Soft tissue tumours

Question 1

T or F? Benign tumours are common in soft tissues.

Answer 1

True

Question 2

What percentage of cancers is malignancy of soft tissues?

Answer 2

1%

Question 3

How many different soft tissue tumours are there?

Answer 3

Over 100

Question 4

Where do soft tissue tumours derive from?

Answer 4

Mesenchyme :

fat, muscle, connective tissue, blood vessel, nerve sheath

Question 5

What are some examples of benign mesenchymal tumours?

Answer 5

Sarcoma

Lipoma

Leiomyoma (fibroids)

Schwanoma

Neuroma

Small round tumours

Osteoma

Fibroma

Fibrolipoma

Question 6

What are some examples of malignant soft tissue tumours?

Answer 6

Osteosarcoma (Codman’s triangle and sunburst)

Ewing’s sarcoma (11:22, onion peel, periosteal reaction)

Leiomyosarcoma (Well Differentiated , myxoma leimyosarcoma) (oestrogen, EBV)

MPNST (malignant peripheral nerve sheath tumour)

Angiosarcoma

Question 7

What are the two types of leiomyosarcoma?

Answer 7

Well differentiated/ WD = most common

Myxoma liposarcoma = 2nd most common

Question 8

What are leiomyomas?

Answer 8

Smooth muscle tumours (fibroids particularly common)

Question 9

What stain can be used for schwannoma?

Answer 9

S100

Question 10

What does MPNST stand for?

Answer 10

malignant peripheral nerve sheath tumour

Question 11

What is angiosarcoma?

Answer 11

Angiosarcoma is a rare cancer that develops in the inner lining of blood vessels and lymph vessels.

Can occur anywhere

Question 12

What is small round cell tumoir?

Answer 12

Desmoplastic small-round-cell tumor (DSRCT) is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord,

Question 13

What is osteosarcoma?

Answer 13

Osteosarcoma is a type of bone sarcoma that usually develops in growing bones. It is most often found in teenagers and young adults. But it can happen at any age. About 1 in 10 osteosarcomas (10%) develop in adults in their 60s and 70s. It is slightly more common in men than women.

Osteosarcoma can affect any bone. But the most common areas are the arms or legs (often near the knee joint).

Question 14

What is Ewings sarcoma?

Answer 14

EWSR1-FLI1 (85%) aggressive

More common in limbs and teenagers

14% of sarcomas

Question 15

How do you confirm osteosarcoma on an X ray?

Answer 15

Codman’s triangle and sunburst pattern

Question 16

How do you confirm Ewings on an X ray?

Answer 16

Periosteal reaction creating onion layers

Question 17

What is the mutation for Ewings?

Answer 17

ESWR1 - FLI1 translocation (85%)