Diseases of the Vulva

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Chapter 8 Diseases of the Vulva

Vulval dermatoses

Vulval dermatoses is the term applied to non-infective non-neoplastic diseases of the vulval skin. Their management has evolved into a multidisciplinary approach involving specialist nursing staff, dermatologists and gynaecologists. Specialist clinics may have direct referrals, but the symptoms of itching, soreness and dyspareunia will usually take the patient in the first place to the gynaecologist.

Normal vulva

Lichen sclerosus

Lichen sclerosus is a common condition found in postmenopausal women complaining of vulval itch. Premenopausal women may also be affected, as well as men and children. The aetiology is unknown, but it appears to be associated with autoimmune disorders.

It is most commonly seen in the vulvo-perineal skin of women, but can affect skin in any region of the body.

Benign tumours of the vulva

The complaint is usually of a ‘lump’ or ‘swelling’ at the vaginal introitus. (In many cases, the patient may mistake prolapse of various types for tumour growth.)

Cyst of Bartholin’s gland This is the commonest simple tumour of the vulva. Caused by obstruction of the duct, the cyst often becomes infected and requires surgical treatment (see p. 133).

Sebaceous cyst of the vulva occurs in the hair-bearing vulval tissue. The cyst may become infected and cause pain. The cyst contains whitish, cheesy sebaceous material. If painful, it can be removed.

Vulval haematoma is a result of direct violence or wounding (it is most commonly found with childbirth). Treatment is by incision, evacuation and drainage if the patient is symptomatic.

Lipoma, fibroma and myoma are found rarely and can become sarcomatous. Treatment is by excision, following appropriate imaging assessment. Magnetic resonance imaging (MRI) is usually the imaging modality of choice.

Vulval intraepithelial neoplasia

Vulval intraepithelial neoplasia (VIN)

There are two main types of VIN – usual and differentiated. Usual type is associated with oncogenic human papilloma virus (HPV), whereas differentiated type is not always associated with HPV infection; however, both can be precursors of vulval carcinoma.

HPV infection can infect all cloacal origin epithelium causing intraepithelial neoplasia. The site of epithelium affected dictates the term used to describe it: vaginal intraepithelial neoplasia (VAIN); anal intraepithelial neoplasia (AIN); and peri-anal intraepithelial neoplasia (PAIN). As such, intraepithelial neoplasia can often be a multifocal disease with histological features similar to that of cervical intraepithelial neoplasia (CIN). The malignant potential of VIN is thought to be less than that of CIN. However, the mechanism whereby it causes dyspaltic change is the same and can be reviewed on page 175.

VIN may present with vulval itch, burning or pain. However, it may be detected when abnormal skin is identified by the patient or when the patient is undergoing gynaecological examination for other reasons.

There is no distinct appearance, with the changes often being subtle. Lesions may be macular, papular with the abnormal area appearing mainly white but also red or pigmented in some cases. Diagnosis can be confirmed by biopsy.

Treatment of VIN

1. Careful observation only as spontaneous resolution can occur.

2. Wide local excision to achieve a clear margin, with or without plastic surgery.

3. CO₂ laser vaporisation.

Following treatment, patients should be seen regularly in the clinic as recurrent or new intraepithelial neoplasia can occur necessitating further treatment.

Paget’s disease of the vulva (adenocarcinoma in situ)

This malignant disease starts as an erythematous, irritant plaque which becomes eczematous (oedema and exudate) and spreads. The disease is multi-focal and the true margins are indistinct. To determine the margins, mapping biopsies from the periphery are required. Histology shows the characteristic Paget’s cells – large round, clear-staining cells with large nuclei, often mitotic.

Thirty percent patients may have an underlying adenocarcinoma of the vulva, cervix, bladder, ovary, rectum or breast. Wide local excision of the lesion is the treatment of choice and plastic surgical reconstruction may be required.

Carcinoma of the vulva

Vulval cancer is very rare in young women.

Incidence

1 per 100,000 women aged 25–44

3 per 100,000 in those aged 45–64

13.2 per 100,000 in women aged 65 and over (UK).

Aetiology

The aetiology of squamous vulval cancer is related to the underlying vulval skin disorder.

The differentiated type is associated with underlying conditions such as lichen sclerosis and is not HPV related. The remaining squamous cancers are associated with oncogenic HPV infection. The mechanism for oncogenic HPV cancer induction can be reviewed on page 175.

Histology

Squamous carcinoma: 85%

Melanoma: 5%

Clinical features

The majority of patients with vulval cancer are over 60. The presenting features include vulval irritation and pruritus (70%), a vulval mass (60%) and bleeding (30%).

The diagnosis is often delayed, partly due to the patient’s reluctance to seek medical help, and partly because of delay in performing a clinical examination once the patient presents.

Any lump on the vulva must be examined and if suspicious biopsied.

The inguinal lymph nodes may be enlarged, but absence of enlargement does not guarantee absence of lymphatic spread.

The FIGO staging of vulvar cancer 2009

Stage	Definition
Stage I	Confined to vulva.
IA	Tumour 2 cm or less maximum diameter. Stromal invasion no greater than 1 mm. No nodal metastases.
IB	As for IA, but stromal invasion greater than 1 mm.
Stage II	Tumour of any size with extension to adjacent perineal structures (lower 1/3 urethra, lower 1/3 vagina, anus). Negative nodes.
Stage III	Tumour of any size with or without extension to adjacent perineal structures (1/3 lower urethra, 1/3 lower vagina, anus) with positive inguino-femoral lymph nodes.
III A	(i) With 1 lymph node metastasis (≥5 mm), or (ii) 1–2 lymph node metastasis(es) (<5 mm).
III B	(i) With 2 or more lymph node metastases (≥5 mm), or (ii) 3 or more lymph node metastases (b5 mm).
III C	With positive nodes with extracapsular spread.
Stage IV A	Tumour invades any of the following: (i) upper urethral and/or vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to pelvic bone, or (ii) fixed or ulcerated inguino-femoral lymph nodes.
Stage IV B	Any distant metastasis, including pelvic lymph node.