Rare Cancer: Gynecologic Extramammary Paget's Disease

There are tests to determine whether immunotherapy will work - mine showed it would not, so i did not have immunotherapy after all. Instead I had a round of Chemo. Doing fine.

Good morning! Oh dear: you are suffering. Too bad you are not in California because cannabis save is legal.... never put it there though but at every manifestation i am told to use Imiquimod. It treats the EMPD of the vulva manifestations. The itching and burning i have is from the Imiquimod that i take topically for like 8 weeks. My oncologist and I have found my tolerance level of side effects. i don't before itch and burn before treatment but a visual from my ob/gyn reveals skin manifestation. i have been told repeatedly that i need to look there every so often because one can see like an aging spot.... I was also told repeatedly that it is a slow growing pre cancer type of cancer. I am 68 and went to what I thought was my last pap smear at 65. There is no pap smear after 65 but it is recommended to get the visual exam once a year. I would be more comfortable if I could get the visual every 6 months. Cold pad wrapped in a towel, and alternatively hot pad wrapped also off and on when itchy and burning. sit bath also (seat bath?)... lidocaine to numb... other meds to help with itching burning... I am all for it since I don't tolerate this kind of pain... let me know how it goes for you over time. Blessings

So glad your treatment is working for you. Did you have any surgery before chemo? Was the chemo difficult to go through? What about the lesion? Did the chemo cause it to go away? As you can see I am full of questions. Just diagnosed and seeing an oncologist on June 19. Where did you go for treatment? What kind of oncologist are you seeing?
I appreciate any insight you can give me.

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.

I'm just reading your post from May 6. I assume that is 2024? I'm wondering how your surgery went or did you opt for something else? What can you tell me about Aldara that you mentioned in your post.
Hope whatever method of treatment you chose that you are doing well.
To @nomikins

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.

Hi @nomikins and @worried123, I appreciate your willingness to share your email. Because Mayo Clinic Connect is a public forum, the community guidelines (https://connect.mayoclinic.org/blog/about-connect/tab/community-guidelines/) recommend using the secure private message to exchange personal contact information.

Having said that, I’d like to underline the benefit of sharing in the group discussions. By posting in the discussions in the Gynecological Cancers support group many members, including people who do not post, benefit from from your knowledge and experience and others can chime in as well.

Thank you for sharing all that information with me. I may take you up on your offer a little later, after I have seen my new oncologist. Right now I don't even know what other questions I have.

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.

Topical imiquimod cream is supposed to be a somewhat effective option - but the recurrence rate is pretty high. Apparently people have also tried photodynamic / red light therapy and lasers but it isn't very common. I can't provide the link I found since this forum blocks it but if you google "henry ford extramammary paget's disease" there's a recent publication titled: "Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease" that explains different treatment recommendations for EMPD.