What is Dermatofibrosarcoma?

Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer. It starts in connective tissue cells in the middle layer of the skin (dermis). Dermatofibrosarcoma protuberans might look like a pimple or feel like a rough patch of skin at first.

What causes Dermatofibrosarcoma?

Causes. Dermatofibrosarcoma protuberans is associated with a rearrangement (translocation) of genetic material between chromosome 17 and chromosome 22. This translocation, written as t(17;22), fuses part of the COL1A1 gene from chromosome 17 with part of the PDGFB gene from chromosome 22.

Is Dermatofibrosarcoma malignant?

Dermatofibrosarcoma protuberans (DFSP) is a rare dermal malignancy affecting the deep dermis and subcutaneous tissues. It was first reported by Darier and Ferrand in 1924. This tumor is locally aggressive with high recurrence rates, but rarely metastasizes.

Is Dermatofibrosarcoma curable?

What is the prognosis for persons with DFSP? The general prognosis for DFSP is excellent. In the past, recurrence rates were high, but with the introduction of Mohs surgery, those rates have decreased. Even with recurrent DFSP, Mohs surgery has a 98% cure rate.

How serious is dermatofibrosarcoma protuberans?

The overall prognosis of dermatofibrosarcoma protuberans is good, with a 10-year survival rate of 99.1%. As metastasis is rare, morbidity due to local recurrence is a more common issue. Age older than 50 is a risk factor for local recurrence.

Dermatofibrosarcoma Protuberans (DFSP) Explained by Mayo Clinic

Can dermatofibroma turn cancerous?

Often these start out as red, turning later to brown, and sometimes itch. They probably are a reaction to a minor injury, such a bug bite or a splinter. We do know that they are harmless and never turn cancerous. For this reason, they are best left alone.

How is Dermatofibrosarcoma treated?

Surgical excision remains the mainstay of treatment for dermatofibrosarcoma protuberans (DFSP). Despite controversy, Mohs micrographic surgery has been increasingly accepted as the treatment of choice, while others advocate wide local excision.

How do you get rid of dermatofibroma?

A dermatofibroma can be completely removed with a wide surgical excision, but there is also a high likelihood of scar formation that may be considered more unsightly than the dermatofibroma itself. Never attempt removal of a growth at home. This can lead to infection, scarring, and excess bleeding.

Who treats Dermatofibrosarcoma?

Dermatologists also are studying new treatment options. One way to reduce DFSP from returning may be to treat patients with both excision and Mohs surgery.

How fast does dermatofibroma grow?

Usually, they develop over time, growing slowly until they reach less than 1 cm (about ¼ inch) in size. On rare occasions, larger lesions do occur, but dermatofibromas larger than 1 cm should be evaluated by a doctor.

What does DFSP feel like?

When dermatofibrosarcoma protuberans (DFSP) first appears on the skin, a person may notice: A pimple-like growth or rough patch of skin. No pain or tenderness where the growth or patch forms. Little change in the growth or patch.

Does dermatofibrosarcoma protuberans metastasis?

Dermatofibrosarcoma protuberans (DFSP) is a tumor that develops in skin or subcutaneous tissue and is characterized by a protuberant growth pattern [1]. DFSP often recurs locally after surgery, but rarely metastasizes to distant sites and is classified as a sarcoma of intermediate-grade malignancy [2–7].

Are Dermatofibromas painful?

Dermatofibromas are usually asymptomatic, but itching and pain often are noted. They are the most common of all painful skin tumors. Women who shave their legs may be bothered by the razor traumatizing the lesion in that region, causing pain, bleeding, erosive changes, and ulceration.

Do dermatofibromas go away?

Dermatofibromas are harmless growths that develop on the skin. However, they will not usually go away on their own. A person can opt for the surgical removal of unsightly or uncomfortable growths, or they can try a variety of other, less invasive treatments.

Who gets DFSP?

Who gets DFSP? People of all ages get this rare skin cancer. Most people are diagnosed when they are between 20 and 50 years of age. Although unusual, children get DFSP.

Is dermatofibroma a mole?

Moles appear when skin cells grow in clusters. While dermatofibromas are usually red, brown, or purplish, moles can be tan, black, blue or pink in addition to the typical dermatofibroma's color. Moles can appear in both exposed and unexposed areas of the body, including the armpits or even under nails.

Can dermatofibroma turn into DFSP?

One year later, Hoffman (2) described the tendency of the dermatofibroma tumor to develop into protruding nodules and termed the condition dermatofibrosarcoma protuberans (DFSP).

Can DFSP come back?

Dermatofibrosarcoma protuberans (DFSP) is very rare tumor of dermis layer of skin with the incidence of only 1 case per million per year. DFSP rarely leads to a metastasis (Less than 5% have metastasis), but DFSP can recur locally.

Can a dermatologist diagnose sarcoma?

Synovial sarcomas are an aggressive type of sarcoma, constituting approximately 10 percent of all soft tissue sarcomas, though not commonly diagnosed in the dermatology office.

What does a dermatofibroma look like?

Cellular dermatofibromas look like small round or oval bumps. They're usually less than 1 centimeter (cm) in diameter. They may be flesh-colored, brown or reddish-brown. Usually, dermatofibromas form on your arms or legs.

Do dermatofibromas get bigger?

Dermatofibromas are usually painless, but some people experience tenderness or itching. Most often, a single nodule develops, but some people can develop many dermatofibromas. They rarely grow larger than a half-inch in diameter. The cause of dermatofibromas is unknown.

Do dermatofibromas appear suddenly?

There are specific situations where dermatofibromas can be associated with underlying health issues. Typically, multiple dermatofibroma lesions that arise suddenly are seen in these situations. If you have multiple skin lesions that have come on suddenly, you should discuss this with your physician.

What's the difference between targeted therapy and chemotherapy?

Differences between chemotherapy and targeted therapy include: Traditional chemotherapy is cytotoxic to cells, meaning it damages healthy cells in addition to cancer cells. Targeted therapy affects cancer cells, leaving normal, healthy cells mostly intact. Traditional chemotherapy kills cancer cells that already exist.

Is mycosis fungoides caused by a fungal infection?

Mycosis fungoides is a rare kind of skin cancer called cutaneous T-cell lymphoma (CTCL). Also called granuloma fungoides, this skin disease may look like a fungal rash but is not caused by a fungus. Mycosis fungoides is a chronic condition that can slowly worsen over time.

What does it mean when a tumor has tentacles?

Anaplastic astrocytomas are rare tumors that tend to have tentacle- like projections that grow into surrounding tissue, making them difficult to completely remove during surgery.