Recognizing Rare Types of Skin Cancer

Hearing you have any form of cancer can feel scary. And hearing you have a rare form might add to those emotions. However, it’s important to know that rare forms of skin cancer — including extramammary Paget’s disease (EMPD) and dermatofibrosarcoma protuberans (DFSP) — are often treatable.

No one quite knows what causes EMPD, and DFSP seems to stem from a genetic mutation. Because both of these rare skin cancers grow slowly, they can go undetected and unaddressed for years. The sooner you seek care for any potential signs, the better your outcome is likely to be.

Skin cancer specialist Ali Hendi, MD, offers on-site Mohs micrographic surgeries on an outpatient basis at his Chevy Chase, Maryland, practice to treat and slow down the growth of these skin cancers. In this blog, Dr. Hendi discusses the symptoms and treatments of EMPD and DFSP.

Symptoms of extramammary Paget’s disease

Extramammary Paget’s disease usually appears on the surface of the skin and then slowly travels to deeper skin layers. It grows so slowly, in fact, that it may take 10-15 years before you notice any visible signs. Extramammary Paget’s disease typically affects the anal area, genitals, and groin. 

A chronic rash is the most common EMPD symptom. The rash usually looks like the flaky bumpiness of eczema, but it won’t respond to topical steroid creams. Additional symptoms may include:

• Burning or itching, which may lead to bleeding
• Thickened crusty, red lesions
• Spreading from the genital region to the thighs

Symptoms of dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans are tumors that form in deep layers of the skin, particularly on the arms, legs, neck, and torso. The first sign you may notice is a small area of firm, discolored or depressed skin. The area might look like a bruise or scar. Then, as the cancer grows, lumps of tissue can form near your skin’s surface, bringing more obvious signs. 

Treatment for EMPD and DFSP

Because EMPD and DFSP tend to grow slowly, most cases aren’t noticed or diagnosed until the cancer is pretty advanced. Thankfully, both forms of cancer are still treatable. 

Both EMPD and DFSP treatment require Mohs surgery. During this procedure, Dr. Hendi removes layers of tissue one at a time and examines each one for any presence of cancer cells. He continues this process until no signs of cancer remain. By removing one layer at a time, this can preserve as much healthy tissue as possible.

Although EMPD may return at some point, Mohs surgery greatly lowers the risk of DFSP’s return. Other possible treatments include radiation therapy, targeted therapy, and clinical trials.

If you have skin cancer and want treatment, or if you want to see if you have skin cancer, book an appointment online or over the phone with Ali Hendi, MD, today.