Dermatofibrosarcoma protuberans (DFSP) | Sarcoma UK
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Dermatofibrosarcoma protuberans (DFSP)

DFSP is a rare tumour that tends to develop from the cells in the middle layer of the skin, called the dermis.

It may also involve the fatty cells of the bottom layer of skin called the subcutis. In rare cases, it can also involve the muscles and connective tissue near the skin.

DFSP can occur anywhere in the body, but it most often occurs in the torso. It also often occurs in the arms, legs, head and neck.

It tends to be associated with young to middle-aged adults who are between 20-50 years old, but it can occur at any age. DFSP affects slightly more males than females.

DFSP is thought to be caused when someone has extra chromosomes, or when someone has certain chromosomes that have been broken apart and put back together in the wrong way. Researchers are still trying to find out why these things happen and whether they cause DFSP.

DFSP tends to grow slowly, and very rarely spreads to other parts of the body. But, it is important to get correct treatment as soon as possible. Without the correct treatment, DFSP can grow deep into the fat, muscle and bone, which makes it more difficult to treat.


There are an average of 147 cases of DFSP diagnosed every year in England.

DFSP makes up 3.73% of all soft tissue sarcomas, and about 0.05% of all cancers.

Although DFSP can affect anyone of any age, the median age at diagnosis is 44 years old.

There are slightly more females than males diagnosed with DFSP.

Signs and symptoms

Symptoms of DFSP can vary depending on the size and location of your tumour. You may experience all, some or none of these symptoms before you are diagnosed with DFSP:

  • A slow growing and often painless lump under the skin
  • A scar or a deep-seated pimple on the skin, that may easily crack open or bleed
  • A lump with the skin over it feeling soft and indented
  • Skin that is pink to brown in colour and sometimes may appear in shades of purple (it may look like a bruise).

If DFSP is left for several years, it can grow through the top layer of the skin and develop into an ulcer.


A specialist doctor will diagnose DFSP through a series of tests. These may include:

  • Physical examination – looking at and feeling any lump
  • A skin biopsy – taking and testing a sample of the tumour on the skin. Using this biopsy, a doctor will look to see if the biopsy looks like DFSP
  • A scan – taking pictures of the inside of the body using scans such as MRI scans.

To read more about these scans and tests, click here.

Who will treat me?

If you have been diagnosed with DFSP, you should be referred to a specialist sarcoma multidisciplinary team (MDT) for treatment. You should also be seen by skin cancer specialists, dermatologists and plastic surgeons, who can offer specialised treatments such as Mohs surgery.
There should be a formal relationship between your sarcoma MDT and your skin cancer MDT.


In most cases, surgery is used to treat DFSP. The surgeon will remove the tumour and will aim to take out an area of normal tissue too – this is known as “taking a margin”. This is to make sure that all the cancer has been removed.

There is a risk that surgery can cause a significant cosmetic change or affect how a body part works – especially if the tumour is located on the face, head or neck.

In these cases, a surgical technique called Mohs surgery can be used. Mohs surgery involves involves removing thin layers of skin at a time. Each layer of skin is then looked at under a microscope for signs of cancer. These layers of skin are removed until there is no sign of cancer left.

You will usually be under local anaesthetic for Mohs surgery.

After Mohs surgery, you can have reconstructive or plastic surgery to help treat any scarring.

Other treatment options

In rare cases, DFSP cannot be removed through surgery in a safe way. This may be because it has come back after surgery, it is too large to be removed by surgery, or it has spread to other parts of the body.

If the DFSP can’t be removed, you may have:

Targeted therapy

Targeted therapy is a treatment that targets specific characteristics within cancer cells to stop them from growing and spreading.

A drug called imatinib is sometimes used in these cases. Imatinib comes in the form of a tablet that you swallow with water after food. You may experience side effects such as tiredness and feeling sick. Your sarcoma clinical nurse specialist can help you to manage your symptoms.


This treatment uses high-energy radiation to destroy cancer cells. Radiotherapy is used to treat DFSP that cannot be removed through surgery or that has come back after surgery.

Radiotherapy can also be used after surgery to kill off any remaining cancer cells.


This treatment uses anti-cancer drugs to destroy cancer cells. It is sometimes used in patients that have a high risk of the cancer coming back. It is also used if the cancer has spread to other parts of the body, also known as “metastasis”.

However, research has found that chemotherapy is not effective in treating DFSP, so it’s rarely used.


In DFSP, researchers have been learning more about treatment known as “immunotherapy”.

Researchers have found that some targeted therapies and immunotherapies have worked well in clinical trials. But, more research and trials are needed before these treatments are approved.

You may be offered an opportunity to take part in a trial to investigate new diagnosis methods, drugs or treatments. Some studies also look at the care and well-being of patients. Your doctor or nurse can give you more information on opportunities for you to take part in a clinical trial.

  • There are trials currently recruiting patients with DFSP. For more information, please follow this link.

After treatment

After treatment, you will have regular follow-up appointments for several years. You should receive a follow-up schedule from your sarcoma clinical nurse specialist. The follow-ups will usually include:
  • A chance to discuss symptoms
  • A physical examination to look for any signs of DFSP
  • Scans such as MRI scans
  • A chest x-ray to rule out any secondary cancers occurring in the lungs
The vast majority of people diagnosed with DFSP recover well after treatment.

What if my DFSP comes back?

DFSP can sometimes come back in the same area where a previous tumour has been removed. This is called a recurrence. If DFSP does reappear, it is important to get treatment as quickly as possible. This could involve further surgery or radiotherapy.

Your treatment will be assessed on an individual basis.

It is useful to check for recurrences yourself through self-examination. If you are worried about your DFSP returning contact your doctor or clinical nurse specialist.

They may decide to bring forward the date of your follow up appointment to investigate your concerns.

Living with

What is my DFSP comes back?

DFSP can sometimes come back in the same area where the previous tumour was removed from. This is called a “local recurrence”.

If you have had Mohs surgery to remove the tumour then a recurrence is very rare.

To check for the cancer coming back, you will have regular follow-ups after your treatment for many years. If DFSP does come back, it is important to get treatment as quickly as possible. This could involve further surgery, or it could mean having targeted therapy, radiotherapy, or chemotherapy. Your treatment will be assessed on an individual basis.

It is useful to check for recurrences yourself through self-examination. This means checking yourself for any lumps, especially in the area around where the first tumour was removed from.

If you are worried about DFSP returning, speak to your doctor or a clinical nurse specialist. They may decide to bring forward the date of your follow-up appointment to investigate your concerns.

What if my cancer spreads to another part of my body?

It is very rare for DFSP to spread to another part of the body. But if it does, treatment may involve surgery, targeted therapy, radiotherapy, chemotherapy, or a combination of these treatments. This will be assessed on an individual basis.

In the rare cases where DFSP has spread to other parts of the body, they tend to spread to the lungs. This is why chest x-rays are taken at follow-up appointments.

If you have any questions or if you need to talk to someone, our Support Line team are here for you.


More information –


Last reviewed: 11 May 2023

Next review due: 11 May 2026


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