Rare Cancer: Gynecologic Extramammary Paget's Disease

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.

Oh my gosh I just had my scouting biopsies done (6 of them) 2 weeks ago and know what you mean by the anesthesia shots - they really hurt! Actually the first time I got a biospy - it was from a ob/gyn not a Mohs surgeon and she didn't give me any anesthesia shots - only a numbing cream when she took my vulva biopsy. It hurt so bad. It wasn't until I talked to a dermatologist friend and the Mohs surgeon where they told me that my ob/gyn should not have done that.

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.

Sorry, Colleen, just seeing this now. Still learning how to navigate this platform. Thank you for your welcome and questions. Yes, that’s the trial I was referring to, but coming out to the Mayo Clinic right now is not an option.
Right now I am looking at doing Mohs surgery where I live. I am in the Bay Area and the team consists of Stanford doctors. I’m seeing a GYN and a Derm. The Derm is a specialist in Mohs surgery and the GYN appears to be very familiar with EMPD. So I feel I’m in good hands. But, I am hoping to find info. on less invasive treatments, like Aldara. They will review my options with me this Fri but I already know they believe the Mohs surgery is the best option for me. I’ve haven’t had surgery since I was much younger, so this is a big deal for me and I’m pretty freaked out. But keeping it together for the sake of my daughter.

Just by way of an update, on my visit to the Mohs surgeon, things went well, I guess. What went well is that she answered my questions and those she didn’t know how to answer (I asked about some non surgical treatments), she vowed to research. What also happened is that she decided to do more scouting biopsies, which is never fun. But at least this time we were able to minimize the pain from the anesthesia shots.

I am very new here and just learning the ropes. Just diagnosed. I thought surgery was the preferred treatment. May I ask what "treatment" is keeping things in check for you? I am 84 and probably can't stand surgery. Wondering if and what any other options are. From what I have read I get the idea that EMPD recurs a lot. Guess that's just the nature of the beast. My dermatologist has been treating me for lichen sclerosis for nearly three years before taking a biopsy last week.

I had surgery at Cedars Sinai in LA. they did wide incision and removed 3 lymph nodes, two of which had the cancer. that makes me 3A. The tricky thing about recovery from the surgery was getting in and out of bed because I couldn't sit. that was a week or 2, i honestly can't remember. about 3 weeks later I started radiation: 5 days a week for I think 6 weeks. That was ok until the last week and the 2 weeks after which were AWFUL!! I was in so much pain that with the permission of my oncologist I doubled the amount of codeine I was taking and ended up in the hospital with an overdose. Well that's behind me now. I started chemo maybe a month later and have my last one in 2 weeks. It's actually been quite easy. The oncologist said chemo was optional. There is so little research on extramammary pagets of the vulva that he said we couldn't make the decision based on it. I opted to do the chemo. no reaction until 3rd and it's been a couple of days of nausea - really nothing to complain about. As I said, I did the surgery in LA where I had previously been seen for breast cancer (2a) and had my breast cancer oncologist there. She recommended the surgeon and he recommended the radiologist. by the way, the surgeon didn't think i needed either radiation or chemo but the radiologist and the oncologist thought i needed both. I live too far from LA to have the radiation there, so I started seeing an oncologist at UCLA-Santa Barbara who recommended the radiologist. The Santa Barbara radiologist didn't think I needed chemo, but as I said, i did it anyway. Well that was a long story.

Hi yes it’s from this year. I’ll be seeing the gyn tomorrow and the dermatologist doc the week after. I’ll let you know how it goes. Everyone is still trying to
Decide which approach is best. No surgery yet.