Vulvar Cancer: Types, Symptoms, Causes And Treatment

Vulvar cancer is also termed as vulval cancer. It is a comparatively rare type of cancer that grows on the female external genitals. The vulva is the area of skin surrounding the urethra and vagina, including the clitoris and labia. It consists of the inner and outer lips of the vagina, the clitoris, and the opening of the vagina, and glands near the vaginal opening. The cancer mostly appears on the outer vaginal lips and accounts for around 0.6 per cent of all cancers in women ^[1] .

However, the cancer can affect other parts of the vulva as well, especially when it begins to spread. The cancer develops at a slow pace, initially beginning as vulvar intraepithelial neoplasia by affecting the healthy skin cells around the vulva. It causes a lump or sore on the vulva that causes itching and is mostly diagnosed in older women ^[2] .

Vulvar cancers can be easily diagnosed and immediate medical attention in its early stages can help cure the cancer. A lack of treatment can result in the abnormal cells turning into malignant cancer ^[3] .

Types Of Vulvar Cancer

The disease is categorised into five major categories ^[4] .

1. Squamous cell carcinoma

It is the most common type of vulvar cancer and affects the skin cell first. About 90 per cent of vulvar cancers are squamous cell carcinoma. It affects the flat, outer layers of skin and the symptoms take several years to be noticeable. Some cases of squamous cell carcinoma have been linked to the human papillomavirus (HPV), which is found mostly in younger women ^[5] .

2. Vulvar melanoma

About 10 per cent of the vulvar cancers are reported to be vulvar melanoma. It develops as a dark patch, like discolouration in the skin and is the second most common type of vulvar cancer. It is mostly reported in Caucasian women aged between 50-80 years. It can also affect young women and are distinguished into three types, superficial spreading, nodular, and acral lentiginous melanoma. Melanoma has a high risk of spreading to other parts of the body ^[6] .

3. Sarcoma

It starts in the bone, connective tissue cells or the bones. Sarcomas are mostly malignant and can develop at any age (even childhood)^[7] .

4. Verrucous carcinoma

This type is a subtype of squamous cell cancer. It appears in the form of a wart and gradually develops with time^[8] .

5. Adenocarcinoma

It develops in the glandular tissue and accounts for a very small proportion of the vulvar cancers^[7] .

Symptoms Of Vulvar Cancer

The initial sign indicating the onset of the cancer is a lump that causes itching, irritation, or bleeding ^[9] .

The most common symptoms of the cancer are as follows ^[10] :

• Wart-like growths
• Abnormal vaginal bleeding or tenderness
• Pain and burning
• Painful sexual intercourse
• Painful urination
• Persistent itching in the vulvar area
• Rawness and sensitivity
• Dark discolouration in cases of melanoma
• Ulceration
• Thickened skin

Causes Of Vulvar Cancer

Usually, cancer develops when the body is not able to control cell growth. The mutations in the DNA cause the cell to grow and divide rapidly. These cells form a cancerous tumour that can spread to the other parts of the body^[11] .

The cancer can spread in the event of two things, when a cancerous cell manages to move through the body using the blood or lymph systems, destroying the healthy tissue. It can also be caused when a cancerous cell manages to move through the body using the blood or lymph systems, destroying the healthy tissue ^[12] .

Risk Factors Of Vulvar Cancer

Although there is uncertainty over the exact cause behind cancer, the following factors can increase the chance of developing vulvar cancer ^{[13] [14] [15]} .

Age: More than half of the women who have been diagnosed with vulvar cancer are aged above 70 years.

Vulvar intraepithelial neoplasia (VIN): It is a sort of precancerous state where certain cells within the vulvar epithelium have low levels of carcinoma. Women with VIN have a higher chance of developing vulvar cancer.

Human papillomavirus (HPV): Women infected with HPV are increasingly prone to developing vulvar cancer.

The other major risk factors pertaining to the development of vulvar cancer are as follows ^[16] :

• Having a skin condition that can affect the vulva, such as lichen planus
• Melanoma
• Sexually transmitted infections (STIs)
• Smoking
• Kidney transplant
• Human immunodeficiency virus (HIV)
• Systemic lupus erythematosus
• Psoriasis
• Radiotherapy
• Weak immune system
• Having a history of precancerous conditions of the vulva
• Having a history of genital warts

Diagnosis Of Vulvar Cancer

In order to understand the extent of your condition, the doctor will begin by checking your overall health. He/she will examine your daily habits, lifestyle, and medical history. Then, the doctor will examine your vulva for the possibilities of lumps or anything unusual ^[17] .

A colposcopy will be conducted using a special magnifying device to examine your vulva. The magnifying glass will help determine the possible growth of any abnormalities.

A cystoscopy will be conducted where your bladder will be examined so as to determine whether cancer has spread to that area.

A proctoscopy will be carried out where your rectum will be examined so as to check whether cancer has spread to the rectal wall.

The diagnosis may also involve imaging scans which will help the doctor in determining whether cancer has spread, and if so, where to. An MRI scan or a CT scan will be used. An X-ray may also be used so as to check whether cancer has reached the lungs ^{[18] [19]} .

Staging Of Vulvar Cancer

If the diagnosis and the following biopsy confirms the presence of vulvar cancer, with the help of the imaging scans the doctor will stage cancer. It helps the doctor in classifying the severity of cancer and thereby aid in developing a suitable treatment plan for you ^[20] .

The location of the primary tumour, the spread of cancer to nearby lymph nodes, and the size and number of tumours are the factors that impact the staging.

Vulvar cancer is staged from 0 to 4 and higher the stage, severe the condition ^{[18] [21]} .

Stage 0: It refers to very early cancer, which is confined to the surface of the skin of the vulva.

Stage 1: The cancer is limited to the vulva or perineum (area of skin between the vaginal opening and anus). Its size goes up to 2 centimetres.

Stage 2: The cancer has spread from the vulva to nearby structures. And has affected the lower portions of the urethra, vagina, and anus.

Stage 3: The cancer has spread to the lymph nodes.

Stage 4A: The cancer has extensively spread to the lymph nodes and to the upper portions of the urethra. The bladder, rectum, or pelvic bone are affected at this point.

Stage 4B: The cancer has spread to the distant organs and lymph nodes.

Treatments For Vulvar Cancer

The stage and severity of the cancer determine the treatment plan. The four main treatment methods used in the event of vulvar cancer are mentioned below ^{[22] [23]} .

1. Surgery

It is the primary treatment method adopted for treating vulvar cancer. It helps in removing the cancer without causing any problems in one's sexual function. In the initial stages, limited surgery will help get rid of the tumour. However in the later stages when the cancer has spread to nearby organs, such as the urethra or rectum, extensive surgeries are required ^[23] .

The surgeries involved are as follows:

Laser surgery, where the laser beam is used as a knife to remove the lesions.
Excision, where the surgeon attempts to remove all the cancer and surrounding tissues.
Skinning vulvectomy, where the surgeon removes the top layer of skin with the cancer.
Radical vulvectomy, where the surgeon removes the whole vulva (including the clitoris, vaginal lips, the opening to the vagina, and the nearby lymph nodes).

2. Radiation therapy

This will aid in shrinking the deep lesions or tumours before surgery, making it easier to remove it. It makes use of high-powered energy beams, such as X-rays and protons, to kill the cancer cells. It also helps in relieving the symptoms and improving the quality of life.

3. Chemotherapy

The aggressive form of chemical drug therapy aids in slowing down the growth of the cancer cells, it also stops the spreading of the cells. Chemotherapy is applied when the cancer is more advanced and has spread to other organs in the body. It is often combined with radiation therapy to shrink large vulvar cancers.

4. Biologic therapy

It is a form of immunotherapy and uses synthetic or natural substances to help the body fight against the cancer ^[24] .

Prevention Of Vulvar Cancer

You can reduce your risk of developing the condition by following the below-mentioned points ^[25] .

• Practising safe sex
• Limiting your number of sexual partners
• Having the HPV vaccination
• Attending scheduled cervical smear tests
• Not smoking