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Dermatofibrosarcoma protuberans

DERMATOFIBROSARCOMA PROTUBERANS (DFSP)

What are the aims of this leaflet?

This leaflet has been written to help you understand more about dermatofibrosarcoma protuberans (DFSP). It tells you about what it is, what causes it, what can be done about it and where you can find out more about it.

What is DFSP?

DFSP is a very rare type of skin cancer. It usually occurs on the trunk, often the chest and shoulders; however it can also affect the limbs, head and neck. It starts in the deep layer of the skin (the dermis) and can invade deeper tissue such as fat and muscle. Although it grows very slowly, it can become quite large. As the edges are indistinct DFSP can come back even if apparently completely removed by surgery. It can, however, be cured if completely removed with a wide margin of normal tissue, or with a specialised form of surgery called Mohs surgery. DFSP almost never spreads to other parts of the body.

What causes DFSP?

Trauma or injury to the skin may be partly responsible in some cases..

Is DFSP hereditary?

No. It occurs in people of all races and ages, usually in early adult life.

What does DFSP look like?

DFSP is usually a slow growing, painless thickened lumpy area of skin. It can range from skin coloured to pink/brown and occasionally can have a blue appearance. It can also feel like a soft indented area on the skin which can make diagnosis especially difficult. If left for several years DFSP can grow through the top layer of the skin, producing a raw ulcer.

What are the symptoms of DFSP?

Most people with DFSP do not have any symptoms. They may notice a thick or discoloured patch of skin, an indented area or a lump increasing in size.

How is DFSP diagnosed?

As DFSP is so rare, the diagnosis is often delayed. It may be mistaken for common harmless skin conditions such as cysts, dermatofibromas or keloids. If DFSP is suspected, a piece of the abnormal skin will be removed under local anaesthetic (a biopsy) and examined under the microscope to make the diagnosis.

Can DFSP be cured?

Yes, DFSP can be cured if completely removed. However, it can sometimes come back. Regular lifelong follow-up visits to the dermatology clinic are therefore needed.

How can DFSP be treated?

DFSP is treated by surgical excision (removal). Treatment plans for people with DFSP are agreed by members of specialist skin cancer multidisciplinary team. There are two main types of surgery:

A wide excision – this involves removal of the DFSP with a margin of normal skin around the edges to ensure no abnormal cells are left behind. The wound will then be reconstructed (closed) using the most suitable type of surgery. This may involve a skin graft. This may happen under local or general anaesthetic depending on the size and location of the DFSP, and the opinions of the patient and surgeon.

Mohs’ micrographic surgery – this is a specialised surgical technique, performed by a dermatologist, in which the DFSP is surgically removed in stages under local anaesthetic. The abnormal tissue is removed and examined under the microscope overnight. A dressing is placed over the wound until the results are ready, and if there are any remaining abnormal cells this process is repeated until the DFSP has been completely removed. The wound will then either be repaired by the dermatologist or a plastic surgeon, depending on the size and location of the wound. This type of surgery offers a higher cure rate than conventional wide excision.

Extremely rarely, DFSP may spread to other parts of the body. Further treatment may then be required, such as radiotherapy or chemotherapy. This is most likely in the unusual fibrosarcomatous variant of DFSP. Radiotherapy or medication can also be used if the DFSP can’t be removed completely by surgery.

Drug treatment for DFSP

Imatinib is a medicine used for DFSP that cannot be removed with an operation or has spread within the body (a metastatic DFSP). It is taken as a tablet. It does not cause hair loss but can make some people feel sick. Most people, however, can manage it with very few side effects.

What tests do I need?

Most people only need surgery for DFSP. If there are concerns that it may have spread, you may need X-ray, ultrasound, MRI or CT scans. If any lumps develop in the scar, the lump will need to be removed surgically for examination under the microscope.

Self care (what can I do?)

Because of the subtle clinical appearance of DFSP, it can be difficult even for doctors to detect. After having had a DFSP, you should do regular checks of your scar at home; if you have any changes in the scar you should contact your dermatologist.

Where can I get more information about DFSP?

Links to patient support groups:

Macmillan Cancer Support

89 Albert Embankment

London SE1 7UQ

Helpline (for emotional support): 0808 808 2020

Helpline (for information): 0808 800 1234

Website: www.macmillan.org.uk

The Rarer Cancers Foundation

The Great Barn

GodmershamPark

Canterbury

Kent, CT4 7DT

Tel: 0122 773 8279

Helpline: 0800 334 5551

Email: helpline@rarercancers.org.uk

Website: http://www.rarercancers.org.uk

Web links to detailed leaflets:

http://www.dermnetnz.org/lesions/dfsp.html
https://www.aad.org/dermatology-a-to-z/diseases-and-treatments/a---d/der...
http://emedicine.medscape.com/article/1100203-overview

For details of source materials used please contact the Clinical Standards Unit (clinicalstandards@bad.org.uk).

This leaflet aims to provide accurate information about the subject and is a consensus of the views held by representatives of the British Association of Dermatologists: individual patient circumstances may differ, which might alter both the advice and course of therapy given to you by your doctor.

This leaflet has been assessed for readability by the British Association of Dermatologists’ Patient Information Lay Review Panel

BRITISH ASSOCIATION OF DERMATOLOGISTS

PATIENT INFORMATION LEAFLET

PRODUCED MARCH 2012

UPDATED JUNE 2015

REVIEW DATE JUNE 2018

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