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Causes of Cancer

Disease Education

Dermatofibrosarcoma Protuberans (DFSP)

 

Overview

What is dermatofibrosarcoma protuberans?

Dermatofibrosarcoma (dur-mah-toe-fy-bro-sar-KO-ma) protuberans (pro-TOO-bur-anz)—DFSP—is a rare type of cancer that occurs in the skin. In particular, DFSP develops in the dermis, which is our second layer of skin.

 

How common is DFSP?

DFSP is exceedingly rare. It develops in only about 1 of every 10 million people a year. It typically affects people in their 20s, 30s, and 40s. Men and women are equally affected.

 

What causes DFSP?

The cause is unknown, but DFSP appears to arise from a rearrangement of a person's genes. Researchers have found that people with DFSP have a change in their genes that causes their bodies to make a protein called a kinase (KY-nase), which promotes the growth of DFSP.

 

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