Vulvar cancers are rare malignancies of the vulva, which is a collective term for the external genitalia in females. The functions of the vulva include protection of the female reproductive system, assisting in sexual arousal and stimulation, and facilitating sexual intercourse via lubrication.
The vulva is made up of five main components. The outer-most section of the vulva is known as the mons pubis, which is the rounded area in front of the pubic bones that functions as a source of cushioning during sexual intercourse. Just below this is the labia majora, also known as large lips, which are two large folds of skin that protect the rest of the vulva. Inside the labia majora is the labia minora, also known as small lips, which are slightly smaller skin folds that protect the vaginal and urethral openings. At the top of the vulva on the labia minora is the clitoris, which is the main organ for sexual pleasure in females. Inside the labia minora are the Bartholin glands, which are two small glands that function to lubricate the vagina.
Vulvar cancers are most commonly found in women who have gone through menopause or over 60 years old, however, it can affect anyone with female genitalia – including women, teenagers, transgender men, non-binary individuals, and intersex people – at any age.
Types of Vulvar Cancer
There are several types of vulvar cancers, which are categorised by the type of cellsthe basic structural and functional unit of all living things they originate from.
Squamous Cell Carcinoma (SCC)
Squamous cell carcinomas (SCCs) are the most common subtype of vulvar cancers that develops from the squamous cells that line organs. The four main types of vulvar SCCs include keratinising SCCs, basaloid SCCs, warty SCCs and verrucous carcinomas.
Keratinising SCCs
Keratinising SCCs are the most common subtype of vulvar cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs, and are classified by the development of excess keratin within the cancer cells. Keratin is a protective protein that is most commonly found in the hair and nails, but can be found in other parts of the body. Keratinising SCCs can be aggressive, and may not have as good of a prognosisto predict how a disease/condition may progress and what the outcome might be as other vulvar cancers.
Basaloid SCCs
Basaloid SCCs are a rare subtype of vulvar cancer, and originate from squamous cells that look similar to basal cells. Basal cells are often found in the bottom layer of the epidermis in the skin, and are responsible for producing new skin cells as the old ones die. Basaloid SCCs are often aggressive, and may not have as good of a prognosis as other vulvar cancers.
Warty SCCs
Warty SCCs, or condylomatous carcinomas, are a rare subtype of vulvar cancer that is often misdiagnosed as genital warts. Warts are abnormal skin growths that are often caused by strains of the human-papillomavirus (HPV). Warty SCCs are tumours that resemble warts, and have also been shown to have a link to HPV. They are often slow-growing and less aggressive than other forms of vulvar cancer, but may metastasise to lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid and other parts of the body. Warty SCCs often have a good prognosis.
Verrucous Carcinomas
Verrucous carcinomas are a very rare variant of SCCs, and very rarely present in vulvar cancers. Much like warty SCCs, they often present as slow-growing wart-like tumours and may be linked to an HPV infectiona condition where harmful pathogens, such as bacteria, viruses or parasites, have entered the body. However, warty SCCs have a different cellular appearance, and rarely metastasise. Verrucous carcinomas are often nonaggressive, and usually have a good prognosis.
Mucosal Melanoma
Melanomas are a type of cancer that develop from melanocytes, which are the cells that produce pigment. While these are generally in the skin, they can also occur in mucosal surfaces. Vulvar melanomas are very rare. For more information about mucosal melanomaa type of cancer that develops from melanocytes, which are the cells that produce pigment generally in the skin (but can develop in other areas of the body), please refer to the Rare Cancers Australia Melanoma (Mucosal) page.
Rare types of Vulvar Cancer
These types of vulvar cancers are very rare:
- Vulvar sarcomas (cancer of the vulva arising from soft tissuetissue/the material that joins, holds up or surrounds inside body parts such as fat, muscle, ligaments and lining around joints).
- Vulvar adenocarcinomas (cancer of the vulva arising from mucus-producing glands, such as the Barothin glands and other vulvar glands).
- Paget’s disease of the vulva (cancer of the vulva arising from Paget cells).
- Vulvar basal cell carcinomas (cancer of the vulva arising from basal cells in tissues that line organs).
Treatment
If a vulvar cancer is detected, it will be staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you.
FIGO Staging System
Gynaecological cancers, such as vulvar cancers, can be staged using the Federation of Gynaecologythe study of the female reproductive system and related diseases and Obstetrics (FIGO) system from stage I to IV:
- Stage I: cancer cells are confined to the vulva only. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby organs in the pelvis, such as the ovaries, fallopian tubes, uterus, bladdera hollow, muscular sac in the pelvis that stores urine and/or bowelportion of the digestive system that digests food (small bowel) and absorbs salts and water (large bowel); also called intestines. This is also known as localisedaffecting only one area of body cancer.
- Stage III: the cancer has become larger and has spread beyond the pelvis into the lining of the abdomenstomach, stomach area, belly (peritoneumthe membrane that lines the abdominal cavity). Lymph nodes are also often affected. This is also known as advancedat a late stage, far along or metastatic cancer.
- Stage IV: the cancer has spread to more distant organs, such as the lungs or the liver. This is also known as advanced or metastatic cancer.
TNM Staging System
The TNM system can also be used to classify a vulvar cancer. The TNM system is comprised of:
- T (tumoura tissue mass that forms from groups of unhealthy cells) indicates the size and depth of the tumour.
- N (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissuea group of cells that work together to perform a function.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment Options
Treatment is dependent on several factors, including location, stage of disease and overall health.
Treatment options for vulvar cancers may include:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, potentially including:
- Wide local excisionremoval of cancerous tissue and a margin of healthy tissue around it to prevent cancer recurrence.
- Vulvectomycomplete or partial removal of the vulva.
- Lymphadenectomysurgical removal of lymph node(s).
- Reconstructive surgerysurgery to restore appearance and/or function to an area of the body.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
While the cause of vulvar cancer remains unknown, the following factors may increase your riskthe possibility that something bad will happen of developing the disease:
- Having been diagnosed with a vulvar intraepithelial neoplasia (VIN).
- Having been diagnosed with HPV.
- Abnormal cervical screening testa screening test that looks for signs of human papillomavirus (HPV) and abnormal cells in the cervix results.
- Skin conditions, including:
- Vulvar lichen planus.
- Vulvar lichen sclerosis.
- Extramammary Paget’s disease.
- Having previously been diagnosed with a cervical or vaginal cancer.
- Having a history of smoking.
- Having a weakened immune system.
Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Symptoms
Patients with vulvar cancer may appear asymptomatic in the early stages of the disease. As the cancer progresses, some of the following symptoms may appear:
- Itching and/or burning of the vulva.
- A painless lump, sore, swelling, and/or wart-like growth on the vulva.
- Thickened, raised skin patches (often red, white or brown in colour) on the vulva.
- An unusual mole on the vulva.
- Bloodthe red bodily fluid that transports oxygen and other nutrients around the body, pus, or abnormal discharge from an area of skin or a sore spot on the vulva (often accompanying a strong and unusual odour).
- Bleeding not related to menstruation.
- Tenderness in the vulvar area.
- An ulcer on the vulva that won’t heal.
- Hard and/or swollen lymph nodes in the groinarea between the abdomen and thighs area.
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Diagnosis
If your doctor suspects you have a vulvar cancer, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Pelvic examinationa physical exam of the external and internal female pelvic organs.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- Pelvic ultrasounda type of medical imaging that uses sound waves to create detailed images of the organs and structures within the pelvis, including the uterus, ovaries, and surrounding tissues.
- PET (positron emission tomography) scana type of medical imaging that uses radioactive tracers to create detailed images of the body.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Colposcopyan examination of the cervix and vagina using a small, flexible instrument known as a colposcope.
- Cystoscopyan examination of the bladder and urethra with a small, flexible instrument known as a cystoscope.
- Proctoscopyexamination of the rectum and anus with a small, flexible instrument called a proctoscope .
- Biopsyremoval of a section of tissue to analyse for cancer cells.
