Vulval and vaginal mucosal melanoma

Vaginal mucosal melanoma affects the lining of the vagina, and can also involve the cervix. Mucosal melanoma can also be present solely in the vulva. These types of melanomas are extremely rare, accounting for only 3% of cancers in this part of the body.  

Types of vulval and vaginal melanoma

Vaginal mucosal melanoma can be found anywhere in this part of the body, but is usually found in the vaginal wall in the lower third of the vagina.

It starts on the pigment-producing cells (melanocytes)  in the lining of the vagina. The melanoma may be darker than the surrounding areas, but in some cases it does not have more pigment or is colourless (amenalotic). 

The vulva contains both skin and mucous membranes. Melanomas that develop on the inner surfaces (mucous membranes) are known as mucosal melanomas. Melanomas that develop on the outer skin of the vulva are described as cutaneous (skin) melanomas and these are the same type of melanoma that can develop on other areas of the skin. For treatment of cutaneous melanoma see our melanoma stages and treatment guide.

What are the causes?

The causes of vaginal and vulvar mucosal melanoma remain largely unknown and may be a combination of age, genetics and environmental factors. Vaginal and vulvar melanoma can be found in women of any age, but it is most common in women over the age of 50.

Symptoms

The most common symptom of vaginal or vulvar melanoma is unexpected bleeding from the area, (bleeding that occurs between periods or after the menopause).

Other possible symptoms include: 

  • Pain in the vagina
  • Unusual vaginal discharge that may include blood
  • A lump or growth in the vulva or vagina
  • Itching in the vagina or vulva that has no other cause and doesn’t go away with common treatment 

It is important to note that 1 in 10 of these types of cancer do not have symptoms, and may be found in a routine cervical screen.

The symptoms are often similar to other symptoms of cancer and conditions in the vaginal region. They can also have a non-cancer related cause. It is therefore important to arrange an early appointment with your GP, so they can take the most appropriate action quickly.

If your GP cannot find another cause for these symptoms, you should be referred quickly (normally within about two weeks) to a clinic, in a specialist hospital for tests. This often involves an appointment with a gynaecological cancer consultant. You may also see melanoma specialists and nurse specialists.

Vaginal and vulvar melanoma: Diagnosis and staging

It is important to see your GP if you have symptoms or any ongoing unexplained symptoms that are worrying you. If your GP has any concerns or they are not sure what the problem is, they will refer you to a hospital specialist who will examine you and will arrange for you to have a biopsy. This is a small sample of the tissue that will be removed under local anaesthetic to look for cancer cells. It is then analysed by a pathologist (a specialist doctor who diagnoses diseases from tissue and cells in the body) to determine whether it is a melanoma or not. It typically takes a few weeks to receive the results of your biopsy and this can be a very anxious time. You may have other tests to help diagnose vulvo-vaginal melanoma and these tests can also be used to check whether your melanoma has spread to other parts of the body.
 

Determining your stage for vaginal and vulvar mucosal melanoma 

After a diagnosis of vulvo-vaginal mucosal melanoma, your medical team will work out the ‘stage’. The stage is a way of describing the key features of your melanoma and is helpful in guiding the best treatment and follow-up for you. Your stage provides an indication of how advanced your melanoma is. Generally, the higher the stage, the more advanced it is.

For most melanomas of the skin (cutaneous melanoma), doctors use an internationally recognised staging system called the TNM system. This system is developed by organisations such as the American Joint Committee on Cancer ( AJCC). It describes the tumour (T), whether cancer has spread to nearby lymph nodes (N), and whether it has spread to other parts of the body (M, metastasis).

However, there is currently no internationally agreed staging system specifically for vulvo-vaginal mucosal melanoma. For this reason, doctors should still record the tumour using similar TNM terms, as this helps ensure important clinical information is captured consistently.

Since there is no agreed staging system, vulvo-vaginal mucosal melanoma is often staged using the Ballantyne staging system:

Stage 1 = the melanoma is ‘localised’ which means it is in the vulvo-vaginal area only and has not spread anywhere else
Stage 2 = the melanoma has spread to nearby lymph nodes. (AJCC equivalent staging for cutaneous melanoma would be Stage 3)
Stage 3 = the melanoma has spread (metastasised) to another part of your body (AJCC equivalent staging for cutaneous melanoma would be stage 4)

Vaginal and Vulvar melanoma: Treatment 

Surgery

The main treatment for vaginal or vulvar mucosal melanoma is surgery. This is often in a specialised centre, and there should be support from an experienced cancer team.

There are a number of different approaches to surgery for vaginal and vulvar mucosal melanoma, depending on its location and how advanced it is. In planning the operation, the medical team will consider the stage of your vaginal or vulvar mucosal melanoma, your general health and how the surgery may affect your quality of life.

Since this is an intimate part of your body, you may want further support or counselling to help with the emotional aspects and consequences of surgery. Your medical team should be able to provide you with details.

In some situations, you may be offered immunotherapy or a targeted therapy after the surgery. This is known as adjuvant treatment and is given to reduce the risk of the mucosal melanoma coming back. In some cases, immunotherapy may be given before surgery (known as neoadjuvant treatment). Your specialist cancer team will discuss whether these treatments may be suitable for you.

Other possible treatments

If the vaginal or vulvar mucosal melanoma has spread to other parts of the body, you may be offered other treatments such as immunotherapy, targeted therapy or the option of taking part in a clinical trial. 

For more information on immunotherapy treatment, click here.

For more information on targeted therapy, click here.

For more information on clinical trials, please visit the Melanoma TrialFinder. 

If you have any questions about your diagnosis or treatment, our specialised Melanoma Helpline nurses are here to support you on

0808 801 0777

Patient Stories

Philippa’s Story:

 

On Christmas day in 2021, Philippa found a small lump in her vulva and after being referred by her GP, was diagnosed with vulval mucosal melanoma. During January 2022 she had an initial operation to remove this and in March 2022 she had a single lymph node removal. Following on from this in May 2022 a PET scan revealed metastatic lymph node outside of the pelvis and Philippa started double immunotherapy treatment (ipilimumab+nivolumab). She underwent further surgeries and her latest scan shows she is cancer-free.

Read Philippa’s full story

References

Carvajal RD, Spencer SA, Lydiatt W. Mucosal Melanoma: A Clinically and Biologically Unique Disease Entity. J Natl Compr Canc Netw (2012) 10(3):345–56. doi: 10.6004/jnccn.2012.0034

Wohlmuth C, Wohlmuth-Wieser I, May T, Vicus D, Gien LT, Laframboise S. Malignant Melanoma of the Vulva and Vagina: A US Population-Based Study of 1863 Patients. Am J Clin Dermatol. 2020;21(2):285-295. doi:10.1007/s40257-019-00487-x

Hou JY, Baptiste C, et al., Vulvar and vaginal melanoma: A unique subclass of mucosal melanoma based on a comprehensive molecular analysis…Cancer. 2017 Apr 15;123(8):1333-1344. doi: 10.1002/cncr.30473. Epub 2016 Dec 27. PMID: 28026870.

Rambhia PH, Scott JF, Vyas R, et al. Genitourinary Melanoma. Chapter 5. Available from: https://www.ncbi.nlm.nih.gov/books/NBK506990/ doi: 10.15586/codon.noncutaneousmelanoma.2018.ch5

American cancer society. Staging for Vulvar Mucosal Melanoma https://www.cancer.org/cancer/vulvar-cancer/detection-diagnosis-staging/staging.html