Dermatofibrosarcoma
protuberans.
DFSP – very rare aggressive skin cancer.
Dermatofibrosarcoma Protruberans
DFSP is thankfully very rare.
Dermatofibrosarcoma
What is Dermatofibrosarcoma protuberans?
Dermatofibrosarcoma protuberans (DFSP) is a rare type of cancer of the skin (1 in 100,000 people per year affected).
The condition is so rare that few doctors will ever see a case! The diagnosis will be made by the pathologist when an unusual skin lesion is removed for histology.
Who gets them?
Dermatofibrosarcoma Protuberans tends to occur in younger adults between the ages of 20-50.
If a women has a DFSP and is pregnant, it tends to grow more quickly.
What are the clinical features?
CLUES
The tumour is most commonly seen on the trunk, followed by the shoulder or limbs and least commonly on the face or neck.
The tumour is slow growing and eventually grows outwards from the skin to form multiple nodules that are pink, violet-red or skin coloured. Another presentation is that of a firm scar.
The lesions starts off quite small between 2-5cm however it can be up to 25cm by the time it is discovered and diagnosed. It rarely spreads to other parts of the body.
Gallery of Dermatofibrosarcoma protuberans
Why does DFSP matter?
The good news is that dermatofibrosarcoma protuberans rarely spread to other parts of the body.
Treatment is important, however, to ensure that the cancer does not grow deeper into the fat, muscle or the bone.
Treatment of DFSP is in the remit of the plastic surgeon working with a dermatologist.
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