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
What is a common location for leiomyoma
Uterus and fibroids
26
What is a malignant smooth muscle tumour called
Leiomyosarcoma
27
Where does leiomyosarcoma occur
Uterus | Extra uterine sites e.g limbs, retroperitoneum, large vessels
28
What are the causes of leiomyosarcoma
Oestrogen Irradiation Immune supression: EBV related
29
What are the sites that a leiomyosarcoma can occur
``` Large blood vessel Soft tissue GIT Genital tract: uterus Urinary tract: kidney, bladder and prostate ```
30
What are the histological featuers of leiomyosarcoma
Abnormal mitosis | Consists of smooth muscle cells
31
What are the cells of the nerve sheath
Schwann cells | Perineural cells
32
What does schwann cells produce
Myelin to facilitate the impulse
33
What does the perineural cells od
Form the layer around the nerve
34
Which immunohistochemistry is positive for schwann cells
S100 protein
35
Which immunohistochemistry will be posisitve for perineurial cells
Epithelial membrane antigen
36
What is the benign tumour that forms schwann cells called
Schawannoma
37
Where do schwannoma occur
Any peripheral nerves of the body
38
How does the tumour arise i.e what doe sit look like in the nerve
A capsule with a fibrous layer around it
39
Histologically what are the 2 patterns of schwannoma
1. Antoni a | 2. Antoni b
40
What is antoni a
Pink intercellular material of a celullar area
41
What is antoni b
A loose cellular arrangement
42
What is a malignant nerve sheath tumour called
Malignant peripheral nerve sheath tumour
43
Where do malignant peripheral nerve sheath tumours arise from
Nerve Trunk Existing neurofibroma
44
What does immunhistochemistry of malignant peripheral nerve sheath tumour show
Patchy positive s100 protein
45
Name a malignant vascular tumour
Angiosarcoma
46
Where can angiosarcoma occur
In the skin due to long sun exposure
47
What is kaposi sarcoma
Tumour of the blood vessel that is locally destructive with intermediate behaviour
48
What are kaposi sarcoma associated with
HHV8 infection
49
What is an example of small round cell tumour
Ewing sarcoma
50
Where does ewing sarcoma occur
Bone or soft tissue sites
51
What are the types of genetic changes that occur in ewing sarcoma
Translocation with EWSR1 fused with FLI1 or ERG
52
What are the approach to diagnosis in tumours
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
When we take a tissue via biopsy how do we fix it
With formalin
54
What is the first type of stain used
Haemotoxyclin and eosine (H and E)
55
What are the better techniques that allow us to help define tumour types
Immunohistochemistry FISH RT-PCR Next generation sequencing
56
What is immunohistochemistry
Using fluroscent tagged antibodies that bind to anitgen in the tissue if detected
57
What does FISH detect
Gene rearrangement and amplification
58
What does rt-pcr detect
Transcripts of new fusion genes and new mutation in the fusion gene
59
What is grading
The degree of differentiation of the tumour
60
What is staging
The extent of spread of the tumour