Table of Contents

Vulval Cancer

Understanding Vulval Cancer

Vulval cancer pertains to malignancies that develop in the external female genitalia, known as the vulva. This includes:

– The labia minora and labia majora, which are the lips surrounding the vagina.

– The clitoris, a key sexual organ for female arousal and climax.

– The Bartholin’s glands, two small glands situated on each side of the vagina.

Often, vulval cancers originate from a condition known as vulva intraepithelial neoplasia (VIN), a precancerous change that can be treated to minimize cancer risk. The majority of vulvar cancers are categorized as squamous cell carcinomas, which develop in the squamous cells, or thin, flat skin cells, commonly found on the vaginal lips. Additionally, a smaller proportion of vulvar cancers are adenocarcinomas, cancers starting in mucus and fluid-producing cells, typically located near the vaginal opening.

Vulvar cancer tends to develop gradually over several years. A condition where abnormal cells grow on the vulvar skin’s surface for an extended period is known as vulvar intraepithelial neoplasia (VIN). Vulval cancer is among the less common gynecological cancers, with a little over 1,000 cases diagnosed annually in the UK. It predominantly affects older women, usually those above 65 years of age.

Varieties of Vulvar Cancer and Their Characteristics

Identifying the specific cell type where vulvar cancer originates is crucial for determining the most effective treatment approach. The primary types of vulvar cancer include:

– Vulvar Squamous Cell Carcinoma (SCC): This is the most prevalent form of vulvar cancer, constituting about 90% of cases in Australia. It originates in the vulva’s surface-lining thin, flat cells. Squamous cell carcinomas represent a significant majority of vulvar cancer cases.

– Vulvar Melanoma: Accounting for 2% to 4% of vulvar cancers, vulvar melanoma starts in melanocytes, the cells responsible for skin pigmentation. These cells are also present in the vulva’s lining. It’s important to note that this type of cancer is not linked to UV radiation overexposure.

– Sarcoma: A less common variant of vulvar cancer, sarcoma arises from muscle, fat, and other tissues beneath the skin.

– Adenocarcinoma: This rare form of vulvar cancer develops from glandular cells in the vulvar glands, which are responsible for mucus production.

– Basel Cell Carcinoma (BCC): An extremely rare type of vulvar cancer, BCC originates in the basal cells located in the skin’s lower layer.

Classification of Vulvar Cancer Stages

Vulvar cancer is categorized into four stages, numbered from 1 to 4. A lower stage indicates limited cancer spread, enhancing the likelihood of successful treatment. The staging criteria for vulvar cancer are as follows:

– Stage 1: The cancer remains solely within the vulva.

– Stage 2: The cancer has extended to adjacent areas like the lower part of the vagina, anus, or lower urethra, but has not affected the lymph nodes.

– Stage 3: The cancer has infiltrated nearby lymph nodes.

– Stage 4: The cancer has disseminated to more distant parts of the body, including farther lymph nodes.

Cancers in stages 1 and 2 are typically considered early-stage cancers, often associated with a higher success rate in treatment. In contrast, stages 3 and 4 represent advanced-stage cancers, where achieving a complete cure may not always be feasible.

Recognizing Symptoms of Vulvar Cancer

The following are signs and symptoms that may indicate vulvar cancer:

– Experiencing pain, sensitivity, or a burning sensation.

– Unusual bleeding or discharge not associated with menstrual cycles.

– Presence of a lump, growths resembling warts, or open sores.

– Persistent itchiness or pruritus lasting more than a few days.

– Enlargement or firmness of lymph nodes in the groin.

– Changes in vulvar moles or pigmented areas, including alterations in shape, color, or size.

– Painful urination.

– Areas of thickened skin with a distinct texture.

– Sores or bleeding.

– Swelling or irregular lumps.

– Dark or reddish patches on the skin.

If you notice any of these symptoms, it is crucial to consult your doctor promptly and request a referral to a specialist. Some conditions require specific diagnostic methods, such as vulvoscopy or a biopsy, for accurate identification. Seeking early medical advice is key for reassurance and timely treatment.

Understanding the Development of Vulval Cancer

Vulval cancer arises when cells in a specific area of the body start to grow and multiply excessively, leading to the formation of a mass known as a tumor. While the specific triggers of vulval cancer remain unclear, it’s understood that cancer generally starts when a cell undergoes mutations in its DNA. DNA serves as the blueprint directing cellular functions. These mutations cause the cell to proliferate rapidly and continue living beyond the normal lifespan of cells. As these abnormal cells accumulate, they can form a tumor, which has the potential to become cancerous, invade nearby tissues, and spread to distant parts of the body.

Risk Contributors to Vulvar Cancer

While the precise cause of vulvar cancer remains unknown, there are several factors that seem to elevate the risk of developing this disease:

– Vulval Intraepithelial Neoplasia (VIN): VIN is a precancerous state where changes in vulvar cells are observed. These changes are non-cancerous but have the potential to transform into cancer over time, often taking more than a decade. Sometimes these cells may revert to normal on their own. Due to the cancer risk, removing these cells is typically advised. VIN symptoms mirror those of vulvar cancer, such as persistent vulvar itchiness and the appearance of raised, discolored patches.

There are two categories of VIN:

  – Usual or undifferentiated VIN, commonly found in women under 50, often linked to HPV infection.

  – Differentiated VIN (dVIN), rarer and usually seen in women over 60, associated with vulvar skin conditions.

– Age Factor: The likelihood of developing vulvar cancer increases with age, most frequently diagnosed around the age of 65. However, it can sometimes affect women under 50.

– HPV Exposure: Human papillomavirus (HPV) is a significant risk factor. This virus, affecting skin and moist membranes, is sexually transmitted and elevates the risk for various cancers, including vulvar and cervical cancers. Most people will contract HPV at some point, but for some, the virus persists and leads to cellular changes increasing cancer risk. In around 40% of vulvar cancer cases, HPV is present, indicating its potential role in increasing the risk.

– Smoking: Smoking contributes to an increased risk of vulvar cancer, possibly by weakening the immune system’s effectiveness in clearing HPV and increasing vulnerability to the virus.

– Compromised Immune System: Individuals with weakened immune systems, such as organ transplant recipients or those with conditions like HIV, face a higher risk of vulvar cancer.

– History of Precancerous Vulvar Conditions: Having precancerous conditions like vulvar intraepithelial neoplasia heightens the risk, though most cases do not progress to cancer. Treatment to remove abnormal cells and regular monitoring are often recommended.

– Skin Conditions Affecting the Vulva: Certain skin conditions, such as lichen sclerosus and lichen planus, that cause itching, soreness, and discoloration of the vulva, are linked to a slightly increased risk of vulvar cancer. It’s estimated that less than 5% of women with these conditions will develop vulvar cancer, and it’s unclear if treatment for these conditions reduces this risk.

Diagnosing Vulvar Cancer

When you visit a gynecologist with symptoms suggestive of vulvar cancer, they may perform a detailed examination of your vulva and recommend a biopsy to confirm the presence of cancer.

Physical Examination

Your doctor will conduct a thorough examination of your groin and pelvic region, including the genitals. A nurse might be present during this process, and you’re welcome to have a family member accompany you. An internal examination may also be conducted to assess your vagina and cervix, involving the insertion of a speculum for a clearer view.

Colposcopy

To examine your vulva and vagina more closely, your doctor might use a colposcope, a magnifying device. This instrument is used externally, and a special liquid is applied to your vulva and vagina to highlight abnormal cells. This application might cause a temporary stinging sensation and result in a brown discharge afterward.

Biopsy

A biopsy involves taking a small tissue sample for microscopic examination to identify cancerous cells. Typically, a local anesthetic is administered to numb the area, making the procedure painless and allowing you to return home the same day. In some cases, a general anesthetic might be used, possibly requiring an overnight hospital stay. The biopsy site may be stitched, leading to minor bleeding and soreness for a few days. Your doctor will schedule a follow-up appointment within 7 to 10 days to discuss the biopsy results.

Additional Tests

If biopsy results confirm cancer, further tests may be necessary to determine its extent, including:

– Cystoscopy: A procedure to inspect the bladder’s interior using a thin tube.

– Proctoscopy: An examination of the rectum’s interior.

– Lymph node biopsies: To check if cancer has spread through the lymphatic system.

– CT or MRI scans: To detect any cancer presence in lymph nodes or other organs.

– X-ray: To ensure the cancer hasn’t spread to the lungs.

 

These tests help in “staging” the cancer, using a numerical system to describe the extent of its spread.

Treatment Approaches for Vulval Cancer

Vulval cancer treatment is tailored based on several factors, including the cancer’s stage. Primary treatment methods encompass surgery, radiotherapy, and chemotherapy.

Surgical Approach Based on Cancer Stage

– Stage 1: For cancer confined to the vulva’s skin, treatment involves wide local excision of the tumor with a significant clear margin, possibly including lymph node removal in the groin.

– Stage 2 and higher: Treatment may require more extensive surgery, including radical excision of the vulva and groin lymph gland removal, often accompanied by reconstructive perineal plastic surgery. This is typically followed by pelvic radiotherapy.

Surgical Techniques

– Excision: Removal of the cancer along with a margin of healthy tissue.

– Radical Vulvectomy: Complete removal of the vulva.

– Laser Surgery: Utilizing a laser beam to precisely cut tissue or excise surface lesions.

– Local Excision: Eliminating the cancer and surrounding normal tissue, with potential removal of nearby lymph nodes.

– Vulvectomy: Removing part or all of the vulva, sometimes including lymph nodes, with possible skin grafts for reconstruction.

– Pelvic Exenteration: Involves removing the lower colon, rectum, bladder, cervix, vagina, ovaries, and lymph nodes, with the creation of openings for bodily waste management.

Radiotherapy

This treatment targets the vulval skin and potential spread areas like groin lymph glands. It employs high-energy beams like X-rays to eradicate cancer cells. External beam radiation is the common form, often used to shrink larger cancers pre-surgery or to treat lymph nodes post-surgery. Typically, sessions last 10-15 minutes.

Radiotherapy Side Effects

These may include diarrhea, blood in stools or urine, frequent urination, and discomfort during urination, persisting for years post-treatment.

Chemotherapy

Used when cancer spreads beyond the pelvis, chemotherapy involves intravenous administration of drugs that target cancer cells. Treatment often spans half a day every 3-4 weeks over six cycles, with side effects like fatigue, nausea, weight and appetite loss, and hair loss.

Immunotherapy

This treatment enhances the immune system’s ability to detect and combat cancer cells. Imiquimod cream is a commonly used immunotherapy for vulvar cancer.

Palliative Care

In advanced vulvar cancer cases, palliative care focuses on symptom relief and quality of life improvement. This may involve radiotherapy, chemotherapy, or other drug treatments to slow cancer spread and manage pain and other symptoms.

Treatment Options for Vulval Cancer

The primary treatment for vulval cancer usually involves surgically removing the cancer along with lymph nodes in the groin area. This may be followed by treatments like radiotherapy or chemotherapy, or both. These treatments might lead to changes in the appearance of the external genitalia. However, advancements like sentinel lymph node dissection are being used to minimize side effects such as lymphoedema, which is swelling in the leg.

Prognosis and Survival Rates

The likelihood of successfully treating vulval cancer is highest when diagnosed at an early stage, such as stage 1, where it’s often completely curable. Statistically, about 70% of women diagnosed with vulval cancer survive for five years or more.

Prevention of Vulval Cancer

– HPV and Vaccination: Certain vulval cancers are linked to the Human Papilloma Virus (HPV). Getting vaccinated against HPV can potentially prevent these types of vulval cancer. Regular skin checks of the external genitalia can help in identifying precancerous conditions early, reducing the risk of developing vulval cancer.

– Cervical Screening: Attending regular cervical screening appointments is important as they can detect HPV and precancerous conditions like vulval intraepithelial neoplasia (VIN).

– Lifestyle Changes: Quitting smoking can also lower the risk of developing vulval cancer.

Outlook for Vulval Cancer

The prognosis for vulval cancer varies depending on factors like the extent of cancer spread, your age, and overall health. Early detection generally improves the chances of successful treatment, particularly in younger individuals. Approximately 70% of women with a diagnosis of vulval cancer survive for at least five years following the diagnosis. However, there is a possibility of the cancer recurring post-treatment. Therefore, regular follow-up appointments are essential for monitoring and early detection of any recurrence.

Vulval Cancer Survival Rates

Stage 1 Survival Rate

Approximately 80% of women diagnosed with stage 1 vulval cancer are likely to survive for five years or more following their diagnosis.

 

Stage 2 Survival Rate

For women with stage 2 vulval cancer, the five-year survival rate stands at about 50%.

 

Stage 3 Survival Rate

In cases of stage 3 vulval cancer, the five-year survival probability is around 40%.

 

Stage 4 Survival Statistics

– About 85% of women with stage 4 vulval cancer in England survive for at least one year post-diagnosis.

– The five-year survival rate, more generally for women with vulval cancer in England, is over 65%.

 

These statistics offer a perspective on the prognosis for vulval cancer at various stages, highlighting the importance of early detection and treatment.