Rare Cancer: Gynecologic Extramammary Paget's Disease

Feb 7 2025
I haven’t checked in for awhile. I thought I had offered everything I could. My update: I had my 7th, yes seventh, Paget’s surgery since 2013 in Dec. 2024. Each time I say “never again” & each time I say, “one more time”, thinking maybe this will be the last one. Never had totally clean margins, but also no invasive cancer, so I guess I feel I’m ahead. I just keep wanting to get rid of as many Paget’s cells as possible.
I unsuccessfully tried Immiquimod two separate times between surgeries. Had burning, itching, & even swelling in my leg.
Each surgery takes a few months to fully recover, so back to skirts, carrying pillows, & depression. Yet I do not regret my decisions to have the surgeries. Anything I can do to fight this wretched disease is worth it. However I will be 80 in 2025, so my recent surgery may be my last.
At least I have educated literally 100s of individuals, including medical personnel, about Paget’s. I talk about it to anyone who will listen. Hopefully, with increased awareness, there will be increased research & help for future sufferers.

Hello...
I am coming in on February 6, 2025. Where do you stand at this point? I had
surgery approximately two to three years ago and the Paget's is back. The
Oncologist has now recommended the Imiquimod. I have Paget's in more than
one place. We are starting with the uppermost spot and if it works, we will be
moving down to the next spot. I really need information on how this worked
for other women.
Thank you and would appreciate hearing back as soon as possible.

I have my follow up next week so until then I'm pretending I'm all good lol I'm sorry you're going through this it's amazing to me that so much is still unknown. Sending lots of prayers ❤️

Sonya,,,
I could have written this myself. I have cancer of the Vulva (never had heard
of this) and have had similar situation. I had surgery and after two years, it
is back and they are now having me use the Imiquimod cream. It burns and
hurts. I do not know what to expect from here. There is not a day that goes
by that I do not burn and itch. But, some days are better than others. I
pray that this does not move into a primary organ. I think I am going to ask
my doctor to do a scan to make sure that the cancer has not spread. I, too,
would like to know the mortality rate of this disease. Please let me know how you are doing.

I have Paget's Disease of the Vulva. I had surgery September 6 and am still recovering. I had no idea what I was in for the doctor gave no inclination of the pain and recovery burning. The sample came back and the margins are not clear, so I don't know where I am going from here. I do not want to have any more surgeries I do not think I can take it. I retired less than a month prior to my diagnosis and am not mentally handling this well. I'm sure to caring for other people, not being down. I hope your doctor actually explains things better. I still don't know what is going on.

Hi everyone - I was recently diagnosed with extramammary paget's disease of the vulva and I'm only 34 years old. Apparently this typically affects older people in their 60s+ but here I am and I have it. I wonder if there are any patients diagnosed and seen prolonged success with nonsurgical interventions like topical imiquimod, photodynamic therapy, lasers etc or how long those treatments were able to stave off eventual surgery. If there any other younger patients like me - also love to get your perspective on your experience and learn.

I still feel like I have at 30 years of good quality of life with my partner and still want to have kids before I have to consider a vulvectomy or any invasive radiation / excision that compromises my genital /reproductive areas and I'm pretty scared about it. Really want to try nonsurgical treatments if possible. Reading the scientific publications and speaking to my dermatologist - it seems the recurrent rates are still pretty high with these nonsurgical treatments Complete response could be somewhere between 30-50% for topical imiquimod cream but it seems it comes back in 30%+ of cases within a year or so. I'm still waiting to see if this is primary EMPD - so that it's localized to just the epidermis on my vulva and not that I have cancer somewhere else like breast, bladder, etc - which hopefully with my age is not the case.

Presentation/Diagnosis
I probably saw some epidermis/skin level change of the vulva changing close to 2 years ago but didn't think much of it since I was asymptomatic until I got an IUD inserted last year and my NP thought I had a yeast infection. It seemed to go away for my first treatment of fluconazole but then it kept coming back and I kept testing negative for candidiasis / yeast infection and the fluconazole they were giving me didn't seem to work anymore. It was also super itchy especially after having sex. It took my not being able to sleep one night that I was fed up and asked to be referred to an ob/gyn doctor where they finally took a biopsy for confirmation of extramammary paget's disease. They also originally those it was lichen simplus chronicus but my ob gyn took a biopsy just in case - thank goodness since it wasn't. So basically - almost a year from when my symptoms started and probably close to 2 years where i didn't have any itching symptoms but likely started seeing skin changes near my vulva. I did also test positive for HPV two years prior - but never for the high grade lesions - only low grade and this most recent pap smear I tested negative for HPV - just wondering if that had anything to do with getting EMPD. Anyone have a similar experience or had HPV history?

Referral Diagnosis
I went to a dermatologist who specializes in EMPD and she did 6 clock biopsies around my vulva including in areas that looks normal and unfortunately the 3 biopsies closest to my clitoris - were positive for CK7+ which is typically indicative of EMPD. In all three of those biopsies it was just on the edge of where I had the itchy patch so basically where my skin looked totally normal - it still tested positive for EMPD. My doctor is referring me to a ob gyn oncologist for further consult but vulvectomy including clitoris removal seems likely given how close the biopsies that tested positive were to my clitoris. We're going to start on the topical imiquimod for now but I don't feel optimistic it's going to work - primarily because it seems even if my skin looks normal - it tests positive for EMPD so I feel like I still have no idea how much to spread the topical treatment around my vulva since vulva that look normal but may still have paget's disease.

Any thoughts on that for folks who have tried it? Was planning to start it later this week. My doctor is recommending 2x a week for 3 weeks and then I have a follow-up. It seems like the side effects of itching / burning are not pleasant.
Also anyone else try photodynamic / red light therapy? or lasers? also saw those are options but not a lot of literature review / scientific articles with data on its efficacy.

Joyce: Hi: originally Imiquimod was used for something else but has been proven good for treating the manifestations of Paget on the vulva. So far, I am proof of it. I think it costs $0 if you have Medicare supplement, like i have with Kaiser. Side effects of using the cream can be minimized.. I will be glad to go into the details if you need me to. Healing thoughts to all!

THANK YOU, HELEN. I am new to this site and I already love it and everyone.

@joyceinil Oh, dear, you have certainly been through it in hte last few months. How frightening to suddenly lose vision in one eye. It's good to know that your doctor was right there and you went to the wonderful hospital in St. Louis. While no one wants to be in the hospital it sounds like you had good care there although you were exposed to COVID? I do hope you are feeling better today, can wean off the steroids and start the treatment again for EMPD.