Sarcoma Flashcards
What is sarcoma?
Malignant tumour arising from cells of mesenchymal origin
Tumour of connective tissue
Very rare
What cells are of mesenchymal origin?
Bone Cartilage Muscle Synovium Adipose Peripheral nerve sheath
What do you call sarcoma of adipose tissue?
Liposarcoma
What do you call sarcoma of cartilage tissue?
Chondrosarcoma
What do you call sarcoma of bone tissue?
Osteosarcoma
What do you call sarcoma of muscle tissue?
Myosarcoma
Where are sarcomas commonly found?
In the lower extremity, especially the thigh
Where can sarcomas develop?
Anywhere in the body where there is connective tissue, i.e. everywhere!
What are the risk factors for sarcoma?
Prevalence increases in age, but can happen at any age
Genetics: some conditions increase the risk of sarcoma
Radiation
Lymphoedema
Exposure to certain chemicals:
Infections + immunocompromised
Which genetic conditions increase a persons risk of sarcoma?
Li Fraumeni syndrome:
- causes increased risk of several types of cancer
Neurofibromatosis:
- benign tumours form in nerves under skin, increased chance of malignant peripheral nerve sheath tumour
Retinoblastoma:
- childhood inherited eye cancer, 13% of these children develop sarcoma within 50 yrs
What is Li Fraumeni syndrome?
Causes increased risk of several types of cancer in families:
- brain
- breast
- kidney
What is neurofibromatosis?
Formation of benign tumours in nerves just under skin
This increases the risk of developing malignant peripheral nerve sheath tumour
What is retinoblastoma?
Cancer of the retina, affects children
13% of them will get sarcoma by age 50
Which chemicals are linked with an increased risk of sarcoma?
Vinyl chloride (make plastic)
Dioxins (make pesticide)
Chlorophenols (make antiseptic)
Which infections are linked with the development of sarcoma?
Herpes virus 8 causes Kaposi’s sarcoma
Also Kaposi’s sarcoma has links to AIDS
EBV is linked with leiomyosarcoma
What is leiomyosarcoma?
Cancer of smooth muscle
What is angiosarcoma?
Cancer of the inner lining of blood vessels
How do deep sarcomas present?
Slow growing, enlarging mass
Painless
Pressure effects
Weight loss, malaise, rigors
What is the proper name for cutaneous sarcoma?
Dermatofibrosarcoma
How do dermatofibrosarcomas present?
Slow growing
Red brown nodule
Who gets dermatofibrosarcomas ?
Males more often than females
Peak age about 35 years
Metastasis is common in dermatofibrosarcoma.
True or false?
False
Metastasis is rare but recurrence is common
How would you treat a dermatofibrosarcoma?
Wide excision!
What is malignant fibrous histiocytoma?
Cancer of the histiocytes in soft tissue and bone
What are histiocytes?
Cells that are part of the mononuclear phagocyte system
What is Kaposi’s sarcoma?
Cancer that arises from the blood vessels
Commonly affects limbs
What is Ewing’s sarcoma?
A small round tumour of the bone, commonly the pelvis, femur, ribs
Who gets Ewing’s sarcoma?
Teenagers and young adults
Who gets Kaposi’s sarcoma?
Immunocompromised people, those with HIV/AIDs
When should you refer a suspected sarcoma to a specialist centre?
If it is superficial but more the 5cm
If it is deep regardless of size
If you are unsure
Investigation of a sarcoma?
Palpate:
- hard or soft
- fixed or moveable
- pain
Imaging:
- angiography to check if its near blood vessels
- Chest CT: sarcomas often spread to chest
Biopsy:
- check what type, what stage
Where do sarcomas commonly metastasise to?
Lung
Management of sarcoma?
Depends on type
Surgery
Chemo
Radiotherapy
What are the potential problems with surgical excision of sarcoma?
Sarcomas are surrounded by a pseudocapsule
If you disturb this, the cancer will release seeds and spread
It is like breaking a pomegranate, the seeds will spread
