The vulva is affected by lack of oestrogen after the menopause. Vulvar pruritis and irritation are common symptoms in a postmenopausal woman. Examination of the vulva should exclude finding ulceration or a mass that may accompany these, as they may also be indicative of infection, inflammation, or malignancy.
When a patient presents with a vulval ulcer the following need to be excluded with culture and/or biopsy with colposcopy:
Sexually transmitted infection:
- Herpes simplex.
- Granuloma inguinale.
- Lymphogranuloma venereum.
- Syphilis (NB: this should not be forgotten as the number of cases in the UK is increasing).
- Invasive disease of the vulva.
- Vulval intraepithelial neoplasia (VIN).
- Paget's disease of the vulva.
Other ulcerative conditions:
- Behçet's disease.
- Disseminated lupus erythematosus.
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- Invasive disease of the vulva
- Benign tumours:
Premalignant disease of the vulva
Vulval intraepithelial neoplasia (VIN)
- Nature: this is considered a premalignant state. It can occur by means of cell transformation in already existing vulvar disorders such as lichen sclerosus and squamous cell hyperplasia or it can occur independently.
- Presentation: most patients have pruritus, but some are asymptomatic. The lesions may be white, grey, red or raised.
- Management: biopsy is performed before laser therapy, to make sure that a lesion does not contain invasive cancer. Conventional treatment is wide local excision or laser ablation. Because of the close association of VIN with human papillomavirus infection, lifelong follow-up is required to watch for recurrence.
- Prognosis: spontaneous regression of the disease at all stages has been reported.
Malignant disease of the vulva
Paget's disease of the vulva
- A patient with Paget's disease of the vulva (adenocarcinoma in situ) may present with pruritus and weeping or bleeding of the lesion.
- The lesion appears to have an eczematous or velvet-like surface.
- Wide local excision is recommended, because the margins of Paget's disease often extend beyond what is seen on gross examination.
- This explains the high incidence of recurrence.
- Because of the 20% incidence of underlying adenocarcinoma, the dermis should be removed for accurate diagnosis.
- Thorough evaluation of the cervix, colon, bladder, gallbladder and breasts is necessary when Paget's disease of the vulva is found; there is a 30% incidence of concomitant primary carcinoma in these locations.
Vulval cancer is a very rare disease and, on average, a general practitioner will only see a new case once every 7 years. There should be a high index of suspicion for abnormal lesions on vulva, including 'warts' in the postmenopausal woman.
- Vulval cancer accounts for approximately 5% of all female genital malignancies.
- It occurs in about 1.5 per 100,000 women-years in developed countries but is 2-3 times more frequent in underdeveloped countries.
- With the exception of the rare sarcomas, this cancer appears most frequently in women aged 65-75 years.
- 85% are squamous, less than 5% are melanoma, the second most common type.
- Squamous carcinomas can arise on a background of atrophic changes such as lichen sclerosus, or in hypertrophic epithelium.
- Squamous vulval cancer can have many different growth characteristics.
- It can occur in an area of epithelial neoplasia that develops into a small nodule, which may break down and ulcerate.
- Small, warty or cauliflower-like growths may be seen and confused with condylomata acuminata.
- 75% of all growths are primarily on the labia.
- Long-term pruritus, lumps or masses on the vulva are present in most patients with invasive vulval cancer.
- Staging recommendations are from the International Federation of Gynecology and Obstetrics (FIGO).
- Most are treated with radical vulvectomy, but in advanced stage primary vulval cancer, treatment is tailored to individual patient needs. Combined treatments have been developed, using chemotherapy, radiotherapy and surgery.
- Melanoma should be considered if there are pigmented lesions on the vulva.
- They are suspicious if they are blue-black in color, have a jagged or fuzzy border, are raised or ulcerated, or are larger than approximately 1 cm.
- Melanomas may be misdiagnosed as undifferentiated squamous carcinoma, particularly if they are amelanotic.
- Most melanomas are located on the labia minora or clitoris and prognosis is related to the size of the lesion and the depth of invasion.
Non-neoplastic skin conditions
- Psoriasis is a multifocal disease that may affect vulvar tissue as well as skin of the joints, knees and scalp.
- Seborrhoeic dermatitis, another multifocal disease of the sebaceous glands and commonly affecting the scalp, may affect the labia majora only.
- Tinea cruris begins as raised, sharply demarcated, red lesions on the thighs and can spread to the labia.
- These lesions are best diagnosed by punch biopsy with local anaesthetic.
- When a woman presents with vulval symptoms, a vulval examination should be offered.
- If an unexplained vulval lump is found, an urgent referral should be made.
- Vulval cancer can also present with vulval bleeding due to ulceration. A patient with these features should be referred urgently.
- A patient who presents with pruritus or pain may be reasonably managed with a period of 'treat, watch and wait'.
- This should include active follow-up until symptoms resolve or a diagnosis is confirmed.
- If symptoms persist, the referral may be urgent or non-urgent, depending on the symptoms and the degree of concern about cancer.
- Other paths of referral may include a genitourinary medicine clinic, gynaecology or dermatology, based on appearance and suspicions of the examining GP.
Further reading & references
- Canavan T, Cohen D; Vulvar Cancer. American Family Physician; October 2002.
- Gupta R, Warren T, Wald A; Genital herpes. Lancet. 2007 Dec 22; 370(9605):2127-37.
- Sen P, Barton SE; Genital herpes and its management. BMJ. 2007 May 19;334(7602):1048-52.
- Young F; Syphilis: still with us, so watch out! J Fam Health Care. 2006;16(3):77-81.
- Gynaecological cancer - suspected, Clinical Knowledge Summaries (2005)
- Schecter JC et al; Bartholin Gland Diseases, eMedicine, Dec 2009
- Meffert J, Lichen Sclerosus et Atrophicus, Medscape, Mar 2011
- Jones RW, Rowan DM, Stewart AW; Vulvar intraepithelial neoplasia: aspects of the natural history and outcome in 405 women. Obstet Gynecol. 2005 Dec;106(6):1319-26.
- Barhan S, Ezenagu L; Vulvar Problems in Elderly Women. Postgraduate Medicine On-line (Oct 1997).
- Feuer GA, Shevchuk M, Calanog A; Vulvar Paget's disease: the need to exclude an invasive lesion. Gynecol Oncol. 1990 Jul;38(1):81-9.
- Management of Vulval Cancer, Royal College of Obstetricians and Gynaecologists (2006)
- Creasman WT, Malignant Vulvar Lesions, Medscape, May 2011
- FIGO; Staging Vulval cancer. 2001.
- van Doorn HC, Ansink A, Verhaar-Langereis M, et al; Neoadjuvant chemoradiation for advanced primary vulvar cancer. Cochrane Database Syst Rev. 2006 Jul 19;3:CD003752.
- Referral for suspected cancer, NICE Clinical Guideline (2005)
|Original Author: Dr Hayley Willacy||Current Version: Dr Hayley Willacy|
|Last Checked: 20/12/2010||Document ID: 1560 Version: 25||© EMIS|
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