In the fall of 2013, Stephen Schroeder noticed a small, purplish pimple on his groin. “I was pretty sure it was an ingrown hair, so I ignored it for six months,” figuring it would go away on its own, he recalled. The pimple disappeared, replaced by a dime-size red, scaly rash on his scrotum. Schroeder, now 58, said he assumed it was jock itch — vernacular for a common fungal infection of the groin — and did nothing for several more months.
“I’m a guy,” said Schroeder, who lived in the Philadelphia suburbs at the time. “It didn’t faze me much.”
The director of membership development for a purchasing cooperative had been successfully treated for melanoma, the deadliest form of skin cancer, in 1989. He had not had a recurrence and was always vigilant about annual checkups.
In the spring of 2014, as his primary-care doctor was on her way out the door after a routine appointment, Schroeder said he remembered the red patch “almost as an afterthought” and mentioned it.
She examined the spot, agreed it looked like jock itch and suggested he try the standard treatment: an over-the-counter antifungal cream. Several weeks later, when the problem remained, Schroeder returned. His physician and one of her partners took a look and prescribed a stronger antifungal medicine.
“They both agreed it was nothing to worry about,” Schroeder recalled. “It wasn’t painful, just annoying” and itchy.
The second medicine was no more effective than the first. Schroeder then consulted his dermatologist. He, too, initially agreed it was a stubborn fungal infection, which thrives in moist, dark areas of the body. Later, the dermatologist changed his mind and suspected eczema, a skin inflammation that causes redness and itching. Another possibility, he told Schroeder, was that he had developed contact dermatitis, a common allergic reaction to the shampoo, soap or fabric softener he used.
Schroeder thought that was odd, but he changed his shampoo and soap brands and stopped using fabric softener. “I think he was winging it as he went,” Schroeder said of his dermatologist. The rash didn’t improve.
Fifteen months after the first symptom, Schroeder returned to the dermatologist and asked him to perform a biopsy to determine the cause of the red patch.
Then, late on the afternoon of Dec. 4, 2014, as Schroeder was waiting to board a flight to Las Vegas for a much anticipated long weekend with his son, his cellphone rang. On the line was his doctor, reporting test results sooner than Schroeder had expected.
“Steve, this is kind of weird,” he remembers the dermatologist saying when he answered his phone.
The doctor told Schroeder that his “jock itch” was actually an extremely rare, invasive cancer called extramammary Paget’s disease (EMPD), a malignancy that involves the apocrine glands that produce sweat; it most often affects the vulva, scrotum or penis.
The cause of the cancer is unknown, though some EMPD patients have close relatives with breast cancer. Schroeder’s mother had the disease.
EMPD is slow-growing, and mimics eczema and other benign conditions. A two-year delay between the appearance of the first symptoms and a definitive diagnosis is not uncommon, studies have found. In some cases, EMPD reflects an underlying cancer elsewhere in the body, but in others, including Schroeder’s, no other cancer can be detected.
If left untreated, EMPD can spread and become lethal. Only a few hundred cases have been reported worldwide, most of them in women over 50.
As Schroeder tried to absorb the news, the dermatologist told him that he knew nothing about EMPD. He recommended that Schroeder consult a plastic surgeon.
Once in-flight and doing research online, Schroeder was aghast at what he read. “It said things like, ‘This is a death sentence,’” he recalled. “I was just freaking out. I spent a lot of time praying and a lot of time crying” as surreptitiously as possible.
The plastic surgeon Schroeder saw told Schroeder he had never heard of EMPD.
Schroeder called his dermatologist for help finding a doctor and was referred to Bradley D. Figler, then the director of reconstructive urology at Thomas Jefferson University. He was reassured when he learned that Figler was treating another man recently diagnosed with EMPD. In fact, Schroeder was the fourth EMPD patient Figler had seen in his 10-year career.
“The cases were all pretty similar,” said Figler, who is now an assistant professor of urology at the University of North Carolina School of Medicine. “They’re all middle-aged white guys.”
In January 2015, Schroeder underwent about eight hours of surgery at Jefferson to remove the cancer, which had grown to encompass an area two inches by three inches, and to rebuild his scrotum, using skin grafted from his left leg.
Figler said that the operation, which he performed with other specialists, involved Mohs surgery, in which thin layers of skin are progressively removed and examined under a microscope by a pathologist until there is no detectable malignancy. The goal is to remove all cancer while minimizing damage to surrounding tissue. That is particularly tricky in EMPD cases, Figler said, because the cancerous cells often are not clumped together.
“He dealt with pretty radical surgery and a very significant recovery well and took it all in stride,” Figler said. “It’s really difficult to find expertise in this disease. He really sought it out.”
After Schroeder’s six-week recovery, the waiting began. Recurrence is the rule, not the exception.
In October 2015, Schroeder discovered another red patch, not far from the location of the first. He underwent a second surgery in Spokane, Wash., where he and his wife had relocated to be closer to relatives.
One of his doctors pointed him to a study underway at Memorial Sloan Kettering Cancer Center in New York involving a noninvasive imaging technique using confocal microscopy, which may be able to detect some cancers earlier than usual and with more precision. Schroeder enrolled in the study.
In early 2016, doctors there found three suspicious areas near the site of his original surgery, including on his leg; biopsies revealed EMPD. Then in August 2016, Schroeder underwent a third operation at the University of Washington in Seattle. He has returned to work and periodically flies to New York for testing as part of the study.
“I’m going to use whatever tools I can find at my disposal to try to keep this thing at bay,” he said.