Vulvar Cancer

Vulvar cancer is a malignancy affecting the vulva, the external part of the female genitalia, predominantly impacting the labia majora but can also involve the labia minora, clitoris, and Bartholin's glands. It is primarily diagnosed in women over the age of 45.

Signs and Symptoms

The clinical presentation of vulvar cancer includes:

• Persistent itching, burning, or bleeding on the vulva.
• Changes in vulvar skin colour, appearing redder or whiter than usual.
• Skin changes resembling a rash or warts.
• Sores, lumps, or ulcers on the vulva that do not heal.
• Pelvic pain, notably during urination or sexual intercourse.

Causes and Risk Factors

The development of vulvar cancer is attributed to two main pathways: human papillomavirus (HPV) infection and chronic inflammation or autoimmune conditions affecting the vulva. HPV DNA is found in up to 87% of vulvar intraepithelial neoplasia (VIN) and 29% of invasive vulvar cancers, with HPV 16 being the most common subtype.

Risk factors include:

• Increasing age and a history of vulvar or cervical intraepithelial neoplasia.
• Multiple male sexual partners and a history of cervical cancer.
• Smoking and infection with human immunodeficiency virus (HIV).
• Vulvar lichen sclerosus and immunodeficiency syndromes.
• Northern European ancestry.

Diagnosis

Diagnosis involves a thorough examination of the vulva, including the perineum, clitoris, urethra, and Bartholin's glands. Suspicious lesions are biopsied, generally under local anaesthesia. Additional evaluations may include chest X-rays, cystoscopy, proctoscopy, and blood tests.

Types of Vulvar Cancer

Squamous Cell Carcinoma

Squamous cell carcinoma accounts for approximately 75% of vulvar cancers. It originates from epidermal squamous cells and may arise from two precursor lesions: usual-type VIN (uVIN) and differentiated VIN (dVIN). These lesions tend to grow locally and spread via the lymphatic system.

Basal Cell Carcinoma

Representing about 8% of vulvar cancers, basal cell carcinoma typically affects older women and grows slowly with a low risk of metastasis. Treatment involves local excision.

Melanoma

Melanoma accounts for 6% of vulvar cancers and arises from melanocytes. It has a poorer prognosis compared to skin melanomas, with a high rate of lymph node involvement at diagnosis. Treatment may include checkpoint inhibitors for advanced stages.

Bartholin Gland Carcinoma and Other Lesions

Bartholin gland carcinoma is rare and usually occurs in women in their mid-sixties. Other forms include invasive Extramammary Paget's disease, adenocarcinoma, and sarcoma.

Staging

The FIGO staging system uses tumour size, lymph node involvement, and metastasis presence to stage vulvar cancer.

Treatment

Treatment primarily involves surgery, which may include wide local excision, radical vulvectomy, and lymph node removal. Sentinel lymph node dissection is used to minimise adverse effects.

Radiation therapy is used for advanced cases, sometimes in conjunction with chemotherapy. Checkpoint inhibitors may be administered for vulvar melanoma.

Prognosis

The five-year survival rate for vulvar cancer is approximately 71%, but this varies significantly with cancer stage and lymph node involvement. Early-stage detection is very important for a favourable prognosis.

Epidemiology

In 2018, vulvar cancer affected about 44,200 people globally, resulting in 15,200 deaths. In the UK, it accounts for about 6% of gynecologic cancers, with 1,200 diagnoses and 400 deaths annually. In the US, it constitutes about 0.3% of new cancer cases, with approximately 6,070 new cases and 1,280 deaths per year.

---

Self-assessment MCQs (single best answer)

Which part of the female genitalia is predominantly affected by vulvar cancer?

What is a common sign of vulvar cancer?

Which virus is most commonly associated with vulvar cancer?

What percentage of vulvar cancers are squamous cell carcinomas?

Which staging system is used for vulvar cancer?

What is the five-year survival rate for vulvar cancer?

Which treatment is primarily used for vulvar cancer?

What is a risk factor for developing vulvar cancer?

What percentage of vulvar cancers are basal cell carcinomas?

Which of the following is a diagnostic method for vulvar cancer?