Premalignant and malignant vulval diseases

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Chapter 22 Premalignant and malignant vulval diseases

Vulval intraepithelial neoplasia (VIN)

VIN was once classified according to the degree of abnormality into VIN1, VIN2 and VIN3 (or mild, moderate and severe dysplasia). Since there is no evidence to suggest VIN 1 is precancerous, it is now called low-grade VIN, while VIN2 and VIN3 are referred to as high-grade VIN.

Classification

The International Society for the Study of Vulvar Diseases 2004 classifies high-grade VIN:

• The usual type (warty, basaloid and mixed) is related to human papillomavirus (HPV) (mostly 16, 18 and 31), is often multifocal, and it occurs in younger, premenopausal women.

• The differentiated type is non-HPV related, usually unifocal, and is seen in older women with chronic vulvar dystrophy, such as lichen sclerosus or squamous cell hyperplasia.

General

• The incidence of VIN is increasing worldwide, especially in younger women.

• Risk factors are smoking, immunosuppression and HPV infection.

• There are up to 50% with associated cervical or vaginal intraepithelial neoplasia.

• Clinical presentation includes: pruritus, perineal pain, vulval lesion, with 50% asymptomatic.

• Diagnosis is by tissue biopsy after colposcopic examination. The entire lower genital tract and perianal area should be examined.

Treatment

• Treatment should be individualised depending on histology, location and extent of disease.

• The goal of treatment is to prevent development of cancer and to relieve symptoms.

• Surgical treatment is preferable, and this includes wide local excision, skinning vulvectomy and laser ablation.

• Medical treatment is reserved for situations where surgery is not feasible. This includes topical imiquimod cream (Aldara) or 5-fluorouracil (5-FU).

• The response rate for medical therapy has been reported to be 75%.

• The recurrence rate for VIN is about 30%. Therefore, long-term follow-up is necessary.

Paget’s diseases of vulva

Paget’s disease is an intraepithelial neoplasia. About 10% of patients have invasive Paget’s disease, and 4%–8% have an underlying adenocarcinoma.

• It mostly affects postmenopausal white women in their 60s and 70s.

• Symptoms include pruritus and vulvar pain.

• The lesion is well demarcated and has an eczematoid appearance with slightly raised edges.

• Diagnosis is by tissue biopsy.

• Patients should be investigated for synchronous cancer, as 25% may have a non-contiguous cancer involving the breast, rectum, bladder, urethra, cervix or ovary.

• Treatment is by wide local excision, including underlying dermis. Positive margins can be common, as Paget’s disease usually extends well beyond the gross lesion.

• Recurrent local recurrences are common and they are treated with surgical excision.

Vulval cancer

Vulval cancer accounts for 3%–5% of all gynaecological cancers.

Aetiology

• There is no clear aetiological agent. Smoking is a risk factor.

• It mainly occurs in postmenopausal women with a long history of vulval irritation/pruritus.

• It is often associated with vulval skin dystrophy; however, dystrophy may be a consequence of irritation and scratching.

• Dystrophies appear to have malignant potential only if there is evidence of atypia.

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