Dermatofibrosarcoma Protuberans: Types, Causes, Symptoms, Risk Factors, Diagnosis and Treatment

Dermatofibrosarcoma protuberans is a rare type of skin cancer that originates in the dermis, or middle layer of the skin.  It might be referred to as DFSP by your doctor.

This slowly growing malignancy rarely spreads. However, you need medical attention to remove the tumour and prevent the cancer from returning (recurrence) or spreading (metastatic cancer). If given the proper care, DFSP has a decent survival rate.

Types Of Dermatofibrosarcoma Protuberans

A microscope is used by pathologists, or medical professionals who examine bodies and bodily tissues, to identify the type of DFSP. Among the types are:

• The cells present in bednar tumours (pigmented DFSP) contain large amounts of melanin, the pigment that gives skin colour. Bednar tumours are coloured in a spectrum that includes red, brown, blue, and purple. These malignancies are linked to about 5% of diagnoses for DFSP.
• Giant cell fibroblastoma is caused by massive cells. This type is occasionally referred to as juvenile DFSP because it tends to affect children and teens.
• Soft tissue sarcomas that are more aggressively malignant are called fibrosarcomatous dermatofibrosarcoma protuberans (DFSP-FS).
• Myxoid stroma is the initial form of an unusual type of connective tissue.

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Causes Of Dermatofibrosarcoma protuberans

A genetic change, or mutation, is the cause of the illness in up to 90% of people with DFSP. This gene change occurs in cells post-birth. You do not inherit the DFSP-causing gene mutation from your parents.

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Symptoms  Of Dermatofibrosarcoma Protuberans

Early signs and symptoms of DFSP are easy to overlook. Tumours typically appear on the back, shoulders, chest, abdomen, or buttocks. Tumours may also form inside your lips, on your scalp, or in your arms or legs.

At first, there can be a little patch of skin that looks bruised. It's a level, painless area. It could feel itchy and seem discoloured. In infants and young toddlers, DFSP can have a birthmark-like appearance. These patches usually have a diameter of 1/2 to 2 inches (1 to 5 cm).

As the cancerous growth advances, the signs and symptoms of DFSP become increasingly noticeable. The growing tumour pushes the epidermis, or outermost layer of skin, inward. "Protuberans" are hard lumps (nodules) of tissue that grow on the skin.

The nodules that you may find are:

• Easy to break or bleed.
• Tightly attached to your skin
• Stretching and widening your skin.
• Either rigid or flexible.
• Reddish-brown to blue-violet to red.
• Tender.

Cellular dermatofibroma, a noncancerous skin disease, may mimic DFSP, especially in the early stages of the cancer. Cellular dermatofibromas are benign soft tissue tumours that typically appear on the legs. They may itch or pain. Most cases of dermatofibromas resolve on their own.

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Risk Factors Of Dermatofibrosarcoma Protuberans

In addition to racial differences, scars or skin injuries may increase the risk of developing delayed onset prostate cancer. Skin injuries can result from the following:

• Radiation therapy 
• Burns
• Incisions made during surgery
• Ink

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Diagnosis Of Dermatofibrosarcoma Protuberans

The protuberances dermatofibrosarcoma is diagnosed by the following tests and procedures:

• Examination of the skin: To look for any signs of skin cancer, your doctor will inspect your skin.
• Skin biopsy: Your doctor may remove a small sample of tissue to be tested. Cancer cells can be found through laboratory testing.
• Imaging tests: Sometimes an MRI or other imaging test is necessary to assess the extent of the malignancy and help with treatment planning.

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Treatment Of Dermatofibrosarcoma Protuberans

In most cases of dermatofibrosarcoma protuberans, surgery is necessary to remove the cancer. More therapies may be used to get rid of cancer cells that may remain after surgery. Treatment options include the following:

• Surgery to remove the malignancy: Your doctor may recommend surgery to remove the cancer along with some good tissue surrounding it. This increases the likelihood that every cancer cell is destroyed.
• Mohs methodology: In a process known as a Mohs treatment, thin layers of cancerous skin are removed until only healthy tissue is left. After removing each layer, the skin is examined for signs of cancer. The treatment continues until no longer shows any signs of cancer. Mohs surgery may be helpful if the cancer has progressed. Dermatofiscolaris protuberans has an unusual development pattern, which makes it difficult to completely eradicate.
• Radiation therapy: Radiation therapy uses powerful energy beams, such as protons and X-rays, to kill cancer cells. If radiation therapy wasn't able to eradicate the cancer after surgery, your doctor might recommend it.
• Targeted therapy: Targeted therapy drugs specifically target specific molecules present in cancer cells. By blocking these chemicals, targeted drug therapies cause cancer cells to die. Some people with dermatofibrosarcoma protuberans have cancer cells that produce an excessive amount of protein. A drug called imatinib (Gleevec) can target and kill particular cells. If your cancer returns after surgery, your doctor may recommend this course of treatment.
• Clinical assessments: Testing innovative medications through clinical trials is one option. Consult your healthcare provider to determine whether you qualify to take part in a clinical study.

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