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 cells 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 cancer, 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 prognosis 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 nodes 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 infection. 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 melanoma, 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 tissue).
- 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, metastasis (whether the cancer has spread to other parts of the body) and how the cancer cells look under the microscope. Staging 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 Gynaecology 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, bladder and/or bowel. This is also known as localised cancer.
- Stage III: the cancer has become larger and has spread beyond the pelvis into the lining of the abdomen (peritoneum). Lymph nodes are also often affected. This is also known as advanced 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 (tumour) indicates the size and depth of the tumour.
- N (nodes) 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 tissue.
- 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:
- Grade 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 testing, 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:
- Surgery, potentially including:
- Local excision (only the tumour is removed – precancerous areas).
- Wide local excision (tumour and part of surrounding area (margin) removed).
- Partial radical vulvectomy (partial removal of the vulva).
- Total radical vulvectomy (complete removal of the vulva).
- Lymphadenectomy (removal of affected lymph nodes).
- Reconstructive surgery (surgery to restore appearance and/or function to vulva).
- Radiation therapy.
- Chemotherapy.
- Clinical trials.
- Palliative care.
For more information on the treatment options, please refer to the Rare Cancers Australia Treatment Options page.
Risk factors
While the cause of vulvar cancer remains unknown, the following factors may increase your risk of developing the disease:
- Having been diagnosed with a vulvar intraepithelial neoplasia (VIN).
- Having been diagnosed with HPV.
- Abnormal cervical screening test results.
- Skin conditions, including:
- 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.
Early 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.
- Blood, 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 groin area.
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Diagnosis/diagnosing
If your doctor suspects you have vulvar cancer, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment.
Pelvic examination
The doctor will inspect the abdomen for any swelling or masses, followed by your genitalia. The doctor will then insert two fingers into your vagina while simultaneously pressing on your abdomen with their other hand to feel your uterus and ovaries. Following this, the doctor may use a device called a speculum into your vagina, which will separate the vaginal walls and allow viewing of your vaginal canal and cervix for any visible abnormalities. You many request a family member, friend, or nurse to be present during this exam.
Imaging & Blood tests
The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), pelvic ultrasound (including abdominal and trans-vaginal ultrasounds) and/or positron emission tomography (PET scan), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and looks for signs of metastasis. Additionally, a blood test may be taken to assess your overall health and help guide treatment decisions.
Colposcopy
A colposcopy is a type of pelvic examination that looks closely at the cervix and vagina. Your doctor will use a speculum to separate the walls of the vagina, and apply a vinegar-like liquid and iodine to the cervix and vagina to help highlight any abnormal areas. The colposcope (a microscope with a light) is placed near your vulva, but does not enter your body. If any abnormalities are observed, a biopsy may be taken.
Cystoscopy
A cystoscopy is a procedure that examines the bladder and urethra. A long, thin tube, with a light and a camera attached (cystoscope) is inserted through the urethral opening and into the bladder to determine if there are any abnormalities. This can be done under a local or general anaesthetic, and is often performed as a day procedure.
Proctoscopy
A proctoscopy is a procedure that examines the rectum and the anus. A long, thin tube, with a light and a camera attached (a proctoscope) is inserted through the anus and into the rectum to determine if there are any abnormalities. This can be done under a local or general anaesthetic, and is often performed as a day procedure.
Biopsy
Once the location of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. This is often done by a fine needle aspiration (FNA), a core needle biopsy (CNB), or during a procedure (such as a colposcopy or large loop excision of transformational zone (LLETZ)). These samples will then be analysed for cancer cells.
Prognosis (Certain factors affect the prognosis and treatment options)
While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on the rate and depth of tumour growth, susceptibility to treatment, age, overall fitness, and medical history. Generally, early-stage vulvar cancers have a better prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence. It is very important to discuss your individual circumstances with your doctor to better understand your prognosis.
References
Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.