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UK Vulva Cancer incidence statistics

This page presents vulval cancer incidence statistics by age, trends over time, geographical variation and socio-economic status. The ICD code for vulval cancer is ICD9 184.4 and ICD10 C51.

The vulva site includes the labia majora, labia minora and the clitoris (Figure 1.1).

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Cancer of the vulva is rare and, when coupled with cancer of the vagina, accounts for less than 1% of all cancer cases (excluding non-melanoma skin cancer) and 6% of gynaecological cancers in the UK.

Incidence rates for vulval cancer in younger women have doubled in the last three decades, survival rates have improved over the last 30 years. This has resulted in a fall in mortality rates.

Infection with the human papillomavirus (HPV) is a major cause of vulval cancers, along with smoking. Unlike other reproductive tract cancers – with the exception of vaginal and cervical cancer – risk of vulval cancer is not related to reproductive factors or exogenous hormones.

In 2004, 1,022 new cases of vulval cancer were diagnosed.(Table 1.1).1-4 The European age-standardised incidence rate of vulval cancer in the UK is around 2 per 100,000 female population.1-4

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Vulval cancer incidence by age

Vulval cancer is very rare in young women aged under 25. Rates are less than 1 per 100,000 among women aged 25–44, rising to 3 per 100,000 in those aged 45–64, and peak at 14 per 100,000 in women aged 65 and over (Figure 1.2).1-4

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Vulval cancer incidence trends

While there were slight falls in the incidence of vulval cancer between 1975 and the mid-1980s, increases since the mid-1990s mean that incidence has now reached a similar level to that of 1975 (Figure 1.3).1-4

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There has been a significant increase in rates of vulval cancer in younger women. The proportion of cases diagnosed under the age of 50 rose from 6% in 1975 to 11% in 2004.1-4A similar trend has been documented in other countries, 5,6 and has been linked to increasing incidence of vulval intraepithelial neoplasia (VIN) in young women caused by infection with HPV.6

Figure 1.4 shows the vulval cancer incidence trend for the UK.

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Geographical variation in vulval cancer incidence

It is estimated that almost 27,000 women worldwide are diagnosed with vulval cancer each year.7

Rates range from less than 0.3 per 100,000 females in parts of Asia to about 1.6 per 100,000 females in north America and Europe ( Figure 1.4).7 This variation is probably related to differing prevalence of HPV infection in world regions, and other lifestyle factors, especially smoking, and their interaction with HPV (see Risk factors).

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Socio-economic status and vulval cancer incidence

Several case-control studies have reported an increased risk of vulval cancer associated with lower socio-economic status and fewer years of education.8-14

Vulval cancer histology

Squamous cell carcinomas (SCC) account for more than 90% of vulval cancers.15 The other 10% includes melanomas, sarcomas, basal cell carcinomas and adenocarcinomas.16

Pre-cancerous lesions of the vulva

A substantial proportion of vulval invasive tumours are found with adjacent evidence of pre-cancers, known collectively as vulval intraepithelial neoplasia (VIN).

There are two main types of VIN. HPV-related, which precedes almost all vulval cancers in women under 45, and lichen sclerosus-related, the major cause of vulval cancer in older women.17,18

The classification system for VIN changed in 2004 to better reflect the two divergent types of lesions (Table 1.2).19,20

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Annual risk of invasive vulval cancer in women with untreated carcinoma in situ (high-grade VIN) of the vulva is at least 10%, while risk of progression in treated lesions over a period of years is between 2% and 5%.21 The absolute risk for women treated for lichen sclerosus is at the top end of this range,20 and relative risks greater than 300 have been reported for vulval SCC in women who have undergone treatment.22,23

References for vulva cancer incidence

1. ISD Scotland Online Cancer Registrations in Scotland.
2. Cancer Registrations in Northern Ireland Northern Ireland Cancer Registry
3. Cancer Registrations in England, 2004 Office for National Statistics
4. Cancer Registrations in Wales Welsh Cancer Intelligence and Surveillance Unit
5. Jones, R.W., J. Baranyai, and S. Stables,Trends in squamous cell carcinoma of the vulva: the influence of vulvar intraepithelial neoplasia. Obstet Gynecol, 1997. 90(3): p. 448-52
6. Joura, E.A., et al.,Trends in vulvar neoplasia. Increasing incidence of vulvar intraepithelial neoplasia and squamous cell carcinoma of the vulva in young women. J Reprod Med, 2000. 45(8): p. 613-5
7. Sankaranarayanan, R. and J. Ferlay,Worldwide burden of gynaecological cancer: the size of the problem. Best Pract Res Clin Obstet Gynaecol, 2006. 20(2): p. 207-25
8. Parazzini, F., et al.,Selected food intake and risk of vulvar cancer. Cancer, 1995. 76(11): p. 2291-6
9. Newcomb, P.A., N.S. Weiss, and J.R. Daling,Incidence of vulvar carcinoma in relation to menstrual, reproductive, and medical factors. J Natl Cancer Inst, 1984. 73(2): p. 391-6
10. Basta, A., K. Adamek, and K. Pitynski,Intraepithelial neoplasia and early stage vulvar cancer. Epidemiological, clinical and virological observations. Eur J Gynaecol Oncol, 1999. 20(2): p. 111-4
11. Brinton, L.A., et al.,Case-control study of cancer of the vulva. Obstet Gynecol, 1990. 75(5): p. 859-66
12. Trimble, C.L., et al.,Heterogeneous etiology of squamous carcinoma of the vulva. Obstet Gynecol, 1996. 87(1): p. 59-64
13. Hildesheim, A., et al.,Human papillomavirus type 16 and risk of preinvasive and invasive vulvar cancer: results from a seroepidemiological case-control study. Obstet Gynecol, 1997. 90(5): p. 748-54
14. Daling, J.R., et al.,A population-based study of squamous cell vaginal cancer: HPV and cofactors. Gynecol Oncol, 2002. 84(2): p. 263-70
15. Woolas, R.P. and J.H. Shepherd, Current developments in the management of vulval carcinoma, in The Yearbook of Obstetrics and Gynecology, P.M.S. O'Brien, Editor. 1999, RCOG Press.
16. Daling, J.R. and J.H. Sherman, Cancers of the vulva and vagina, in Cancer epidemiology and prevention D. Schottenfeld and J. Fraumeni Jr, Editors. 1996, OUP: Oxford. p. 1117-1129.
17. Ridley, C.M.,The aetiology of vulval neoplasia. Br J Obstet Gynaecol, 1994. 101(8): p. 655-7
18. Canavan, T.P. and D. Cohen,Vulvar cancer. Am Fam Physician, 2002. 66(7): p. 1269-74
19. Sideri, M., et al.,Squamous vulvar intraepithelial neoplasia: 2004 modified terminology, ISSVD Vulvar Oncology Subcommittee. J Reprod Med, 2005. 50(11): p. 807-10
20. Perrett, C.W., The molecular biology of lichen sclerosus and the development of cancer, in Lower Genital Tract Neoplasia, A.B. MacLean, A. Singer, and H. Critchley, Editors. 2003, RCOG Press: London.
21. Jones, R.W.,Vulval intraepithelial neoplasia: current perspectives. Eur J Gynaecol Oncol, 2001. 22(6): p. 393-402
22. Carli, P., et al.,Squamous cell carcinoma arising in vulval lichen sclerosus: a longitudinal cohort study. Eur J Cancer Prev, 1995. 4(6): p. 491-5
23. Scurry, J.P. and K. Vanin,Vulvar squamous cell carcinoma and lichen sclerosus. Australas J Dermatol, 1997. 38 Suppl 1: p. S20-5

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