Vulvar Cancer: Comprehensive Health Information

This information is provided by SSCHRC to help you understand vulvar cancer and its management. It is written in a patient-friendly and medically accurate style.

1. General Information

The vulva is the external part of a woman's genitals, situated at the opening of the vagina (birth canal). Vulvar cancer is categorised based on the specific type of cell in which it originates.

The main parts of the vulva include:

  • • Two skin folds around the opening of the vagina: Outer lips (labia majora), which are larger and have hair, and Inner lips (labia minora), which are smaller and hairless
  • • The Clitoris, which is vital for sexual stimulation
  • • Bartholin glands, one on each side of the vaginal opening, which help to lubricate the vagina during sexual activity

Cancer can develop in any of these areas, and tumours may extend beyond a single site.

Main Types of Vulvar Cancer

Squamous Cell Carcinoma

This is the most common type, originating from the flat cells lining the vulva. A subtype is verrucous vulvar cancer, which may appear as a wart-like growth.

Adenocarcinoma

This typically starts in the Bartholin glands or sweat glands of the vulva, accounting for approximately 8% of all vulvar cancers. Certain conditions, such as Paget's disease of the vulva, can increase the risk of developing adenocarcinoma.

Melanoma

A form of skin cancer that begins in the cells responsible for producing pigment.

Sarcomas

These are rare forms of vulvar cancer that start in the soft tissue and can be found in both children and adults.

Statistics

Vulvar cancer is considered rare. It typically progresses slowly and may start as precancerous changes that can be treated before they become malignant. The median age at diagnosis is 69, and the disease's five-year survival rate is generally about 71%.

Causes and Risk Factors

A risk factor is anything that increases your likelihood of developing vulvar cancer. Key risk factors include:

Demographic & Medical Factors

  • • Age: Over half of women diagnosed with vulvar cancer are over 70 years old.
  • • Vulvar Intraepithelial Neoplasia (VIN): A precancerous condition, usually associated with HPV.
  • • Cervical Cancer: Women with a history of cervical cancer have a higher risk.
  • • Vulvar Skin Conditions: Such as Lichen sclerosus and Lichen planus.

Infections & Lifestyle

  • • Human Papilloma Virus (HPV): Around half of all vulvar cancers are linked to HPV.
  • • Smoking Tobacco
  • • HIV (Human Immunodeficiency Virus)
  • • Melanoma or Atypical Moles: Having melanoma or unusual moles in other parts of the body increases the chance of vulvar cancer.
  • • Family History of Melanoma

Important Note: It is important to remember that not all women with risk factors will develop vulvar cancer. If you have any concerns regarding your risk, you should discuss them with your healthcare provider.

2. Symptoms

The signs and symptoms of vulvar cancer can vary among women. They may include:

Potential Symptoms

  • • A red, pink, or white lump (or multiple bumps) with a rough or scaly surface on the vulva
  • • Burning, pain, or persistent itching in the genital area
  • • Pain during urination
  • • Vaginal bleeding or discharge outside of a menstrual period
  • • A sore on the vulva that fails to heal within a month
  • • A change in the size, colour, or shape of a mole in the genital area
  • • A lump near the opening of the vagina
  • • Pain or bleeding during sexual intercourse

Important Note: These symptoms can also be indicative of other health issues. It is crucial to consult a doctor to discuss any persistent symptoms.

3. Diagnosis

Early and accurate diagnosis is essential for vulvar cancer. Determining the extent and location of any potential cancer spread helps doctors to select the most appropriate treatment plan.

At SSCHRC, our specialised experts utilise modern and accurate equipment for diagnosis. Our pathologists, diagnostic radiologists, and specially trained technicians meticulously determine the exact extent of the disease, which contributes to a higher likelihood of successful treatment.

Biopsy

A biopsy is the definitive method for diagnosing vulvar cancer. This procedure involves removing a small tissue sample, which is then examined under a microscope for cancerous cells. A doctor may use a colposcope (a magnifying instrument) to enhance the view of the area before removing the tissue via:

  • • Excisional biopsy: Removal using a small surgical knife
  • • Punch biopsy: Removal using a tiny punch tool

Imaging Exams

Imaging tests are used to visualise cancer inside the body, helping to locate tumours and track the body's response to treatment. For vulvar cancer, these may include:

  • • CT or CAT (computed axial tomography) scans
  • • MRI (magnetic resonance imaging) scans
  • • PET (positon emission tomography) scans
  • • Chest X-ray
  • • Lymphoscintigraphy

Vulvar Cancer Staging

A cancer's stage describes the size of the primary tumour and how far the cancer has spread within the body. This information is vital for doctors to understand the disease and formulate a comprehensive treatment plan.

The stages of vulvar cancer are set by the International Federation of Gynecology and Obstetrics (FIGO) and range from Stage I to Stage IV. A higher stage number indicates a more advanced, and generally more challenging, cancer to treat.

Stages (I to IV)

Stage I

Cancer is formed and is found only in the vulva.

  • Stage IA: Tumour is 2 centimetres or smaller and has spread 1 millimetre or less into the vulva tissue. Cancer has not spread to the lymph nodes.
  • Stage IB: Tumour is larger than 2 centimetres or has spread more than 1 millimetre into the vulva tissue. Cancer has not spread to the lymph nodes.

Stage II

The tumour is any size and has spread to the lower one-third of the urethra, vagina, or anus. Cancer has not spread to the lymph nodes.

Stage III

The tumour is any size and has spread to the upper two-thirds of the urethra or vagina, the inner lining of the bladder or rectum, or to any number of lymph nodes.

  • Stage IIIA: Cancer is found in lymph nodes in the groin that are not larger than 5 millimetres.
  • Stage IIIB: Cancer is found in lymph nodes in the groin that are larger than 5 millimetres.
  • Stage IIIC: Cancer is found in lymph nodes in the groin and has spread through the outer covering of the lymph nodes.

Stage IV

The tumour is any size and is attached to the bone, or cancer has spread to distant parts of the body, or to lymph nodes that are not movable or have become ulcerated.

  • Stage IVA: The cancer is attached to the pelvic bone or has spread to lymph nodes in the groin that are not movable or have become ulcerated.
  • Stage IVB: The cancer has spread beyond the pelvis to distant sites in the body.

4. Treatment

At SSCHRC, a team of nationally recognised experts focuses on providing the most advanced treatments for vulvar cancer, always prioritising the patient's quality of life. Our therapies are designed to target cancer using advanced methods while striving to minimise side effects.

Our physicians are pioneers in innovative approaches, including sentinel node biopsy procedures. Current standard care for vulvar cancer has progressed to a combination of surgery with other therapies such as chemotherapy and radiation. SSCHRC treats a significant number of women each year for this complex cancer, providing a high level of experience and expertise.

If you are diagnosed with vulvar cancer, your doctor will discuss the optimal treatment options, which are determined by several factors:

  • • The specific type and stage of the cancer
  • • Your overall health
  • • Your age
  • • Your personal preferences

Surgery

Vulvar cancer treatment commonly involves surgery. The success of the surgery is greatly increased when performed by a specialist with extensive experience in the procedure. SSCHRC surgeons are among the most skilled and recognised globally, performing a large volume of vulvar cancer surgeries annually using the least invasive and most advanced techniques.

The main types of surgery for vulvar cancer include:

  • Laser surgery: Utilised for treating precancerous changes, where abnormal cells are burned off with a laser beam. Lasers are not used for invasive vulvar cancer.
  • Wide local excision or simple vulvectomy: These procedures may be used for treating precancerous lesions or small, non-invasive cancer lesions.
  • Radical partial vulvectomy: The goal of this surgery is to remove any invasive cancer on the vulva.
  • Pelvic exenteration: A complex surgery to remove the vulva and lymph nodes in the pelvis. Depending on the cancer's spread, the lower colon, rectum, bladder, uterus, cervix, and/or vagina may also be removed.
  • Full inguinal lymph node dissection: The surgeon removes all the lymph nodes on one or both sides of the groin area. A potential side effect is lymphedema, caused by decreased fluid drainage.
  • Sentinel inguinal lymph node biopsy: Only the first lymph nodes to which the cancer is likely to have spread are removed on one or both sides of the groin. This technique may also cause lymphedema, but it can potentially limit the severity of the condition.

Topical Therapy

This treatment may be used for precancerous vulvar cell changes, involving the application of medicine directly to the growth.

Chemotherapy

Chemotherapy drugs are used to kill cancer cells, control their growth, or alleviate disease-related symptoms. Treatment may involve a single drug or a combination of two or more drugs, tailored to the type and growth rate of the cancer.

Radiation Therapy

Radiation therapy uses powerful, focused beams of energy to destroy cancer cells. Various techniques are available, allowing doctors to precisely target the tumour while minimising damage to surrounding healthy tissue. Radiation treatment is made more precise through advanced delivery techniques and the use of MRI guidance, which helps to minimise impact on healthy tissue.

Immune Checkpoint Inhibitors

These are a form of immunotherapy that prevent the immune system from prematurely "switching off" before the cancer is completely eliminated. Patients may receive a single or a combination of immunotherapy drugs.

Targeted Therapy

Targeted therapy drugs are specifically designed to stop or slow the growth or spread of cancer at a cellular level. Cancer cells depend on specific molecules (often proteins) to survive, multiply, and spread. These therapies interfere with, or target, these molecules or the cancer-causing genes that produce them.