Dermatofibrosarcoma protuberans

Overview

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.