21 Vulval cancer accounts for approximately 4% of all gynaecological malignancies and 1% of all cancers in women. Ninety per cent of vulval cancer consists of squamous cell carcinoma. Vulval squamous cell carcinoma (VSCC) is the fourth most common gynaecologic cancer, following cancer of the uterine corpus, ovary and cervix. The five‐year survival rate is 70% but morbidity is high, despite the change in surgery to less invasive procedures over the last few decades, which has not changed survival but has decreased the complication rate and improved quality of life. This is particularly important because the rate of vulval cancer in younger women has increased dramatically since the 1960s due to the rise in HPV‐related tumours. Vulval squamous cell carcinoma is mainly diagnosed in elderly women with a mean age at diagnosis of approximately 70 years. Fifteen per cent occur in women under 50 years of age. The incidence is around 2 per 100 000 women per year. Mortality rates have been stable in younger women but have declined in older women. Risk factors are HPV‐related undifferentiated vulval intraepithelial neoplasia (uVIN) and lichen sclerosis (LS). Risk factors associated with HPV infection include early age at first intercourse, multiple sexual partners, human immunodeficiency virus (HIV) infection and cigarette smoking. Two independent pathways of vulvar carcinogenesis currently exist, both with their own premalignant lesions and invasive histologic forms. One type is related to LS and the second type is related to HPV infection. The most common type occurs in elderly women on a background of LS and often differentiated VIN (dVIN) and leads to mostly keratinizing carcinoma. There is no relation to HPV. Differentiated VIN is underreported and has probably a relatively short intraepithelial phase before progression starts (see Chapter 19). The second type consists of mainly nonkeratinizing carcinomas and affects primarily younger women. It is caused predominantly by HPV 16 and 18 infection, resulting in undiffentiated VIN (uVIN), which may lead to VSCC. These women tend to present with early‐stage disease. Verrucous carcinoma is a variant of VSCC with cauliflower‐like appearance (Figure 21.1). It has a verrucous configuration on histology. The lesion grows slowly and rarely metastasizes to lymph nodes but it may be locally destructive. Figure 21.1 Verrucous squamous cell carcinoma. Pruritus or pain is a common complaint. Vulval bleeding or discharge, dysuria, or an enlarged lymph node in the groin, are less frequently encountered symptoms and suggestive of advanced disease. On the other hand, patients may be asymptomatic at the time of diagnosis. Most patients present with a unifocal vulval plaque, ulcer or mass on either the background of multifocal uVIN or LS. Multifocality may be present. A synchronous second malignancy, most commonly cervical neoplasia, is found in up to 20% of patients. Vulval squamous cell carcinoma metastasizes by a variety of mechanisms. Modes of spread include direct extension to adjacent structures (e.g. vagina, urethra, clitoris, anus), lymphatic spread to regional lymph nodes and hematogenous dissemination. Most VSCC spread first to the groin, which is why these nodes are sampled as part of staging (see Box 21.1). Lymphatic spread to regional lymph nodes can occur early in the course of disease, even in patients with small lesions. Lesions that are on one side of the vulva generally spread only to the ipsilateral groin nodes. The pretreatment risk of groin lymph node metastases appears to be in the order of 15%. Lymphatic spread does not occur in lesions with less than 1 mm depth of invasion. In tumours up to 2 mm, the chance of invasion is 8%, and in those more than 5 mm, 34% are likely to have lymph node involvement. Groin lymph node metastases are associated with a poorer prognosis and adjuvant radiotherapy is indicated in these patients. Palpation of the groin is part of the physical examination but this it inadequate. Among patients with no enlargement with palpation of groin nodes, 16% to 24% have clinically occult metastases. Hematogenous dissemination, which typically occurs late in the course of the disease to lungs, liver and bones, is rare in patients without inguinofemoral lymph‐node involvement.
Vulval Squamous Cell Carcinoma
Introduction
Epidemiology
Aetiology / histology
Symptoms and Clinical Features
Management
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