Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans is a rare skin cancer that begins in your dermis, your skin’s middle layer. It’s pronounced “dur-MAT-toe-fy-bra-sar-CO-ma” “pro-TOO-bur-anz.” Your healthcare provider might call it DFSP.

This slow-growing cancer rarely spreads. Still, you need treatment to remove the tumor and prevent cancer from coming back (recurrence) or spreading (metastatic cancer). With proper treatment, DFSP has a high survival rate.

Is dermatofibrosarcoma protuberans (DFSP) benign?

No. DFSP is a malignant (cancerous) soft tissue tumor and a type of skin cancer.

DFSP is a sarcoma (soft tissue tumor), cancer that develops in muscle, fat and skin. These tumors can also affect bones.

Can DFSP metastasize (spread)?

Cancer that spreads outside the original tumor is metastatic cancer. Approximately 1 in 20 people with DFSP experience cancer spread.

Metastatic DFSP is most likely to occur if you don’t get treatment or the cancer goes deep into fat and muscle.

For unknown reasons, DFSP can be more aggressive when it develops during pregnancy.

A small percentage of people with DFSP have an aggressive type called fibrosarcomatous dermatofibrosarcoma protuberans (DFSP-FS). These tumors are more likely to spread and come back after surgical removal.

How common is dermatofibrosarcoma protuberans (DFSP)?

DFSP is a rare cancer that affects approximately 4 out of 1 million people worldwide each year. They account for 1% to 6% of all soft tissue sarcomas.

The cancer typically affects adults ages 20 to 50, but children also get this skin cancer. Some infants have DFSP at birth. DFSP appears to affect people who are Black more often than people of other ethnicities.

What are the types of DFSP?

Pathologists (doctors who examine bodies and body tissues) examine cells under a microscope to determine the type of DFSP. Types include:

• Bednar tumors (pigmented DFSP) contain cells that have a lot of melanin, the substance that gives skin its color. Bednar tumors may have a mix of colors, including red, brown, blue and purple. These tumors account for about 5% of DFSP diagnoses.
• Giant cell fibroblastoma consists of giant cells. This type is also known as juvenile DFSP because it mostly affects children and teens.
• Fibrosarcomatous dermatofibrosarcoma protuberans (DFSP-FS) is a more aggressive cancerous soft tissue sarcoma.
• Myxoid DFSPs are made of an abnormal type of connective tissue called myxoid stroma. This type of DFSP is rare.