Overview
Vulvar cancer is a rare form of cancer that develops in the external female genital area, known as the vulva. It accounts for approximately 4% of all gynecological cancers and around 0.3% of all cancers diagnosed in women. Although it primarily affects older women — with most cases diagnosed after the age of 65 — it can occur at any age, and cases in younger women have been increasing, largely due to infections with the human papillomavirus (HPV).
When detected early, vulvar cancer is highly treatable, which makes awareness of its signs and symptoms essential. Unfortunately, many women delay seeking medical help because of embarrassment, lack of awareness, or the assumption that their symptoms are caused by a minor skin condition. This article provides comprehensive, reliable information to help readers understand vulvar cancer, recognize its warning signs, and know when to seek medical attention.
What Is Vulvar Cancer?
The vulva is the collective term for the external organs of the female genital area. It includes:
- The labia majora and labia minora (the outer and inner “lips” or skin folds)
- The clitoris
- The opening of the vagina (introitus)
- The openings of the urethra (urinary channel)
- The mons pubis and the perineum (the area between the vagina and the anus)
- Bartholin’s glands, located on each side of the vaginal opening
Vulvar cancer occurs when abnormal cells in any of these structures begin to grow uncontrollably, forming a tumor. If left untreated, these cells can invade deeper tissues and spread (metastasize) to nearby lymph nodes — particularly the lymph nodes in the groin — and eventually to distant organs.
Most vulvar cancers develop slowly, often over several years. Before invasive cancer forms, abnormal changes may occur in the surface cells of the vulvar skin. This precancerous condition is called vulvar intraepithelial neoplasia (VIN). Not all cases of VIN progress to cancer, but monitoring and treating VIN can significantly reduce the risk of invasive disease.
Types of Vulvar Cancer
Vulvar cancer is not a single disease; several distinct types exist, classified according to the kind of cell in which the cancer begins.
| Type of vulvar cancer | Approximate proportion of cases | Cell of origin | Key features |
|---|---|---|---|
| Squamous cell carcinoma | ~85–90% | Thin, flat surface (epithelial) cells | Most common type; develops in the skin covering the labia and perineal area |
| Vulvar melanoma | ~2–5% | Melanocytes (pigment-producing cells) | Often found on the clitoris or labia minora; behaves aggressively |
| Adenocarcinoma | ~3–4% | Glandular cells (e.g., Bartholin’s glands) | Usually presents as a lump on the side of the vaginal opening |
| Basal cell carcinoma | ~1–2% | Basal layer of the skin | Slow-growing; rarely spreads to lymph nodes |
| Soft tissue sarcoma | <1% | Connective tissue cells | Rare; often appears as a deep, enlarging mass |
| Paget’s disease of the vulva | <1% | Gland-related skin cells | Presents as an eczema-like, weepy, itchy rash |
Squamous Cell Carcinoma — Two Distinct Pathways
Medical research recognizes two main pathways by which squamous cell carcinoma of the vulva develops:
- HPV-associated pathway: More common in younger women. Linked to persistent infection with high-risk HPV strains (especially HPV-16 and HPV-33) and arises from usual-type vulvar intraepithelial neoplasia (uVIN).
- HPV-independent pathway: More common in older women. Associated with chronic inflammatory skin conditions, particularly lichen sclerosus, and arises through a less well-understood precancerous change called differentiated VIN (dVIN), which can progress to cancer relatively quickly.
Causes and Risk Factors
The exact cause of any individual case of vulvar cancer is rarely known. However, research has identified several factors that significantly increase a woman’s risk:
Established Risk Factors
- Age: Risk rises sharply with age; most cases occur in women over 65, though HPV-related cancers can develop much earlier.
- Human papillomavirus (HPV) infection: Persistent infection with high-risk HPV types is responsible for a substantial proportion of vulvar cancers, particularly in younger women.
- Vulvar intraepithelial neoplasia (VIN): A precancerous change in the vulvar skin that can progress to invasive cancer if untreated.
- Lichen sclerosus: A chronic inflammatory skin condition that causes thin, white, itchy patches on the vulva. Women with this condition have an estimated 4–6% lifetime risk of developing vulvar cancer and require regular monitoring.
- Smoking: Tobacco use increases risk, especially in combination with HPV infection; it also impairs the immune system’s ability to clear the virus.
- Weakened immune system: Women with HIV infection, organ transplant recipients, or those on long-term immunosuppressive therapy face a higher risk.
- History of cervical or vaginal cancer or precancer: These share common risk factors with vulvar cancer, particularly HPV.
- Pelvic radiation therapy: Previous radiation to the pelvic area modestly increases the risk.
Important note: Vulvar cancer is not contagious and cannot be passed to a sexual partner**. However, HPV itself is sexually transmissible and is best prevented through vaccination and safer sexual practices.
How Does Vulvar Cancer Look?
One of the most important — yet least discussed — aspects of vulvar cancer is its visible appearance. Because many women rarely examine their vulva, early changes often go unnoticed. Knowing what vulvar cancer can look like may lead to earlier diagnosis and a much better outcome.
Vulvar cancer does not have one single appearance. Depending on the type and stage, it may look like any of the following:
Typical Visual Signs
- A lump, bump, or thickened area of skin — often firm to the touch, most commonly found on the labia majora, but possibly on the labia minora, clitoris, or perineum. It may be painless or tender.
- An ulcer or open sore that does not heal — a wound that persists for more than a few weeks, may bleed easily, ooze, or develop a crust, and fails to respond to ordinary wound care.
- A wart-like or cauliflower-shaped growth — resembling a genital wart but persisting, growing, or changing over time. This appearance is more typical of HPV-related cancers.
- Flat, discolored patches of skin:
- Red or pink patches — areas that look inflamed or raw
- White patches (leukoplakia) — thickened, pale areas of skin
- Darker patches — brownish or black areas of pigmentation, which may signal melanoma
- A mole-like spot that changes — for vulvar melanoma, a pigmented spot that grows, darkens, has irregular borders or uneven color, bleeds, or itches.
- Thickened, rough, or scaly skin — skin that looks and feels different from the surrounding tissue.
Key Characteristics to Note
| Feature | What to look for |
|---|---|
| Persistence | Any change lasting longer than 2–4 weeks despite normal hygiene or simple treatments |
| Growth | A lump, spot, or patch that is slowly enlarging |
| Bleeding | Spontaneous or contact bleeding from a vulvar lesion |
| Color change | Areas becoming red, white, brown, or black compared with normal skin |
| Texture change | Skin becoming rough, warty, thickened, or broken down |
A Word About Self-Examination
Medical professionals encourage women to perform occasional vulvar self-examination using a mirror and good lighting. Familiarity with your own normal appearance makes it far easier to notice early changes. The examination is quick, painless, and can be life-saving.
See a doctor promptly** if you notice any lump, sore, patch, or pigment change on the vulva that does not disappear within a few weeks — even if it is not painful. A painless lesion can still be serious.
Symptoms of Vulvar Cancer
The symptoms of vulvar cancer vary between individuals. Some women notice striking visible changes, while others experience only subtle sensations such as itching. In the earliest stages, there may be no symptoms at all.
Most Common Symptoms
- Persistent itching (pruritus) of the vulva — the most frequently reported symptom. While most itching has innocent causes (such as thrush, eczema, or dermatitis), itching that does not respond to standard treatments should always be investigated.
- A lump, sore, ulcer, or discolored patch on the vulva, as described in the section above.
- Pain, burning, soreness, or tenderness in the vulvar area that does not go away.
- Bleeding or blood-stained discharge not related to menstruation, including bleeding after sexual intercourse or gentle washing.
- Pain or discomfort during urination, or a burning sensation when urine touches the affected skin.
- A raw, “raw-feeling” area or a sensation that the skin has broken down.
Less Common and Advanced Symptoms
- A lump or swelling in the groin — this may indicate that the cancer has spread to the lymph nodes.
- Unusual or foul-smelling vaginal discharge.
- Enlarged, firm, or ulcerating areas on the vulva in more advanced disease.
- Swelling of one or both legs (lymphedema) — usually a sign of lymph node involvement or a complication after lymph node treatment.
- Pain in the pelvis or groin in advanced stages.
Symptoms by Cancer Type
| Type | Typical presenting symptom |
|---|---|
| Squamous cell carcinoma | Itching, lump, ulcer, or non-healing sore |
| Vulvar melanoma | A changing, pigmented (dark) spot; bleeding; itching |
| Bartholin gland adenocarcinoma | A firm, deep lump near the vaginal opening |
| Paget’s disease of the vulva | Weeping, eczema-like, intensely itchy rash |
Remember: The vast majority of vulvar symptoms are caused by benign** conditions such as infections, cysts, or skin disorders. However, only a medical examination — and sometimes a biopsy — can distinguish a harmless condition from cancer. Never let embarrassment delay a consultation.
How Is Vulvar Cancer Diagnosed?
1. Medical History and Physical Examination
The doctor will ask about symptoms, their duration, and relevant risk factors. A careful inspection and palpation of the vulva follows, including the groin lymph nodes, the vagina, and the cervix.
2. Biopsy — The Definitive Test
The only way to confirm vulvar cancer is a biopsy. Under local anesthetic, the doctor removes a small sample of suspicious tissue (punch biopsy) or the entire small lesion (excisional biopsy). A pathologist then examines the cells under a microscope. A biopsy is quick, is performed in the clinic, and causes only minor, brief discomfort.
3. Vulvoscopy and Colposcopy
The vulva may be examined with a magnifying instrument (colposcope), sometimes after applying a dilute acetic acid solution that highlights abnormal areas and helps select the best biopsy site.
4. Imaging and Further Staging Tests
If cancer is confirmed, additional tests determine whether it has spread:
- MRI scan — to assess the local extent of the tumor
- CT scan (chest, abdomen, pelvis) — to look for spread to lymph nodes or distant organs
- PET-CT — in selected advanced cases
- Cystoscopy or proctoscopy — if there is suspicion of invasion into the urethra, bladder, or anus
- Sentinel lymph node biopsy — a specialized surgical technique in which a small amount of radioactive tracer and/or blue dye identifies the first draining lymph node(s) in the groin, which are removed and examined. This helps avoid extensive lymph node removal in patients with early disease.
5. Cervical Screening and HPV Testing
Because vulvar, vaginal, and cervical cancers share risk factors, the doctor may also perform a cervical examination and, where appropriate, cervical screening and HPV testing.
Staging of Vulvar Cancer
Staging describes how large the cancer is and how far it has spread. The internationally used FIGO classification (International Federation of Gynecology and Obstetrics) divides vulvar cancer into four stages:
| FIGO stage | Description |
|---|---|
| Stage I | Cancer is confined to the vulva and/or perineum; lymph nodes are not involved. Stage IA tumors are 2 cm or smaller with minimal (≤1 mm) depth of invasion; Stage IB tumors are larger or invade more deeply. |
| Stage II | Cancer of any size that has grown into nearby structures such as the lower third of the urethra, the lower third of the vagina, or the anus — still without lymph node spread. |
| Stage III | Cancer has spread to the lymph nodes in the groin (one or both sides), with variable size and whether the nodes are mobile or partially fixed. |
| Stage IV | IVA: Cancer is fixed to pelvic bone or has caused non-resectable/ulcerated, fixed lymph nodes, or has invaded the upper urethra, bladder, rectum, or pelvic bone. IVB: Cancer has spread to distant organs, such as the lungs or pelvic lymph nodes. |
Staging has a direct influence on treatment choice and prognosis: the earlier the stage at diagnosis, the better the expected outcome.
Treatment of Vulvar Cancer
Treatment is tailored to each patient based on the stage of the disease, the size and location of the tumor, the patient’s age and general health, and her personal preferences. Care is usually coordinated by a multidisciplinary team including a gynecological oncologist, radiation oncologist, medical oncologist, pathologist, and specialist nurses.
Surgery
Surgery is the main treatment for most vulvar cancers.
- Wide local excision / radical local excision: Removal of the tumor together with a margin of healthy tissue (usually about 1 cm) around it. Used for small, localized tumors.
- Partial or total vulvectomy: Removal of part or all of the vulva for larger tumors. Modern surgical techniques aim to preserve as much healthy tissue and as much function — particularly of the clitoris and urethra — as possible.
- Lymph node assessment: Because vulvar cancer commonly spreads first to the groin lymph nodes, these are evaluated by either:
- Sentinel lymph node biopsy (for selected early-stage patients), or
- Inguinal lymphadenectomy (removal of groin lymph nodes) when nodes are involved or the tumor is above a certain size/depth.
- Reconstructive surgery: When large areas are removed, plastic surgery with skin flaps or grafts may restore appearance and function.
Radiation Therapy
Radiation uses high-energy beams to destroy cancer cells. It may be given:
- After surgery (adjuvant therapy) if the risk of recurrence is high — for example, when lymph nodes are involved or resection margins are narrow.
- Before surgery to shrink a large tumor.
- As primary treatment (often combined with chemotherapy — called chemoradiation) for advanced tumors where primary surgery would be too mutilating, or for patients unable to undergo surgery.
Chemotherapy
Chemotherapy uses medication to kill cancer cells. In vulvar cancer it is most commonly given together with radiotherapy (chemoradiation) to sensitize tumor cells to radiation, or for recurrent and metastatic disease as palliative systemic therapy.
Other and Emerging Treatments
- Topical therapy for precancer (VIN): Creams such as imiquimod (an immune-response modifier) or topical fluorouracil may be used for non-invasive disease.
- Immunotherapy: Drugs such as pembrolizumab (a checkpoint inhibitor) may be considered for certain advanced or recurrent cancers with specific biological markers.
- Clinical trials: Offer access to new treatment approaches; patients may wish to ask their care team about eligibility.
Treatment of Precancerous Changes (VIN and Lichen Sclerosus)
- VIN may be treated with surgical excision, laser ablation (carefully selected cases), or topical imiquimod, with long-term follow-up.
- Lichen sclerosus is treated with high-potency topical corticosteroids, which control symptoms and likely reduce the risk of progression to cancer.
Treatment side effects can include wound healing problems, lymphocele (fluid collection in the groin), lymphedema of the legs**, skin reactions from radiation, urinary discomfort, changes in sexual function and body image, and early menopause in premenopausal women if pelvic radiation is given. Specialist nurses, physiotherapists, and psychosexual counselors form an important part of recovery.
Outlook (Prognosis)
The outlook for vulvar cancer depends mainly on:
- The stage at diagnosis — the single most important factor
- Whether the groin lymph nodes contain cancer
- The size and depth of the tumor
- The patient’s general health and response to treatment
Overall, the 5-year relative survival rate for vulvar cancer is around 70%, but this figure varies widely by stage:
- Localized disease (confined to the vulva): approximately 85–90%
- Spread to regional lymph nodes: approximately 50–60%
- Distant spread: considerably lower, though treatment can still control the disease and maintain quality of life
These figures are statistical averages and cannot predict the outcome for any individual patient. Regular follow-up examinations — especially in the first few years after treatment — allow any recurrence to be detected early.
Prevention
Although not every case of vulvar cancer can be prevented, the following measures substantially reduce risk:
- HPV vaccination: Vaccines such as the 9-valent HPV vaccine protect against the HPV types most often linked to vulvar cancer. Vaccination is most effective when given before exposure to the virus (typically in adolescence).
- Do not smoke, or stop smoking: Smoking increases the risk of vulvar cancer and weakens the immune defense against HPV.
- Manage lichen sclerosus properly: Consistent treatment with prescribed steroid cream and regular medical check-ups (typically every 6–12 months) can reduce the risk of malignant change.
- Practice safer sex: Condom use reduces the likelihood of HPV transmission.
- Attend regular gynecological examinations: Routine check-ups allow early detection of precancerous changes.
- Know your vulva: Regular self-examination with a mirror helps detect changes early.
- Seek medical advice promptly for persistent itching, lumps, sores, or skin changes — do not wait for them to “go away on their own.”
Living With and Beyond Vulvar Cancer
A cancer diagnosis affects far more than the body alone. Common challenges include:
- Body image and intimacy: Surgery can change the appearance of the vulva and affect sexual sensation and confidence. Open communication with a partner and support from specialist nurses or psychosexual therapists can help.
- Physical after-effects: Lymphedema, scar tightness, and urinary changes can often be improved with physiotherapy, compression garments, and skincare.
- Emotional health: Anxiety, low mood, and fear of recurrence are normal. Psychological support, counseling, and peer support groups are valuable resources.
- Follow-up care: Attending all scheduled follow-up appointments is essential; they typically include vulvar and groin examinations and, when needed, imaging.
Women are encouraged to raise any concern with their care team — no question is too small or too embarrassing.
When to See a Doctor
Arrange a medical evaluation promptly if you experience any of the following:
- A lump, thickened area, or growth on the vulva
- A sore or ulcer that does not heal within 2–4 weeks
- Persistent vulvar itching or burning that does not respond to standard treatment
- Bleeding or blood-stained discharge unrelated to menstruation
- Changes in skin color (white, red, or dark patches)
- A new or changing mole or dark spot on the vulva
- Pain during urination or intercourse
- A lump or swelling in the groin
Early assessment can be lifesaving. Most of these symptoms will turn out to have a non-cancerous cause — but they should always be checked rather than ignored.
Key Points to Remember
- Vulvar cancer is rare but treatable, especially when caught early.
- It can look like a lump, non-healing sore, wart-like growth, discolored patch, or changing mole.
- Persistent itching is the most common symptom and deserves investigation if it does not resolve.
- HPV infection, lichen sclerosus, smoking, and older age are the main risk factors.
- HPV vaccination, smoking cessation, and proper management of skin conditions reduce risk.
- A biopsy is the only way to confirm or exclude vulvar cancer.
- Never let embarrassment prevent you from seeking medical care about vulvar changes.
References
- American Cancer Society (2024) Vulvar Cancer. Atlanta, GA: American Cancer Society. Available at: https://www.cancer.org/cancer/types/vulvar-cancer.html (Accessed: 2024).
- Berek, J.S. and Hacker, N.F. (2021) Berek & Hacker’s Gynecologic Oncology. 7th edn. Philadelphia: Wolters Kluwer.
- Cancer Research UK (2024) Vulval cancer. London: Cancer Research UK. Available at: https://www.cancerresearchuk.org/about-cancer/vulval-cancer (Accessed: 2024).
- European Society of Gynaecological Oncology (ESGO), European Society for Radiotherapy and Oncology (ESTRO) and European Society of Pathology (ESP) (2023) Vulvar cancer: ESGO/ESTRO/ESP Guidelines for the management of patients with vulvar cancer — Update 2023. International Journal of Gynecological Cancer, 33(7), pp. 1023–1043.
- International Federation of Gynecology and Obstetrics (FIGO) Committee on Gynecologic Oncology (2022) ‘Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium’, International Journal of Gynaecology and Obstetrics, 157(2), pp. 219–228.
- Koh, W.J., Abu-Rustum, N.R., Bean, S., Bradley, K., Campos, S.M., Cho, K.R., Chon, H.S., Chu, C., Cohn, D., Crispens, M.A. and Damast, S. (2019) ‘Vulvar Cancer, Version 3.2019, NCCN Clinical Practice Guidelines in Oncology’, Journal of the National Comprehensive Cancer Network, 17(4), pp. 345–378.
- National Cancer Institute (2024) Vulvar Cancer Treatment (PDQ) — Health Professional Version. Bethesda, MD: National Cancer Institute. Available at: https://www.cancer.gov/types/vulvar/hp/vulvar-treatment-pdq (Accessed: 2024).
- NHS England (2023) Vulval cancer. London: National Health Service. Available at: https://www.nhs.uk/conditions/vulval-cancer/ (Accessed: 2023).
- Oonk, M.H.M., Planchamp, F., Baldwin, P., Bidzinski, M., Brännström, M., Landoni, F., Mahner, S., Mahantshetty, U., Mirza, M., Petersen, C., Querleu, D., Regauer, S., Rob, L., Rouzier, R., Ulrikch, E., van der Velden, J., Vergote, I., Woelber, L. and van der Zee, A.G.J. (2017) ‘European Society of Gynaecological Oncology Guidelines for the Management of Patients with Vulvar Cancer’, International Journal of Gynecological Cancer, 27(4), pp. 832–837.
- Rogers, L.J. and Cuello, M.A. (2018) ‘Cancer of the vulva’, International Journal of Gynaecology and Obstetrics, 143(Suppl 2), pp. 4–13.
- van der Stoep, N., van der Avoort, I.A.M. and de Hullu, J.A. (2022) ‘Human papillomavirus and vulvar cancer: an update on prevention and management’, Current Opinion in Oncology, 34(5), pp. 483–491.
- World Health Organization (2020) WHO Classification of Female Genital Tumours. 5th edn. Lyon: International Agency for Research on Cancer.
Disclaimer: This article is intended for general informational purposes only and does not substitute professional medical advice, diagnosis, or treatment. Always consult a qualified healthcare provider with any questions regarding a medical condition.