Rare Cancer: Gynecologic Extramammary Paget's Disease

Welcome, @moffitt. As you said, extramammary Paget's disease (EMPD) is very rare. I had not heard of it. But through my searching online I did find an open clinical trial at Mayo Clinic that may be of interest to you:
- Gynecologic Extramammary Paget's Disease https://www.mayo.edu/research/clinical-trials/cls-20387127

To help me better understand the condition, I also found this educational tutorial for clinicians to learn how to properly evaluate for extramammary Paget disease. It also highlights the role of the interprofessional team in caring for patients with this condition.:
- Extramammary Paget Disease https://www.ncbi.nlm.nih.gov/books/NBK493224/

Moffitt, is this a new diagnosis for you? Are you currently in treatment?

I have had EMPD since 2013 but did not know it. Every dermatologist or OBGYN told me I had Herpes or Ezcema or some kind of keritosis. I was prescribed steroid creams and herpes drugs. Finally, in 2021 I went to a new dermatologist for annual skin cancer screening. I asked if he was comfortable checking a condition in my gential area. Yes he was; and he did a biopsy. The biopsy came back EMPD. The dermatologist had previously worked at Moffitt Cancer center in Tampa; and was able to get me in for consult land surgery. One side of my labia was completely removed; and the other side was partially removed. They suspected that is was non-invasive.
Shortly after, they suspected it was invasive because my lymph node in my groin became enlarged.
Groin lymph node biopsy came back as having triple negative breast cancer. Subsequent 3-D mammogram and PET scan showed no breast cancer.
I was started on Taxotere chemo since they did not know what I really had or what to do with me. Tumor boards at both Moffitt and Florida Cancer Center in Tavares, FL were convened.
more.......if interested and/or important. I would like to enroll in the clinical observational study you mentioned in your previous response.

Thanks for sharing more of your diagnosis story. I'm not surprised that this is a cancer that often goes unrecognized. To find out more about your elibility and enrollment in the study, please contact Mayo Clinic Clinical Cancer Trials. Here's the info:

CANCER-RELATED CLINICAL STUDIES QUESTIONS
https://www.mayo.edu/research/clinical-trials
Phone: 855-776-0015 (toll-free)
Contact form https://www.mayo.edu/research/forms/cancer-clinical-trials

Thank you for the information. I filled out the information for a EMPD clinical trial.
My history and experience will be helpful to early diagnosis.
Nancy Gilligan

Hi all. New to this page and just diagnosed with EMPD after a year of missed diagnoses. Saw oncologist last week and having surgery next week. Procedure will be wide excision; cancer is now down both right and left labia. Doc will also scope bladder and anus while I’m under anesthesia. Have been rushing to get several tests done to check for spread: pelvic ultrasound, sigmoidoscopy, chest X-rays, cardiology consult (am open heart patient 2x), lots of blood work, and ekg. I’m 65 so while I’m dreading disfigurement, I’m long married and not in the dating pool ~ I’m so sorry for those who are. My big concern is pain afterwards as doc said it’s not unusual to pop a couple stitches because of where they’ll be. Oncologist said 40% of EMPD patients have cancer somewhere else, but hey that means 60% don’t:) She also says it’ll be managed from here on with surgery (unless other organs are involved then chemo, etc.) because since it has such a high rate of recurrence they’ve found chemo and/or radiation at this point probably wouldn’t make a difference. I get it, but I’m only going to go so far with surgeries.

Anyway, best of luck to anyone dealing with EMPD. Hopefully my story has helped someone:)

I love your positive attitude! EMPD I was misdiagnosed for 6 years by multiple gynecologists and dermatologists who prescribed steroid creams snd/or herpes medication. 2021 a biopsy revealed EMPD. Surgery was easy, no pain afterwards and fast healing. Although doctors thought it was non-invasive, it was not to be. One month later it had spread to my lymph nodes.

Oh no. How are you doing now? I’m so sorry to read that it’s spread. I’ve read EMPD is often misdiagnosed ~ but for 6 years? That’s terrible. I’m sending you good thoughts and I appreciate your comment.

@catlazz, it's high time I check in with you. How did surgery go? I'm hoping the scoping of the bladder and anus didn't reveal any unwanted surprises. How is the recovery?

Thanks so much for the check in! It’s been 2 weeks since surgery and all is ok. There’s been very little pain which is saying a lot for 30+ stitches in my vulva:) Doc just messaged me yesterday to say there was no underlying cancer according to pathology but that there are multiple places where the margins aren’t clean. No cancer anywhere else so far!! This is a really frustrating cancer because the only treatment is more surgeries. Doc said even if it spread and the new spread was treated with radiation/chemo, the Paget’s of the vulva would not respond. So it seems I’ll always have an active cancer of the vulva and I’ll be checked every 3 months for the next few years. But, as I’ll tell anyone who’ll listen, I am actually lucky because I know I have cancer, vs so many who have it and don’t know. 🙂

My EMPD has now spread to my liver and my spine(L-4). Radiation of the L-4 starts tomorrow. 10 consecutive treatments. Too many tumors on the liver to radiate or remove surgically. I was told that my EMPD is “a fast moving train.” We need to stop
or slow down the spread. I am still getting immunotherapy ever 3 weeks and have refused chemo. I do not want to spend whatever time I have left as sick as chemo has made me.