The Dangers of a Rare Skin Cancer

An Ordinary Bump, a Shocking Diagnosis

A noted New York City dermatologist, Diane S. Berson, MD, can swiftly spot the warning signs of skin cancers. Yet, even she was caught off guard to learn that the shiny, benign-looking, cyst-like bump on her mother's forehead was a Merkel cell carcinoma (MCC), a rare, virulent skin malignancy usually caused by sun exposure. The disease quickly spread to her lymph nodes, and just six months after being diagnosed, Dr. Berson's beloved mother Florence passed away in 2007 from complications of the metastasis and subsequent treatment.

"If this hadn't happened, she would have had an active lifetime ahead," Dr. Berson lamented. "She turned 80 on her first day receiving chemo in the hospital, but was more like an energetic 60-year-old. To the last moment, she was the smartest person I've ever known. We were very close; she was always the first person I called to get together when I had free time."

Florence was many things throughout her life: an incredibly supportive mother of two daughters, opera singer, travel agent, English teacher, and eventually, office manager for her husband's psychiatry/neurology practice. She was also a beach enthusiast, which raised her risk for skin cancer; about half of all MCCs occur on sun-exposed parts of the body. "Her favorite thing was to walk or read on the beach," Dr. Berson relates. "She was fair-skinned, blue-eyed, and blonde-haired, as am I," thus highly vulnerable to sun damage.

Florence previously had both basal cell and squamous cell carcinomas, and improved her sun protection habits afterwards, but she could not eliminate a lifetime of sun damage. This time the skin cancer was lethal; 40 percent of MCC patients die within 5 years.

"People must respect any new or changed skin growth — even if it looks harmless."

As a dermatologist, Dr. Berson found herself even more frustrated than the average person. "Finding out the diagnosis of this small lesion led to frustration, as there was a lack of consensus then on how to manage this kind of tumor," she explains. After surgery, followed by two courses of radiation, the only option available was chemotherapy, which in many cases kills the patient before it kills the cancer. In part, that's because MCC is so rare; though the figure has tripled in the past 20 years, only 1,500 new cases are diagnosed annually in the US. "More public recognition about this cancer came a little after my mother's death, with the discovery that a specific virus is also involved in this disease." Studies may ultimately show how to address the growth of the virus. "Research is very important, so that future families have more options," says Dr. Berson, whose father also died from side effects of chemotherapy. "We might have been luckier with more targeted (less dangerous) therapies. There is still so much more to learn."

One thing is certain, says Dr. Berson. "People must respect any new or changed skin growth — even if it looks harmless. I'm sure many people with MCC are diagnosed late because it doesn't always appear to be a malignant lesion. As a daughter, I lost my mom and best friend; being a dermatologist made it that much more difficult."

Published on May 12, 2014, Updated on May 11, 2016