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Carcinoma of the vulva accounts for 4% of all female genital tract malignancies.1 The incidence in the UK is two per 100,000 female population.2 Vulval cancer is rare in young women with rates of more than one per 100,000 among women aged 25–44 years in the UK.2 In the US, 15% of vulval cancers are reported to occur in women <50 years of age.3,4 Prior studies have reported an incidence of 3.3% in women <35 years old,5 2.3% in women <40 years old and 6.1% in women <45 years old.6
In recent years, an increased incidence of vulval cancer in younger women has been observed.7–10 The UK incidence in younger women has doubled in the last three decades.2 Predisposing factors include infection with human papillomavirus (HPV) or herpes simplex virus type 2,11–13 vulval intra-epithelial neoplasia (VIN), lichen sclerosis, smoking11,14 and immunosuppression. HPV is strongly linked with tumours in young women, with an 11-fold increase reported for VIN and early-stage cancer in women <45 years of age with serological evidence of HPV infection.15 The increased incidence of vulval cancer is linked worldwide with an increasing incidence of VIN16,17 in younger women caused by HPV infection.18–20 Immunosuppression in young patients is identified as accelerating the progression of VIN to invasive vulval squamous cell carcinoma.21 There is an increased risk in HIV-positive women,10,21,22 with a strong relationship for women <30 years of age.23,24 HIV is also reported to cause rapid progression of these cancers.25 In the UK the annual number of newly diagnosed HIV individuals has increased by 182% over the past 10 years, with the majority of newly diagnosed persons aged between 25 and 44 years.26
Reports of either vulval carcinoma presenting during pregnancy,25,27–29 or of pregnancy following treatment for vulval cancer, are rare29–31 because the majority of women with the condition are perimenopausal with completed families, post-menopausal or rendered infertile or of reduced fertility following treatment with radiotherapy and chemotherapy.31 With the increasing incidence of HPV, VIN and HIV, and a concomitant increase in the incidence of vulval carcinoma in younger women, we are progressively more likely to encounter patients with either vulval carcinoma in pregnancy or pregnancy following treatment for vulval cancer.