Dermatofibrosarcoma protuberans
Make an Appointment
Talk to DoctorOverview
Dermatofibrosarcoma protuberans (DFSP) is a type of skin cancer that begins in the connective tissue cells in the dermis. It may resemble a pimple or feel like a rough area of skin. This rare condition commonly develops on the arms, legs, and the trunk.
Dermatofibrosarcoma protuberans originates in the dermis, the middle layer of the skin that serves multiple functions such as safeguarding the body, providing structural support to the epidermis, sensing various sensations, and generating sweat.
Dermatofibrosarcoma protuberans is categorized into several subtypes, such as:
- Bednar tumors (pigmented DFSP): About 5% of diagnosis for the DFSP are within this category. A spectrum of hues, including red, brown, blue, and purple, may be present in bednar tumors. These melanin-rich cells are what give skin its color.
- Giant cell fibroblastoma. This type of DFSP primarily affects kids and teenagers. It is also referred to as juvenile DFSP.
- Fibrosarcomatous dermatofibrosarcoma protuberans (DFSP-FS): It is characterized by a more aggressive growth pattern of cancerous soft tissue sarcoma.
- Myxoid DFSPs: This is an uncommon type of DFSP constructed from an abnormal form of connective tissue known as myxoid stroma.
DFSP has a slow growth rate and seldom spreads outside of the skin. Radiation therapy, targeted therapy and surgery are among the treatment options for dermatofibrosarcoma protuberans.
Symptoms
The signs and symptoms of DFSP become more visible, as the tumor starts to protrude into the outermost skin layer, resulting in the formation of firm tissue lumps referred to as protuberans. However, its initial signs can be subtle and easy to overlook.
Typically, DFSP develops in areas like the chest, back, shoulders, abdomen, or buttocks, although they can also develop on the arms, legs, scalp, or even inside the mouth. The nodules may be seen to be:
- Rubbery or hard
- Firmly adhered to your skin (immobile)
- Tender
- Easy to bleed or crack open
- Reddish-brown to violet, blue, or red in color
- Increasing in size and stretching the skin
If any of the signs and symptoms are observed, consult a healthcare provider for proper diagnosis and treatment. Medical diagnosis is important especially in cases where there is persistent or new skin growth, changes in moles, birthmarks, scars, tattoos, or skin growths that bleed easily. These skin changes could potentially indicate underlying health issues that require attention and expert assessment.
Cause
Experts believed that dermatofibrosarcoma protuberans is caused by a genetic mutation that arises in cells after birth. Up to 90% of individuals who develop DFSP experience a genetic mutation that leads to the condition.
Risk factors
The risk of DFSP is increased by skin injuries or scars, which can result from burns, radiation therapy, surgical incisions, or tattoos. These factors can contribute to an increased susceptibility to DFSP development.
Diagnosis
The diagnosis of dermatofibrosarcoma protuberans involves several tests and procedures, such as:
- Skin examination: Symptoms of skin cancer are initially detected during a visual examination of the skin. A healthcare provider will look for any unusual skin changes, growths, or lesions.
- Skin biopsy: This procedure can determine whether cancer cells are present. During a biopsy, a small sample of the affected skin tissue, a portion or all of the tumor is removed for laboratory analysis.
- Imaging tests: If the skin biopsy confirms DFSP, an MRI may be required to find out how deep and how big the tumor is.
Treatment
Dermatofibrosarcoma protuberans may involve a combination of treatments to become successful. In most cases, the treatment starts with surgery and may require further therapies to remove the remaining cancer cells.
- Cancer removal surgery: This can increase the likelihood that all cancer cells are eliminated. The procedure involves removing the cancer and some of the surrounding good tissue.
- Mohs surgery: The primary treatment for DFSP is surgical removal, typically carried out through Mohs surgery. This procedure involves numbing the treatment area with a local anesthetic, surgically excising the cancerous tumor along with a small amount of healthy tissue and then examining the tissue edges under a microscope for cancer cells. If cancer cells are found at the margins, more tissue is removed until no cancer cells remain. After ensuring complete removal, reconstructive surgery may be performed.
- Radiation therapy: If surgery is unable to completely eliminate all the cancer, radiation therapy may be required. This employs high-energy beams like X-rays and protons to destroy cancer cells.
- Targeted therapy: Imatinib is often utilized to address metastatic DFSP or tumors that are either too sizable or challenging for surgical removal. It can also reduce the tumor size, facilitating surgical intervention when necessary. Cancer cells in some people with dermatofibrosarcoma protuberans overproduce proteins. Targeted therapy focus on specific substances found in cancer cells. By blocking these substances, targeted drugs induce the death of cancer cells.
- Clinical research: Taking part in a clinical trial dedicated to evaluating new treatments for DFSP can also be a possibility. However, it is essential to discuss this with a healthcare provider, particularly the unknown risks that come with it.
