Melanomas of the vulva and vagina.

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6/9/2013 4:15pm
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An internet friend and I run a Facebook information and support group for those with vulva and vagina cancers and related issues.

I've been searching the internet for information about melanomas of the vulva and vagina. This is a summary of my current understanding of the topic, based solely on what I've found on the internet. I stress that I'm not medically trained, but I do have a science degree from a university which does help me considerably in understanding articles and scientific papers and determining how reliable they may be. I thought it worthwhile to post the information I've found into this forum, as melanomas of the vulva and vagina are rare and there's not much info about them on the internet.

The American Cancer Society on its website says "Around 5 of every 100 melanomas in women occur on the vulva, usually on the labia minora and clitoris. They make up about 6 of every 100 vulvar cancers."

There are two kinds of vulva melanoma (perhaps more but I haven't seen any reference to any others) - cutaneous and mucosal. Both start in pigmented cells called melanocytes.  

Cutaneous melanoma of the vulva starts in melanocyte cells in the skin, usually on the outer (large) labia. It's the same kind of melanoma that occurs elsewhere on the skin, the one that is commonly caused by too much exposure to ultraviolet light in sunlight or the ultraviolet light used in tanning beds, and sometimes it's a consequence of inherited genes.

Mucosal melanoma starts in melanocytes in mucosal membranes that line some of the inner parts of the vulva including the inner lips and near the entrance to the vagina. **

Mucosal membranes can contain considerable numbers of melanocyte cells. It's not yet known for sure why melanocyte cells occur in mucosal tissue but they do, and they can give rise to mucosal melanoma in the vulva, vagina, cervix, anus, sinuses, mouth and other parts of the body where mucosal tissues occur.

Cutaneous melanoma is the same kind as the skin cancer melanoma most of us have heard about. The earlier cutaneous melanoma is found and surgically removed, the more chance it won't cause any further problems. If cancer cells have detached from the initial site and moved through the lymph system or blood vessels to another site or sites, often firstly to nearby lymph nodes, it's considerably more difficult to treat.

As far as I know, mucosal melanoma is the only kind of melanoma that occurs in the vagina and in the cervix, and it's even rarer in those places than it is in the vulva.

Mucosal melanoma of the vulva and vagina and cervix is so rare that experts are still developing their understanding of how it develops and evolves and the best ways to treat it. As with cutanoeus melanoma, it seems that surgery to remove it while it's in early stages of development currently offers the best chance of preventing further problems. Because the vagina and the inner parts of the vulva are hidden from view to casual observation, mucosal melanoma is less likely to be discovered early on than is cutaneous melanoma.

Scientists and the medical profession are still at an early stage in their understanding of mucosal melanoma of the vulva and vagina and cervix. There's not much information about it on the internet and it's barely or not mentioned on the biggest and best general cancer websites I've searched.

An article published by Dr. Richard D. Carvajal, "Memorial Sloan-Kettering Cancer Center, New York City", gives the clearest introduction to mucosal melanoma I've found on the internet. I've put a link to his article below. He says among other things:

"Melanoma, regardless of where on the body it occurs, originates from normal pigment cells called melanocytes. These melanocytes produce melanin, the vital pigment which protects the skin from damage caused by the sun's ultraviolet rays. Like any other cell in the body, melanocytes can transform into cancerous cells, and when this transformation occurs, the end result is the development of melanoma. Although normal melanocytes are present in the skin, they are also present elsewhere, such as the mucosal surfaces of the body. These mucosal surfaces line the sinuses, nasal passages, oral cavity, vagina, anus, and other areas. In fact, while there are approximately 800 melanocytes per square millimeter in the skin of the abdomen, there are 1,500 melanocytes per square millimeter present within the mucous membranes of the nose and mouth. Like the melanocytes found in the skin, these mucosal melanocytes can also become cancerous. This transformation results in the development of a type of melanoma called mucosal melanoma."

"Although first described in 1856, mucosal melanoma is not a well-known entity due to its relative rarity. Indeed, of the 69,000 cases of melanoma diagnosed in the United States in 2010, less than 5% were mucosal melanoma. Slightly over half of all mucosal melanomas begin in the head and neck region, approximately one quarter of mucosal melanomas arise from the anorectal region, and another 20% arise from the female urogenital tract. Melanomas originating in mucosal surfaces lining the esophagus, gallbladder, bowel, conjunctiva, urethra, and other sites are far less common."

"While melanoma arising from the skin and mucosal surfaces all originate from melanocytes, cutaneous melanoma and mucosal melanoma differ dramatically in their behavior and underlying biology. The initiation and development of cutaneous melanoma is usually associated with a history of sun exposure, particularly during childhood; however, this association is certainly not the case for mucosal melanoma. Indeed, at this time, no clear predisposing exposures, family history, or other risk factors have been established for this disease."

"As with most cases of melanoma, complete surgical removal of the primary mucosal melanoma provides patients with the greatest likelihood of a cure. ... ".

There is some very interesting and reasonably promising research and some trials going on at present on new ways to treat metastatic melanoma, including looking for ways to encourage the body's immune system to destroy the cancer cells. The most promising aspect of this new research and trials to me is that it's working down at the molecular level where the action really is.

If you visit links I posted here, you may encounter statistics, and worst-case scenarios, and discussions and comments that may not be good for your nerves even though most of them probably don't apply to your particular situation!

A paper called "Primary mucosal melanomas: a comprehensive review" provides good information and discussion about mucosal melanoma, albeit rather difficult to understand in places. Its address is http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3466987/

I quoted above from an article by Dr. Richard D. Carvajal above. It's good but it rather emphasises the negative rather than the positives at times, perhaps because its title and chief intent is "A Call to Arms in the Battle against Mucosal Melanoma". Its address is http://www.susanfazio.org/mucosalmelanoma.html

If you've been diagnosed with melanoma of the vulva or vagina you've joined a pretty exclusive club, and at the same time you've joined another very big club. In the US for example the American Cancer Society says "The American Cancer Society's estimates for melanoma in the United States for 2013: About 76,690 new melanomas will be diagnosed (about 45,060 in men and 31,630 in women).

** Wikipedia in an article called "Mucous membrane" on the page http://en.wikipedia.org/wiki/Mucous_membrane says:

"The mucous membranes (or mucosae or mucosas; singular mucosa) are linings of mostly endodermal origin, covered in epithelium, which are involved in absorption and secretion. They line cavities that are exposed to the external environment and internal organs. They are at several places contiguous with skin: at the nostrils, the lips of the mouth, the eyelids, the ears, the genital area, and the anus. The sticky, thick fluid secreted by the mucous membranes and glands is termed mucus. The term mucous membrane refers to where they are found in the body and not every mucous membrane secretes mucus."

"The glans clitoridis, glans penis (head of the penis), along with the inside of the foreskin and the clitoral hood, are mucous membranes. The urethra is also a mucous membrane. The secreted mucus traps the pathogens in the body, preventing any further activities of diseases."

Here's a link to what may be an important news item that just appeared in my inbox via Google News. The link to the item is

http://www.domain-b.com/technology/biotech_pharma/20130608_molecular.html

It starts:
 "Genome sequencing reveals mucosal melanoma's bullseye news 
08 June 2013
"Scientists may have found a molecular 'bullseye' for a rare form of melanoma, opening up opportunities for new targeted treatment, according to new research being published in the Journal of Pathology.Genome sequencing reveals mucosal melanoma's bullseye news
08 June 2013

Genome sequencing carried out at Cancer Research UK's Paterson Institute for Cancer Research at the University of Manchester has revealed that the genetic fingerprint of mucosal melanoma is completely different from that of its more common counterpart – cutaneous or melanoma skin cancer."

Back to my post, if anyone reading this post sees any errors or misinterpretations, or you can add more information, please do post below.

Support groups for those with vulva or vagina cancers or vin or vain can be found by putting "vulva cancer" into the Facebook search window.  

Cheers - Ed.
9th June 2013.

Ed,

    I would like to suggest that you search the MPIP current and archives for both mucusal and c-kit.  I have made many posts about mucosal and the 10-30% of mucosal malanoma's that have either or both the c-kit oncoprotein and one of the c-kit DNA mutations. As you can see from my profile I have anal mucousal c-kit melanoma. 

     While melanoma in the more private areas of ones body is often talked about as being very deadly, this is because it tends to be advanced before being discovered.  A huge number of dermatoloists never check THERE (front or rear). After learning of my problems a lady friend of mine got upset with her female derm about whom she stated " SHE NEVER had me drop my panties!"   There are ladies that have posted that are doing well now after surgery..

     If one has either the c-kit oncoprotein and/or a c-kit DNA mutation there may actually be good news.  About 2001 Imatinib mesylate was FDA approved for 3 types of cancer and has reduced some of those forms from catastrophuic diseases into chronic diseases.  (All three have c-kit factors.)

     About 1/2 of the states have off-label usage laws that mandate that in their states, state regulated insurance companies must cover FDA approved cancer treatments for off-label usage if at least one Oncologist has published one article in a peer reviewed medical journal about the success for the usage your Oncologist wants to make of  it. 

    When the IL-2 stopped working in Dec 2008, My Oncologist put me on Gleevec  in March 2009.  Within 30 days my wildly advancing melanoma was again stabilizing.  It is still stable with slight reductions in the tumor load in mid 2013!  (Imatinib mesylate is an cytostatic targeted chemotherapy that may ar may not induce apoptosis to ones tumor cells.)

      Imatinib mesylate is the first of the tageted chemotherapy drugs approved and like the BRAF drugs, melanoma will in some iinstances find a path around it.   Some people have been on it for over ten years now for CML, GIST and some lymphomas.

     There are at least three additiional drugs along the lines of Gleevec that are being tested on c-kit cancers to detemine which works best on which C-kit DNA mutation.

     I have not heard any results yet regarding anti-PD-1 drugs agaiins mucousl and/or c-kit melanomas.  This is an areas to keep an eye on as well.

    As a targeted chemo Imatinib mesylate has few negative side effects after adaptiing to it, if it is taken with food.  The longest term side effect seems to be fatique which gets worse after a few years on it.

   I will be glad to assist you any way I can.

I'm me, not a statistic. Praying to not be one for years yet.

Golly! Thanks Jerry for your very comprehensive reply and links. Sounds like you're a true mm warrior! I'll study your reply and the links and post a reply in due course - might take me a while :-)

Any idea what genes/DNA mutations the UK article is referring to?  They give a tremendous lack of info as to what was actually found.  I want more detealis!

I'm me, not a statistic. Praying to not be one for years yet.

The only item I've red is the one I've mentioned above, and the abstract to the scientific paper which can be found here http://dx.doi.org/10.1002/path.4204  To read the full paper would require you to part with some hard-earned lucre. I guess it will eventually be published free somewhere on the internet. It does sound like they are following a potentially valuable line of research and we could hope it might lead to new treatment options :-) 

I had a friend with this type of melanoma.  She survived after surgery. 

 

Cindy VT

Ed,

This is a great compiling of info.  it truly is interesting and we're learning more than ever!

Ed,

This is a great compiling of info.  it truly is interesting and we're learning more than ever!

Thanks Cindy and Cooper for commenting on this thread!
Jerry I will advise the ladies in the vulva group about the existence of this thread and of your knowledge and the information and support you are posting here on mpip. I'll suggest they can search mpip for relevant posts, and if they join mpip they can go to your profile and find all your posts listed there :-)  
It's very encouraging to read that you and some other members here are doing well years after a diagnosis of mucosal melanoma. I didn't even know mm existed till a lady posted about it less than a month ago in the vulva group. Thanks again for your detailed post here and your offer of assistance :-)  Good luck and blessings to you and to everyone with an mm visitation on mpip!

I sent Ed an email with many URL's thatrelate.  We often have trouble listing URL's he due to changes in the New MPIP Bulletin Board software.    To such the older posts one has to go to the archives to get posts back to the 1990's.           

    If anyone wants the list I emailed Ed, ask my and I will gladly send the list.

    Ed, Ladies have often been hesitant about giving names when they first fiind us.  This is one of the reasons that we allow people to post as anonymous, after they register.  There is a Glosed Facebook Group that started as a Mucosal Melanoma group. We are "The Morning Glories"  (started by some Ladies, but then they found me and invited me to join.)  They now have some none mucosal members.  Good group of people, some are also on the MPIP.

I'm me, not a statistic. Praying to not be one for years yet.

Thanks Jerry for the info about the Facebook Morning Glories group. I found the group by putting "Morning Glories" into the Facebook search window. Do you think I should put a link to it into my vulva cancer group - is it mostly about mucosal melanoma and do they want more members do you know?  Putting mucosal melanoma into the Facebook search window doesn't turn up any Facebook support groups.
Cheers - Ed

I'm curious if anyone has any more information on cutaneous melanoma? There was a short section describing this type of melanoma but I would love to have more information if possible. I have recently discovered 9 black-blue lesions on my labia majora and have a Dr.'s appointment this Friday to see if it might be vulvar melanoma. I would really like to find out more information on cutaneous melanoma since this form tends to form on the labia majora. If you have any more information on this please let me know. Thank you!

http://med.stanford.edu/clinicaltrials/trials/NCT00700882

A Phase II Trial of Dasatinib in Patients With Unresectable Locally Advanced or Stage IV Mucosal, Acral and Vulvovaginal Melanomas

I'm me, not a statistic. Praying to not be one for years yet.

I'm me, not a statistic. Praying to not be one for years yet.

I also have mucosal melanoma of the vagina - I had surgery in December of 2012 and a round of chemotherapy using temodar and cisplatin which they started to do in China where mucosal melanoma is far more prevelant. Dr. Carvajal, who was mentioned above is not sure if this therapy works but offers it as an option because  there are so few alternatives.

 

I wrote to the Patterson Institute in the UK to see if they were doing any clinical trials for mucosal melanoma - they said they did not have anything to offer that we did not have in the states but suggested that if one is Ckit negative - one should look into the PD-1 trials.

 

I read somewhere, and I can't find where, so I have not gone back to confirm, that the genetic makeup of mucosal melanoma is more like breast and prostate cancer than cutaneous melanoma. I wish I could find the article because I think that would give some insight in how to treat this.

 

I have been doing a lot of research now on the anti PD-1 and the PD-l1 therapies that are being developed. Oncologists are very excited about this because it appears that they work across many cancer lines and are finding different targets to treat that are blocking the immune system from attacking the cancer. What it is really coming down to is that it is becoming less important where the cancer is in the body - it is more important to know the genetic make up of the cancer so the immune system can be encouraged to attack it.

 

Insurance companies have to get on board soon to cover the cost of dna sequencing of cancer tumors - the cost is coming down significantly but this needs to be a standard part of the diagnosis for all cancer patients because this is where the science it going.

 

As for me, I am in remission after the surgery and chemo, but do not expect to stay that way. I cannot get an answer out of my doctor about the efficacy of the chemo. When it comes back, I almost wish that I could get the chemo again and let the science progress before I go into a trial. In the meantime, I am eating extremely well and exercising to get stronger so that I can withstand any therapy that comes my way.

 

I wuold love an opportunity to regularily communicate with someone in a similar position who follows the research.

 

Good luck and good prayers to all.

Mary

 

 

 

 

Mary, glad to interact.  Mucousal melanoma is not even one thing.  It can be several different hings depending on which oncoprotein is expressed  and  which of many different DNA mutations is envolved.  Even when one gets down to the c-kit portion of mucosal melanoma there can be various DNA mutations involved, which might require different targeted drugs to fight.

I'm me, not a statistic. Praying to not be one for years yet.