagged antibodies that bind to anitgen in the tissue if detected

57
Q

What does FISH detect

A

Gene rearrangement and amplification

58
Q

What does rt-pcr detect

A

Transcripts of new fusion genes and new mutation in the fusion gene

59
Q

What is grading

A

The degree of differentiation of the tumour

60
Q

What is staging

A

The extent of spread of the tumour