Vulval Cancer

Vulval cancer is a rare form of cancer affecting women and those with vulva. The vulva is the female exterior genital area, which consists of the labia minora and labia majora (lips that surround the vaginal opening), the Bartholin’s glands (two small glands on either section of the vagina) and the clitoris (a sex organ that plays a role in the sexual climax).

Most vulval cancer cases occur in older women and those above 65 years who have vulva. However, this condition is uncommon in females below the age of 50 who haven’t reached menopause.

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Types of Vulval Cancer

Vulval cancers mostly occur in the labia majora and labia minora. The majority of vulva cancer cases are squamous cell carcinomas, a form of cancer that originates in the skin cell. Usually, vulvar squamous cell carcinomas develop when the tiny flat cells that line the vaginal surface mutate or change. These mutated cells tend to multiply rapidly.

Other less prevalent forms of vulvar cancer include the following:

Melanoma

This is the second most prevalent kind of vulvar cancer that usually manifests as a pigmented wound on the vulva. In addition, amelanotic lesions may develop.

Basal cell carcinoma

About 2-7% of all vulval cancers are basal cell carcinomas

Bartholin gland carcinoma

This comprises up to 5% of all vulval cancers originating from the Bartholin gland (a small gland located just with the vaginal opening).

Sarcoma

This makes up 1 to 2% of vulval cancers. Soft tissue sarcomas involve leiomyosarcomas, liposarcomas, rhabdomyosarcomas, angiosarcomas, epithelioid sarcomas, neurofibrosarcomas, and uncategorized sarcomas.

Paget’s disease of the vulva

This represents less than 1% of all vulva cancers.

Symptoms of Vulval Cancer

Depending on the cancer’s kind and origin, vulvar cancer symptoms can differ. Squamous cell carcinoma, the most prevalent type of vulvar cancer, often presents the following noticeable symptoms:

An open sore that takes weeks or months to heal
Vaginal bleeding or discharge that isn’t part of the usual menstruation
Growths that resemble genital warts or cauliflower
Itchiness or burning feeling
A noticeable bump with a rough, raw surface
Pain around the vaginal area
Discoloration (skin that appears unusually red or pink or that is lighter or darker compared to the surrounding skin)

Other forms of vulvar cancer may present different symptoms than those of Bartholin gland carcinoma. You might see a bump close to the vaginal entrance. This bump might, however, be a benign cyst.

Causes and Risk Factors of Vulval Cancer

Many vulvar squamous cell carcinoma cases are linked to two main risk factors: human papillomavirus (HPV) and long-term inflammatory or autoimmune conditions.

HPV: Nearly 50% of all cases of vulvar squamous cell cancer are associated with HPV, the most prevalent sexually transmitted infection (STI) in the US, affecting about 43 million individuals annually. Although cancer may appear years after infection, most people with the virus get exposed during their early 20s. To lower the risk of HPV-related cancer, a safe and efficient vaccination is available. Although the HPV vaccine is recommended for individuals between the ages of 9 and 45, it is best to get it around age 9 if possible. Precancerous and malignant vulvar lesions can result from HPV infection.
Autoimmune or chronic inflammatory conditions: Some skin disorders like lichen sclerosus can cause the occurrence of precancerous and cancerous vulvar lesions not associated with HPV infection.

The following additional risk factors may also raise an individual’s risk of vulvar cancer:

Age: Females above 50 years are diagnosed with vulvar cancer in about 80% of cases.
HIV infection: The immune system is usually damaged by the human immunodeficiency virus (HIV) causing AIDS. In addition to increasing the chance of contracting HPV, this may also impair the body’s capacity to identify and kill cancer cells before spreading further.
Lichen sclerosus: This disorder results in thin, itchy, irritated skin surrounding the vulva. Only 4% of females with this disorder eventually become vulvar cancer.
Melanoma: The risk of vulvar cancer is higher if you have a family history of melanoma or numerous atypical moles.
Other genital cancers: You are at a high risk of having vulvar cancer if you have other genital malignancies, most frequently cervical cancer.
Smoking: You are more likely to develop vulval cancer if you smoke. The risk is also higher in those who smoke and have HPV.

While having one or more risk factors increases the likelihood of developing vulvar cancer, it does not mean you will get it. For this reason, pelvic exams and routine checkups are crucial. During these examinations, your doctor can check for early indicators of vulvar cancer.

Diagnosis

Routine wellness examinations with a gynecologist or primary care physician are the most effective method of diagnosing vulvar cancer. The doctor will check for typical vulvar cancer symptoms during these examinations, and if something seems off, testing may be ordered.

Colposcopy: Magnifying equipment is used during a colposcopy to further examine the skin cells surrounding the vulva and identify any indications of malignancy. A biopsy will be conducted if the area seems suspicious.
Biopsy: After numbing the vulva region, a small tissue sample is taken and sent to a laboratory for a pathologist to assess the presence of cancer. To confirm a vulvar cancer diagnosis, a biopsy is usually necessary.
Imaging: If your doctor suspects that your vulvar cancer may have extended to other parts of your body, they may recommend imaging tests, including positron emission tomography (PET), CT scan, or MRI.

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Stages of Vulval Cancer

To determine if cancer has spread and the extent, healthcare providers often use cancer staging. This can assist them in developing the most effective vulvar cancer treatment strategy.

Stage 1: Cancer is only on the vulva.
Stage 2: Cancer has extended to the anus, the urethra (a tiny tube connecting the bladder), or the lower section of the vagina.
Stage 3: The cancer has moved to the vagina, bladder, rectal mucosa, upper urethra, or adjacent lymph nodes.
Stage 4: Cancer has advanced and may involve the bone, distant metastases, or ulcerated lymph nodes. In addition, it may have reached further lymph nodes or other body parts.

Treatment

The common vulvar cancer treatment options include the following:

Surgery

Surgery is often performed to take out the vulva lesion. Depending on the location and size of the lesion, it may involve a simple, broad local excision or the removal of a bigger part of the vulva. To help ensure that all of the malignant cells are eliminated, the surgeon also removes a few healthy cells that surround the cancer.

Lymph node biopsy

This procedure is crucial to identify whether the cancer has progressed to the lymph nodes. Removal of lymph nodes is not necessary for the early stages of vulvar cancer. However, surgical evaluation of lymph nodes by sentinel lymph node biopsy or total lymphadenectomy is essential for any tumor larger than 2 centimeters or with a depth of more than 1 millimeter.

Radiation therapy

To destroy cancer cells or reduce a tumor before the surgical procedure, high-powered radiation beams are focused on the tumor.

Chemotherapy

Drugs destroy cancer cells that have metastasized to other body parts. The oncologist can suggest chemotherapy only or in conjunction with radiation and other treatments.

Immunotherapy

This treatment prompts the body’s natural defenses to identify and kill cancerous cells.

Prevention

Although vulvar cancer cannot be entirely prevented, these steps may help lower the risk:

Engaging in safer sexual activity, as using a condom during intercourse, can protect against HPV strains.
Going for cervical screening visits. Cervical screening can identify HPV and precancerous disorders like vulval intraepithelial neoplasia (VIN).
Quitting smoking

Additionally, the HPV vaccine may lower your risk of vulval cancer. As part of the regular childhood vaccination program, this is now available to all boys and girls aged 12 and 13.

Prognosis

The extent to which vulvar cancer has spread determines the prognosis. The 5-year survival rates are as follows:

86% survival rate when the cancer is confined to the vulva and hasn’t extended to lymph nodes or adjacent tissues.
53% survival rate when the cancerous cells have moved to the surrounding lymph nodes and not to other body parts.
19% survival rate when the cancer has metastasized to lymph nodes or distant organs.