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.

Vulval cancer incidence in the UK

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 7% of gynaecological (ovarian, uterine, cervical, vaginal and vulval) cancers diagnosed in women in the UK.

It has been estimated that the lifetime risk of developing vulval cancer is 1 in 316 for women in the UK. These were calculated on February 2009 using incidence and mortality data for 2001-2005 24.

In 2006, 1,063 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 around 1 per 100,000 among women aged 25-44, rising to around 3 per 100,000 in those aged 45-64, and peak at 13.2 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 15% in 2006. 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 1.5). 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

UK Vulva Cancer mortality statistics

This page presents vulval cancer mortality statistics by age and trends.

Vulval cancer mortality in the UK

Each year in the UK, there are between 300 and 400 deaths from vulval cancer. 1-3 The European  age-standardised death rate for vulval cancer is 0.6 per 100,000 female population. 1-3Table 2.1 shows the average number of deaths and mortality rates over the five year period, 2003 to 2007.

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Vulval cancer mortality by age

The mortality rate for vulval cancer is 0.1 per 100,000 women aged 25-44, 0.5 for women aged 45-64, rising to 6 per 100,000 women aged 65 and above.( Figure 2.1) The rate continues to increase after this age, to nearly 12 per 100,000 women aged 80 and over. 1-3

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Vulval cancer mortality trends

Mortality rates for vulval cancer in the UK have declined steadily since the early 1970s. The rate fell by more than half (52%) from 1.3 per 100,000 female population in 1971 to 0.6 per 100,000 in 2007 ( Figure 2.2). 1,2, 3

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Among women aged 45-64, the vulval cancer mortality rate in the UK dropped by almost 70% between 1971 and 2007, from 1.4 per 100,000 to 0.4 per 100,000 women. The rate among women aged 65 and over fell from 8.8 per 100,000 population in 1971 to 6 per 100,000 in 2007, a decrease of 31%. 1-3

Vulva Cancer survival statistics

This page presents vulval cancer survival statistics including trends, deprivation, age and stage at diagnosis.

Survival data is only available for cancers of the vagina and vulva combined. Five-year relative survival rates for vulval and vaginal cancers vary significantly by stage of disease and age at diagnosis. The overall five-year relative survival rate for women diagnosed with cancers of the vulva and vagina in England and Wales in 1996-99 was 58%, and the one-year survival rate was 76%. 1

Vulva cancer survival trends

One- and five-year relative survival rates for vaginal and vulval cancer among women diagnosed in England and Wales improved by an average of 3% every five years between 1971 and 1990. This trend continued for patients diagnosed in the 1990s. Rates improved from 62% to 76% for one-year survival and from 40% to 58% for five-year survival between the periods 1971-75 and 1996-99 ( Figure 3.1). 1, 2

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Deprivation and vulva cancer survival

Evidence suggests that survival rates for the most deprived women are significantly lower than rates for the most affluent patients. 3

Vulva cancer survival by age

Vulva cancer survival rates decrease with increasing age at diagnosis. Among women diagnosed between 1996-99 in England and Wales, the five-year relative survival rates for those aged 15-49 was 83%, for women aged 50-69 it was 65% and for those aged 70+ it was 52% ( Figure 3.2). 1

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Vulva cancer survival and stage at diagnosis

An international survey of patients with vulval cancer reported five-year survival of 31% for women with a FIGO stage III tumour, and 77% for women with stage I tumours.4 The five-year survival rate for node-negative patients following surgery is 70-90% but falls to 25-40% if nodes are involved. 4, 5

Vulva Cancer risk factors

Vulval cancer is caused by sexually transmitted infections (STIs) - primarily HPV - and is subject to some additional risk factors, including other sexually transmitted infections, other medical conditions, smoking, iatrogenic immunosuppression and other factors.

Vulva cancer risk and HPV infection

HPV - human papillomavirus - is present in a proportion of vulval tumours, and HPV 16 is the most commonly detected type. 1, 2

About 70-90% of in situ vulval squamous cell carcinoma (SCC) contain HPV DNA, 3 while the detection rate of HPV DNA in invasive vulval SCC is 20-60%. 3, 4,21 HPV is most strongly linked with tumours in younger women, with an 11-fold risk increase reported for vulval intraepithelial neoplasia (VIN) and early-stage cancer in women under the age of 45 with serological evidence of HPV infection, but no increase in women over this age. 5

Four studies have shown that women with a previous cervical cancer or cervical intraepithelial neoplasia grades 3-5 have up to a ten-fold increased risk for vulval cancer. 6, 7,22, 23 However, one study showed no association of a previous diagnosis of invasive or pre-invasive cervical cancer with risk of invasive vulval SCC, after taking into account other risk factors, including sexual behaviour. 24

Increased risks have also been reported for women diagnosed with cancers at other anogenital sites, 8 or with a family history of anogenital cancer or upper aerodigestive SCC. 9,25

Genital warts (associated with infection with HPV 6 and HPV 11) increase risk for vulval cancer. 9-14 Increased risk has also been reported for individuals with a prior SCC of the skin, which may be related to HPV infection. 15

Vulva cancer risk and other sexually transmitted infections

Presence of antibodies to the herpes simplex virus type 2 in blood samples has been associated with an increased risk of vulval cancer and pre-cancer, after HPV infection is controlled for. 3,9,13,16

There is an increased risk for vulval cancer and pre-cancers in HIV-positive women, with a particularly strong relationship for women under the age of 30. 17, 18

Vulva cancer risk and other medical conditions

One study has shown a more than three-fold increased risk of vulval and vaginal cancer in women with systemic lupus erythematosus. 26

Vulva cancer risk and smoking

There is a well-documented association between smoking and risk of vulval SCC, with reported six-fold and three-fold risk increases in current smokers for in situ and invasive tumours, respectively. 3,19

Risk increases with greater intensity and number of years of smoking and remains elevated more than five years after cessation. 3,19 There is a likely interaction between cigarette smoking and HPV infection in risk of vulval cancer, with a 25-fold risk increase reported for women smoking 20 or more cigarettes a day who also have serological evidence of HPV 16 infection. 3

Iatrogenic immunosuppression and vulva cancer risk

Incidence of vulval cancer has been shown to be increased 100-fold following renal transplants. 20

Other factors and vulva cancer risk

One study of people hospitalised for psoriasis in Sweden reported that risk of vulval cancer was increased more than three-fold in this group. 27

Vulva Cancer molecular biology and genetics

There are only a few reports describing the genetic changes associated with vulval cancer. Gene copy number is thought to be important, with gain of gene copies more commonly reported than loss of gene copies.

One study analysed 51 samples from vulval and vaginal tumours and found genetic abnormalities in 37 of them. The most common changes were gains to chromosomes 3q, 5p, 8q, 9q, and 19q and losses from 11q. 1

In one study of vulval cancer 11 out of 12 samples had alterations in the thymosin beta 10 gene (TMBs10), whose protein product inhibits protein polymerisation. 2

Another study of vulval squamous cell carcinoma (SCC) and lichen sclerosus and vulva intraepithelial neoplasia (VIN) found mutations in the TP53 tumour suppressor gene, and TP53 overexpression in more than 70% of SCC and about a quarter of pre-cancerous lesions. 3

Further studies will help to define the genes that are involved in vulval carcinogenesis, and this will help with diagnosis, prognosis and treatment of this cancer.

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Vulval cancer symptoms and treatment

This page presents information on the symptoms, treatment and staging of vaginal cancer. It also includes information on screening and prevention.

Vulval cancer symptoms

The majority of patients diagnosed with vulval cancer present with an enlarging lump or ulcer on the vulva. Many patients have a long history of vulval soreness and irritation and may have had previous biopsies showing vulval intraepithelial neoplasia (VIN) or lichen sclerosus. 1

Staging of Vulval cancer

After biopsy has confirmed the presence of vulval cancer, staging is carried out with assistance of computed tomography (CT) scanning or magnetic resonance imaging (MRI). According to international data for women treated in 1996-98, 11% of vulval cancer patients present with in situ tumours, 56% with stage I or II tumours, 18% with stage III, and 7% with stage IV. 2

Older patients are more likely to present with advanced vulval cancer. In a recent British series, 67% of patients aged over 60 presented with vulval cancer stage III or IV, whereas 52% of patients under 60 had tumours localised to the vulva. 3

Table 6.1 shows the FIGO staging guidelines for vulval cancer.

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Vulval cancer treatment

While the mainstay of treatment for vulval cancer is surgical excision, the current emphasis for management of VIN is on conservative treatments, such as wide local excision or hemi-vulvectomy, rather than total vulvectomy; laser ablation is also employed. 4

An early-stage UK trial has shown the possible benefits of an HPV vaccine in women with VIN, 5 and other experimental treatments include an anti-viral cream to treat HPV 6, 7 and photodynamic therapy. 8 Regular follow-up is important as recurrences are relatively common. 4,9, 10

Early vulval cancer is treated with surgery. 11 With advanced or multifocal disease a total vulvectomy is indicated, but in many cases, particularly where the tumour is well lateralised, a wide local excision of the primary tumour or a hemi-vulvectomy and unilateral lymph node dissection gives excellent local control. 12-14

Depending on the position of the primary tumour, lymph nodes may be removed on both sides. The depth of excision and the width of the excision margins are important prognostic factors for local control. 15 Sentinal node biopsy may help to identify those patients at low risk of inguinal lymph node involvement, who are unlikely to benefit from lymphadenectomy. 16, 17

Local radiotherapy is used as an adjuvant to surgery for deeper lesions, where adequate clear margins are not obtained or where nodes are involved. 18, 19

Radical radiotherapy is used in patients for whom surgery is not an option and may be combined with chemotherapy. 20

Cure of advanced vulval cancer requires combined modality treatment using radiotherapy with concomitant chemotherapy and radical surgery. A small trial of pre-operative chemoradiotherapy and radical surgery in patients presenting with grossly enlarged or fixed inguinal nodes showed that local control in the nodes was achieved for 36/37 patients and at the primary site for 29/38. 21

Another group of women in this trial who presented with locally advanced primary tumours, which would have required exenteration (operation in which all the contents of a body cavity are removed) for control, were treated under the same regimen and results showed that a good level of local control was achieved while mainly avoiding exenterative surgery. 22

Combined modality treatment is associated with very high acute morbidity and should only be considered in the very fittest patients. Where elderly and frail patients present with very advanced and inoperable local disease, referral to palliative care specialists may be more appropriate. 23

Vulval cancer screening and prevention

Regular surveillance for early vulval cancer or pre-cancer is recommended for women with a previous diagnosis of anogenital cancer or VIN, 4 and the International Society for the Study of Vulvovaginal Disease recommends monthly self-examination for all women. 24

Preliminary trials of immunotherapies have been promising for VIN, although numbers of trial participants have been small and larger-scale trials are needed to confirm results. 5,25, 26

If smoking rates and levels of HPV infection remain at current levels, we may see a continuation of the increasing trend for vulval cancer, observed since the mid-1990s. In this case, vulval cancer will affect greater numbers of pre-menopausal women. Given that we know the role played by both smoking and HPV in the causation of vulval cancer, there is strong potential to reduce incidence, especially by tackling smoking rates.