Fluorouracil Plus Calcipotriene May Help Refractory Extramammary Paget Disease

NEW YORK (Reuters Health) - In patients who fail other therapies for extramammary Paget disease (EMPD), palliative therapy with topical fluorouracil and calcipotriene may be an option, a small case series suggests.

"EMPD occurs most frequently in the groin and genital area, and treatment is associated with a significant amount of morbidity and high recurrence rates," Dr. Peggy Wu of the University of California, Davis in Sacramento told Reuters Health by email.

"Because EMPD disproportionally affects a part of the body that is functionally, psychologically, and socially significant, optimizing treatment to deliver the greatest benefit while minimizing side effects is paramount," said Dr. Wu, who was at Beth Israel Deaconess Medical Center in Boston during the study.

"Most patients are offered surgery as initial therapy, but even with the movement towards tissue-sparing Mohs surgery, the potentially disfiguring nature of surgery is challenging," she noted. "Although other modalities, such as radiation, chemotherapy, and photodynamic therapy have also been tried, the data on efficacy are limited at best. Thus, finding a topical therapy which is both well-tolerated and efficacious would be ideal."

Hoping to find other patients, he revealed a cancer often mistaken for ‘jock itch’

Hoping to find other patients, he revealed a cancer often mistaken for ‘jock itch’

Stephen Schroeder figured he had little to lose, his growing sense of desperation fueled by the loneliness of his unusual diagnosis.

For more than two years, Schroeder had been coping with an extremely rare, invasive cancer called extramammary Paget’s disease (EMPD), which had invaded his scrotum, requiring multiple surgeries. Women account for roughly half of EMPD cases; the cancer, often misdiagnosed as eczema or contact dermatitis, attacks the sweat-producing apocrine glands, including those in the genital and anal areas.

The slow-growing cancer, which in men is frequently misdiagnosed as “jock itch” — slang for a fungal infection — can be fatal. And while treatment is often grueling, for Schroeder the worst part was his sense of isolation: He had never spoken to anyone who shared his diagnosis.

But in time Schroeder would engineer a solution to his isolation, reaping its benefits by connecting with others in similar circumstances. The richness of that experience, he said, has exceeded anything he could have imagined.

‘Jock Itch’ Nearly Killed Me: A cancer patient’s odyssey to find health and support

It all started with a small purple pimple on my scrotum. I was pretty sure it was an ingrown hair, so it was easy to ignore. Then it turned into a dime-sized rash that looked and felt a lot like jock itch. I wasn’t overly concerned.

When I finally showed the rash to my family doctor, nine months later, she concurred with my self-diagnosis and suggested trying a different antifungal cream. When the rash didn’t go away I went back to my doctor and she invited one of her colleagues to also take a look. They both agreed it was jock itch but I just needed a stronger treatment. By this time the dime-sized rash had grown to the size of a quarter.

After a year, when the rash remained, I decided to ask my dermatologist to take a look. (As someone with a past history of malignant melanoma – diagnosed more than two decades ago – I visit a dermatologist annually.) The dermatologist continued down the same path of treating a fungal problem. On the second visit he decided I must have an allergic reaction from either shampoo, soap or fabric softener. After five more months of chasing the problem, I requested a biopsy.

Living with a rare skin disease

Living with a rare skin disease

David Kendall shares his story

David Kendall has Extramammary Paget’s disease (EMPD), a rare cancer-related condition which is characterised externally by a chronic eczema-style rash of the skin in the anogenital regions or as is the case with David, the groin area.

Doctors described David’s EMPD case as “one in a million” due to its rarity, with the result being that he has found it difficult to access definitive information about it online.

Doctors thought he just had jock itch. Then it spread.

Doctors thought he just had jock itch. Then it spread.

Late Friday afternoon on Dec. 4, 2014, Stephen Schroeder was waiting to board his packed flight from Philadelphia to Las Vegas for a much anticipated long weekend with his son when his cellphone rang. On the line was an unexpected caller: his doctor, reporting test results sooner than Schroeder had expected.

Listening intently, Schroeder was flooded with disbelief as he struggled to comprehend what he was hearing. Using the lip of a trash can as a writing surface, he scribbled notes on the back of his boarding pass, making the doctor spell out each unfamiliar word. Then he sent a terse text to his wife, who was at their home in the Philadelphia suburbs, and got on the plane.

Onboard, Schroeder, then 55, fired up the balky in-flight Internet, desperate for information.

What he read over the next five hours left him alternately terrified, stunned and then, as denial took over, skeptical. “I kept thinking this must be some kind of really stupid mistake,” he recalled. “The diagnosis had to be wrong.”

Il morbo di Paget extramammario

Il morbo di Paget extramammario

Il morbo di Paget extramammario è una neoplasia spesso non diagnosticata clinicamente e che deve essere conosciuta per poterla porre in diagnosi differenziale. Si tratta istologicamente di un carcinoma intraepidermico a differenziazione ghiandolare apocrina, di solito localizzato in aree ove sono presenti ghiandole sudoripare.

East Kilbride family man takes on Men's 10K on Father's Day for charity based on his own experiences with skin cancer

East Kilbride family man takes on Men's 10K on Father's Day for charity based on his own experiences with skin cancer

An East Kilbride dad will take on a charity 10K on Father’s Day as he bids to raise money based on his own experiences with skin cancer. 

Dad-of-two David Kendall (57), who suffers from a skin condition called Extramammary Paget’s disease (EMPD), hopes his efforts will boost the funds of the British Skin Foundation by £1000.

The illness is a rare cancer-related condition which is characterised externally by a chronic eczema-style rash of the skin in the anogenital regions, and in David’s case, the groin area.

MSK Researchers Develop Targeted Test for Mutations in Both Rare and Common Cancers

MSK Researchers Develop Targeted Test for Mutations in Both Rare and Common Cancers

Building on our rich history of innovation, genome scientists, bioinformaticians and molecular pathologists at Memorial Sloan Kettering Cancer Center have developed a targeted tumor sequencing test, MSK-IMPACT™ (Integrated Mutation Profiling of Actionable Cancer Targets), to detect gene mutations and other critical genetic aberrations in both rare and common cancers. Until now, genomic testing of tumors has been routine practice only for patients with certain solid tumors, such as melanoma, lung, and colon cancer. MSK-IMPACT™ is much more inclusive and can be used on any solid tumor regardless of its origin, potentially offering better treatment options for thousands of patients in the form of precision oncology

Extramammary Paget's Needs More Than Mohs

Extramammary Paget's Needs More Than Mohs

Extramammary Paget’s disease poses a particular challenge because of its multifocal/multicentric nature.

"What you see with extramammary Paget’s is not necessarily what you get," Dr. Theodore Rosen cautioned at the Hawaii Dermatology Seminar sponsored by Skin Disease Education Foundation (SDEF).

This is an uncommon neoplasia that’s typically described as an erythematous, erosive, itchy patch or plaque having a strawberries-and-cream appearance. Yet there may be other areas of subclinical involvement at a distance from the obvious lesion. 

YouTube Video: https://www.youtube.com/watch?v=WmMnODBDUPY

Immunostaining Can Help Classify Paget Disease

EMPD is an uncommon condition occurring primarily in the elderly, with more women than men affected. It arises as a cutaneous adenocarcinoma with a proclivity for sites rich in apocrine glands. Patients with EMPD often present with a prominent solitary plaque lesion in the anogenital or vulvar region. The lesion is erythematous, eczematous, and often pruritic. The course is often locally aggressive, with frequent recurrences.

What Caused this Patient’s Groin Itch?

What Caused this Patient’s Groin Itch?

Case Presentation A 67-year-old white man presented with a 6-month history of a slowly increasing in size, slightly pruritic, erythematous rash in his right lower abdominal quadrant extending to his inguinal fold and right side of his scrotum. He reported having a similar lesion in his right axilla 7 years earlier that was surgically excised with no clinical evidence of recurrence. Otherwise, he was healthy with no history of systemic diseases. On examination there was an 8-cm by 6-cm erythematous, slightly elevated, papillomatous plaque extending from his right pubic area to the right lateral scrotal wall. No alopecia was noted overlying the plaque. No inguinal lymphadenopathy was appreciated on nodal examination. A well-healed scar was present in the right axilla, but no axillary adenopathy was noted. A KOH preparation of the lesion was negative for hyphae. Histologic examination of a 4-mm punch biopsy specimen taken from the inguinal plaque revealed an atypical cellular proliferation composed of clear cells ascending through the epidermis. These cells were positive for carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA) and mucicarmine stains. The S-100 stain did not stain these cells. What’s Your Diagnosis? Turn to page 98 for an answer and for more details.