Vulval cancer

Clinical picture

The vulva is the collective name for the female external reproductive organs. They consist of the labia, the clitoris, the vaginal entrance and the perineum (the area between the vagina and the anus). Vulval cancer arises when there is uncontrolled malignant cell growth in this anatomical area.

There are several types of vulval cancer, depending on their origin.

Squamous cell carcinoma is the most common type of vulval cancer.
Basal cell carcinoma grows from the deepest layer of cells of the epidermis (the skin).
Adenocarcinoma grows from glandular cells in the vulva.
Melanoma grows from pigment cells.

This chapter will only discuss squamous cell carcinoma, as the other types of vulval cancer are extremely rare.

Patients with vulval cancer often have had a pre-stage of cancer. Before actual cancer is diagnosed, there are some abnormalities that, although benign in origin, can develop into cancer.

The number of women diagnosed with vulval cancer has been rising steadily. Most women are over 60 years old when they are diagnosed and in 70% of all cases the cancer is diagnosed as stage I. These patients have a 5-year survival rate of 90%. This number drops sharply to 19% for women who are diagnosed with a stage IV cancer.

Symptoms

Vulval cancer is one of the few cancers that occurs on the exterior of the body, which explains why so many women are diagnosed with stage I. Patients with an early stage of vulva cancers may present:

itching
pain or burning sensation
lesions
skin discolouration

In later stages, women may also show the following symptoms:

vaginal discharge
pain or burning sensation during urination
swelling of the vagina

Cause

Certain risk factors have been identified:

Lichen sclerosus of the vulva (LSV): a benign condition that affects the skin of the labia. It can cause skin to become thinner and can cause discolouration. This condition mainly affects women over 60 years old.
Vulvar intra-epithelial neoplasia (VIN): this is caused by the human papilloma virus (HPV). This virus is sexually transmitted, but in most cases the immune system is able to get rid of the virus. In some cases, the virus causes restless cells in the vulva, resulting in discolorations and vaginal warts. VIN is mostly found in women between the ages of 30 and 40.

Smoking does not directly cause vulval cancer but does diminish the body’s ability to fend off the HPV virus.

Diagnosis

A GP who suspects a patient may have vulval cancer will refer their patient to a gynaecologist for further examination. The gynaecologist will perform a biopsy and sometimes blood and urine testing. If these indicate the presence of tumorous cells, subsequent tests are needed in order to determine the tumour stage and to see whether the cancer has spread. These tests include: ultrasound, lung X-ray, CT scan, MRI scan, PET-CT scan and endoscopy. A sentinel node procedure can help determine whether cancer has spread to nearby lymph nodes.

After all these tests, the cancer can be graded into one of four stages.

Stage I: The tumour only grows in or on the vulva and has not spread into the lymph nodes
Stage II: The tumour has grown to other nearby areas but not into the lymph nodes
Stage III: As stage I or II, but with cancerous cells found in the lymph nodes of the pelvis
Stage IV: The cancer has spread into the organs around the vulva and possibly further into the body

Treatment

If a woman is diagnosed with a precursor to vulval cancer, treatment will focus on preventing the cancer from actually developing, and includes:

certain creams and ointments (this can provide relief and even cure the disease)
removing the affected area by means of laser light
surgery to remove the suspicious areas

The above treatments can also be combined. Sometimes there are suspicious conditions, in which case a woman remains under medical surveillance.

If the diagnosis is cancer, the patient may be treated by surgery, radiation and chemotherapy, or a combination thereof. Surgery is usually the first option. Research into the use of targeted therapy is also underway in this cancer setting.