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Introduction
Clinical features
Differential diagnoses
Complications
Diagnosis
Treatment
Vulval SCC
Vulval melanoma
Vulval BCC
Vulval extramammary Paget disease
Bartholin gland carcinoma
Vulval (vulvar) cancer is any malignancy arising on any part of the vulva, the external female genitalia. It is the fourth most common location for a gynaecological malignancy after the uterus, ovary, and cervix, accounting for 3–5% of female genital tract malignancies.
Over 80% of vulval cancers are squamous cell carcinomas (SCC) including both precursor lesions and invasive disease. Melanoma and basal cell carcinoma (BCC) are the next most common types of vulval cancer.
Uncommon causes of vulval cancer include:
The pathogenesis, epidemiology, clinical features, and specific treatment options for each of these are covered in more detail below.
Rare vulval cancers that will not be covered in any further detail here include:
Metastases from non-vulval primary malignancies may present as progressively enlarging vulval nodules, e.g. breast, cervix, ovary, endometrium, kidney, and colon.
Vulval cancer is often asymptomatic and found incidentally on examination or as a lump noticed on wiping.
Symptoms can be nonspecific and may indicate late disease. These include:
Routine examination of the vulva during a skin check or gynaecological examination may detect a suspicious lesion the patient is unaware of.
Vulval cancers can only be diagnosed on biopsy.
Clinical examination may need to include general skin examination, palpation of regional lymph nodes, pelvic examination, cystoscopy, proctoscopy, and/or colposcopy with cervical cytology.
Further investigations may be required to stage the disease:
Vulval cancers are usually treated by surgical excision, local or radical, with or without lymph node removal, and psychosexual support.
Vulval cancer requires regular careful follow-up as recurrence is common.
Vulval squamous cell carcinoma has a reported annual incidence of 2–7 cases per 100,000 women. Although it was regarded as a disease of postmenopausal women with a median age at diagnosis of 69 years in the USA, over the past forty years the incidence has increased markedly in women under 50 years of age. This is probably due to a change in sexual habits and increased exposure to human papillomavirus (HPV).
Vulval SCC risk increases with:
The precursor lesion for vulval SCC is vulval intraepithelial neoplasia (VIN) which can be classified as:
Stage |
Description |
I |
Tumour confined to the vulva IA: Tumour size ≤2 cm and stromal invasion ≤1 mma IB: Tumour size >2 cm or stromal invasion >1 mm (a) |
II |
Tumour of any size with extension to lower one-third of urethra, lower one-third of vagina, or lower one-third of anus, with negative lymph nodes |
III |
Tumour of any size with extension to upper part of adjacent perineal structures, or with any number of non-fixed, nonulcerated lymph nodes IIIA: disease extension to upper two-thirds of urethra or vagina, bladder or rectal mucosa, or regional nodes(b) ≤5 mm IIIB: regional lymph nodes(b) >5mm IIIC: regional lymph node(b) metastases with extracapsular spread |
IV |
Tumour of any size fixed to bone, or fixed ulcerated lymph node metastases, or distant metastases IVA: disease fixed to pelvic bone or fixed or ulcerated regional lymph node metastases IVB: distant metastases |
a depth of invasion is measured from the basement membrane of the deepest adjacent tumour-free rete ridge to the deepest point of invasion.
b regional lymph nodes refers to inguinal and femoral lymph nodes.
Vulval HSIL tends to be slow-growing with spontaneous regression in some cases. Differentiated VIN rapidly progresses to invasive SCC with a high recurrence rate and worse prognosis. Vulval SCC presents with locally advanced disease in 30% of cases which may be unresectable. The 5-year recurrence rate is 37%. Prognosis is poor with lymph node or distant metastases.
Vulval melanoma accounts for 5–10% of all vulval cancers and 2% of all melanomas in women. The vulva is at a higher risk of developing melanoma than the skin elsewhere. The reported incidence is <0.2 cases/100,000 women with a wide reported age range of 10–107 years, predominantly in white women aged 40–60 years.
Vulval melanoma should be staged using the AJCC system.
Vulval melanoma diagnosis is often delayed and is clinically aggressive with one-third having regional and/or distant metastases at presentation. Median survival is approximately 50 months and five-year survival less than 50% (compared to cutaneous melanoma 92%) due to late presentation, different tumour biology, and approach to treatment.
Vulval basal cell carcinoma is typically diagnosed in white women aged 60–80 years (mean 70 years).
Multiple lesions are associated with basal cell naevus syndrome (BCNS).
Vulval basal cell carcinoma is typically slow-growing with a low risk of metastasis. Local invasion can occur if untreated resulting in tissue destruction.
The vulva is the commonest site for EMPD (65%) and typically affects Caucasian postmenopausal women (median age 72 years).
Vulval EMPD often requires multiple biopsies to make the diagnosis and classify as:
Vulval EMPD typically follows an indolent course with a good prognosis although diagnosis is often late and recurrence is common. Invasive vulval EMPD has a worse prognosis than in situ disease.
Bartholin gland carcinoma is a rare cause of vulval cancer (2–7%) presenting in women aged 40–50 years.
Bartholin gland carcinoma recurs following surgery in one-third of cases. Prognosis depends on the FIGO stage.
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