Types of Vulvar Cancer: A Layman's Guide

By Dr. Hemant B. Tongaonkar in Nanavati Max Institute of Cancer Care

Jul 02 , 2024 | 11 min read

Vulvar cancer is cancer that develops in the vulva — the external female genital area, including the labia, clitoris, and the openings of the vagina and urethra. It is rare, and when found early it is often highly treatable. Understanding the different types helps with recognising warning signs and seeking timely care.

In India, compared to other forms of cancer affecting women like breast or cervical cancers, vulvar cancer may not be as common. However, its incidence cannot be overlooked. Globally, it is considered a rare cancer, with approximately 6,500 new cases diagnosed annually in the United States.Certain risk factors associated with vulvar cancer include being over the age of 50, having a history of human papillomavirus (HPV) infection or genital warts, and experiencing chronic inflammatory conditions of the vulva.

What Is Vulvar Cancer?

Vulvar cancer can affect any part of the vulva. The most common type by far is squamous cell carcinoma, which makes up over 90% of cases and begins in the flat skin cells of the vulva. Vulvar cancer is uncommon, accounting for around 4% of cancers of the female reproductive system, and most often affects women after menopause, with a median age at diagnosis of about 68. (Most published figures are from the US and global registries; in India, it is less commonly reported but still important to recognise.)

Signs and Symptoms of Vulvar Cancer

Symptoms can be subtle and are easy to dismiss. See a doctor if you notice any of the following lasting more than a couple of weeks:

Persistent itching or burning that does not go away
A lump, swelling, wart-like growth, or ulcer on the vulva
A change in the colour or thickness of the vulvar skin (a patch that is paler, darker, red, or white)
Persistent pain, tenderness, or soreness
Bleeding or unusual discharge not related to your period
Pain during sex or when passing urine
A mole on the vulva that changes shape, size, or colour

Understanding the Primary Types of Vulvar Cancer

Understanding the various Types of Vulvar Cancer is crucial in early detection, effective management, and treatment. While there are rarer forms, here we explore the major classifications:

1. Squamous Cell Carcinoma (SCC)

Squamous cell carcinoma (SCC) is the most common type of vulvar cancer, accounting for approximately 9 out of 10 cases . It originates from the squamous cells that make up the outer layer of the skin on the vulva.

Signs and Symptoms: Symptoms of SCC often include long-term itching around the vulva, persistent pain, and sometimes skin changes such as colour or thickness.
Pathogenesis Note: SCC typically develops through one of two pathways: an HPV-dependent pathway, common in younger women, or an HPV-independent pathway, linked to chronic inflammatory skin conditions like Lichen Sclerosus in older women .
Causes and Risk Factors: Risk factors include age (it's most common in post-menopausal women over 50 ), having a weak immune system, or being infected with certain strains of HPV.
Diagnosis and Treatment: Treatment for SCC depends on the stage of cancer and may involve surgery to remove cancerous tissue and possibly nearby lymph nodes. Radiotherapy or chemotherapy might be recommended if cancer has spread beyond the vulva or comes back after treatment.

Read More about Most Common Cancer In Women & Its Symptoms

2. Vulvar Melanoma

Melanoma is the second most common, yet aggressive, type of vulvar cancer that originates from pigment-producing cells in the skin.

Signs and Symptoms: It can present symptoms such as changes in existing moles on your vulva or development of new pigmented areas. These changes may include asymmetry, irregular borders, multiple colors, or an increasing diameter (the 'ABCD' rule) .
Causes and Risk Factors: The exact causes are not well known, but having many moles (atypical nevi) or a family history of melanoma could increase the risk of developing Vulvar Melanoma . Unlike skin melanoma, UV radiation exposure is not considered a primary risk factor for vulvar melanoma .
Treatment Approach: Melanoma requires aggressive treatment due to its nature to quickly spread within the body. Treatment mainly involves surgery to remove melanoma and some normal tissue surrounding it. For advanced stages, doctors might recommend immunotherapy drugs or targeted therapy drugs that work to stop the growth of melanoma cells.

3. Adenocarcinoma and Related Types

Adenocarcinoma, a rare type of vulvar cancer, begins in the glandular cells in the lining of the vulva, often arising from the Bartholin's glands .

Bartholin Gland Carcinoma: These cancers start in the mucus-secreting glands near the vaginal opening. They are often detected late due to their deep location .
Extramammary Paget's Disease (EMPD): This rare, slow-growing cancer is a type of intraepithelial adenocarcinoma found in the top layer of vulval skin. Although often non-invasive, it requires wide surgical excision due to its tendency to recur and can, rarely, be associated with an underlying invasive adenocarcinoma .

4. Other Rare Types of Vulvar Cancer

To provide a complete overview of Types of Vulvar Cancer, rarer histological forms include:

Basal Cell Carcinoma (BCC): Comprising 2% to 7% of all vulvar cancers, BCC is slow-growing and has a highly favorable prognosis. It is typically cured with local surgical excision .
Sarcoma: These are malignant tumors that arise from the soft connective tissues, such as muscle, fat, or cartilage beneath the skin. They are extremely rare, accounting for only 1% to 2% of cases, and often occur in younger women .
Verrucous Carcinoma: A rare, wart-like subtype of Squamous Cell Carcinoma that is generally locally aggressive but has a very low potential for spreading (metastasis) .

Know More about Ovarian Cancer

How Vulvar Cancer Is Diagnosed

Diagnosis starts with a physical examination and a discussion of your history. If something unusual is found, tests may include:

Biopsy: removing a small piece of tissue for analysis — the only way to confirm cancer.
Colposcopy: examining the vulva closely with a magnifying device.
Imaging (CT, MRI, or PET): to check whether the cancer has spread.
Sentinel lymph node biopsy (SLNB): identifying the first lymph node the cancer would drain to, so surgeons can remove fewer nodes and reduce the risk of lymphoedema.

Vulvar Cancer Staging (FIGO 2021)

Staging describes how far the cancer has spread and guides treatment. Doctors use the International Federation of Gynecology and Obstetrics (FIGO) system, revised in 2021:

Stage	What It Means
Stage I	Cancer is confined to the vulva (and/or perineum), with no spread to lymph nodes.
Stage II	Cancer has spread to nearby lower structures (lower urethra, lower vagina, or anus) but lymph nodes are clear.
Stage III	Cancer involves the groin lymph nodes or upper nearby structures. Sub-divided as IIIA (node deposits up to 5 mm, or spread to upper urethra/vagina, bladder or rectal lining), IIIB (node deposits larger than 5 mm), and IIIC (cancer extends beyond the lymph node capsule).
Stage IV	Stage IVA: cancer is fixed to the pelvic bone, or there are fixed or ulcerated groin nodes. Stage IVB: cancer has spread to distant organs or distant lymph nodes.

Treatment Options for Vulvar Cancer

Treatment depends on the type, stage, and your overall health. Surgery is the mainstay for most vulvar cancers.

Local (wide) excision: removing the tumour with a margin of healthy tissue.
Vulvectomy: removing part (partial) or all (total) of the vulva.
Lymph node surgery: sentinel node biopsy or, if needed, removal of groin nodes.
Laser surgery: for pre-cancer (VIN) or very superficial cancers.
Pelvic exenteration: an extensive operation reserved for advanced or recurrent central tumours.
Radiation therapy: high-energy beams, sometimes combined with chemotherapy (chemoradiation).
Chemotherapy: often with radiation for locally advanced disease; common drugs include cisplatin and fluorouracil.

Targeted therapy and immunotherapy: newer options for selected advanced or recurrent cancers.

Read More about the Different types of cancer treatment

Vulvar Cancer Pre-cancerous Conditions and Prevention Strategies

The precise cause of vulvar cancer is not entirely known, but there are several key risk factors to be aware of:

Age: This type of cancer is more common in women over the age of 50.
HPV Infection: This sexually transmitted infection is a significant risk factor, particularly for the squamous cell type .
Smoking: Tobacco use increases the probability of developing various types of cancers, including vulvar cancer .
Weak Immune System: Having a weak immune system or conditions such as HIV/AIDS are potential risk factors.

Pre-cancerous Conditions

The following conditions are not cancer but signal changes that may lead to the development of vulvar cancer over time, requiring close monitoring:

Vulvar Intraepithelial Neoplasia (VIN): This is a precancerous condition, usually caused by HPV, where abnormal cells are found in the surface layer of the vulvar skin. Treating VIN significantly reduces the risk of invasive cancer .
Lichen Sclerosus: Chronic inflammatory skin conditions affecting the vulva, such as Lichen Sclerosus, should warrant a visit to the doctor as they are potential risk factors inducing the risk of vulvar cancer .

The good news is that many aspects leading to this disease are preventable. Here are some steps individuals can take to lower their chances:

HPV Vaccine: Research suggests that the vaccine, especially when administered to younger women before they become sexually active, can significantly reduce the risk of HPV-related vulvar cancers .
Regular Screening & Self-Examination: While no routine national screening program exists for vulvar cancer, early detection through routine pelvic exams and HPV tests can catch any abnormalities promptly. Regular self-examination of the vulva is highly recommended to notice any changes early .
Quitting Smoking: The lesser contact with tobacco, the better it is for overall health.
Safe Sexual Practices: Using protection during intercourse can help prevent HPV, thereby reducing one's risk.

Coping and Support: The Emotional Journey

Navigating the stormy seas of a cancer diagnosis isn't merely a physical journey; it involves dealing with emotional and psychological challenges as well.

When facing this type of cancer, you may experience feelings of fear, anxiety, sadness, or even anger. These are normal reactions and are an integral part of coming to terms with the diagnosis. The uncertainty that accompanies any cancer diagnosis can feel overwhelming. This is why emotional and psychological support is paramount at every stage.

Emotional support can come from various sources. Your close family and friends can provide a comforting shoulder to lean on, while support networks offer a community of individuals who understand exactly what you're going through. This could include online communities, local support groups, or patient advocacy organisations.

In addition to these sources of support, professional counselling can be incredibly beneficial. Counsellors can help you navigate your feelings and teach coping strategies that can assist in easing the burden of anxiety and stress related to your condition.

However, remember that it's okay not to have all the answers right away. It's normal to need time to absorb the news and decide on your next steps. When ready, talk about your feelings with loved ones and consider seeking professional guidance.

Furthermore, coping strategies should not be overlooked. The following methods may be helpful:

Regularly practising mindfulness exercises like meditation or yoga.
Engaging in hobbies or activities that you enjoy.
Maintaining a balanced diet and regular exercise routine.
Prioritising rest and quality sleep.
Staying informed about your condition – including the different Types of Vulvar Cancer and potential treatments – but avoid inundating yourself with excessive information.

Most importantly, don't hesitate to seek professional help if you find it difficult to cope with your emotions or if they interfere with your daily life. A mental health professional can equip you with tools and techniques that can make the journey easier.

When to See a Doctor

Make an appointment with a gynaecologist if you notice any of these lasting more than two to three weeks:

Persistent vulvar itching, burning, or pain
A new lump, ulcer, wart-like growth, or sore that does not heal
A change in skin colour or thickness (a white, red, or dark patch)
Unusual bleeding or discharge unrelated to your period
A mole on the vulva that changes in size, shape, or colour

Conclusion

As we conclude this journey into understanding vulvar cancer, let's reflect on the key points discussed. We explored the various Types of Vulvar Cancer, examined the crucial staging system, and emphasised how early detection can significantly improve outcomes.

The role of awareness in combating this disease cannot be overstated. The more informed we are about such health issues, the better equipped we become to identify early warning signs and seek timely medical intervention. Remember, knowledge is power and sharing it multiplies its impact.

Help others understand the importance of acknowledging any unusual symptoms and visiting their doctor at the earliest for a check-up. For further reading on common gynecologic cancers, see: Read more about - Most Common Cancer In Women & Its Symptoms.

Disclaimer

This article provides general health information and is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of a qualified healthcare provider with any questions you may have regarding a medical condition.

Frequently Asked Questions

1. What are The Common Symptoms of Vulvar Cancer?

Some common signs include a persistent itching or burning sensation in the vulvar region, thickening or colour change of the skin, pain during intercourse, bleeding or discharge not related to menstruation, and a lump or ulcer on the vulva that persists for more than a month.

2. How is Vulvar Cancer Diagnosed?

The first step towards diagnosing vulvar cancer involves a detailed physical examination by your doctor, who will also ask about your medical history. If something unusual is found, you may be referred to a gynaecologist for further evaluation.

3. What are The Available Treatment Options?

Treatment options for vulvar cancer depend on factors such as the stage of the cancer and your overall health condition. Commonly used treatments include surgery (ranging from local excision to vulvectomy), radiation therapy that uses high-energy beams to kill cancer cells.

4. How Common is Vulvar Cancer, and Who is at Risk?

Vulvar cancer is relatively rare, accounting for about 3% to 5% of all female genital tract cancers . In the US, approximately 6,500 new cases are diagnosed annually . Women over 50 are at higher risk, though it can occur at any age.

5. What Support is Available for Individuals Diagnosed With Vulvar Cancer?

Being diagnosed with any form of cancer can be overwhelming; however, various forms of support are available. This ranges from medical and emotional support provided by your healthcare team to financial aid and patient support groups where you can connect with others going through similar experiences.

References

1. American Cancer Society. (2023). What Is Vulvar Cancer?
https://www.cancer.org/cancer/types/vulvar-cancer/about/what-is-vulvar-cancer.html

2. National Cancer Institute. (n.d.). Squamous Cell Carcinoma of the Vulva.
https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq

3. National Comprehensive Cancer Network (NCCN). (2024). NCCN Guidelines for Patients®: Vulvar Cancer.

https://www.nccn.org/patients/guidelines/content/PDF/vulvar-cancer-patient.pdf