B-QuiCK: Vulval cancer

B-QuiCK: Vulval cancer – early detection and referral

Early detection and prevention

• Recommend prophylactic HPV vaccination (funded for females and males aged 9 – 26 years inclusive)
  • Vaccination is most effective when administered prior to the onset of sexual activity but vaccinating those who have already commenced sexual activity is still recommended
  • The vaccine can be administered (not funded) to people aged 27 years and older if they have not been vaccinated before and are likely to benefit, e.g. people who are newly sexually active, people with higher risk sexual activity
• Diagnose and effectively manage lichen sclerosus (with topical corticosteroids) to reduce the risk of progression to differentiated VIN and invasive squamous cell vulval cancer
• There are no screening programmes for the early detection of vulval cancer, so diagnosis relies on investigating reported vulval lesions or symptoms, opportunistic detection of vulval abnormalities, e.g. during cervical screening and following up patients who are at increased risk (e.g. those with lichen sclerosus)
  • Symptoms and signs of vulval cancer may include vulval pruritus, pain, irritation, bleeding, discharge or the presence of a vulval lesion or mass

Patients with suspicion of vulval cancer

• Take a focused history, considering relevant risk factors (e.g. lichen sclerosus)
• Perform a pelvic examination (including speculum or bimanual examination as indicated)
  • Palpate the groin to assess for any enlarged lymph nodes
  • Offer cervical screening to patients who are due
  • Swabs for sexually transmitted infections, e.g. genital herpes, are not usually indicated if a tumour is present, but may be useful if there is ulceration, bleeding or discharge to exclude other potential causes
• Examine the vulval region, paying close attention to the labia majora as this is the most common site of vulval cancer
  • Vulval cancers (and precursor lesions) can vary widely in appearance and may be multifocal or involve a single lesion (see Table 1 for specific features). In general, look for:
    • Changes to the surface of the skin, i.e. irregular epithelial structures
    • Swelling
    • Changes in colour, e.g. red, white or other pigmented areas
    • A visible lesion, e.g. flat, raised, lump (or apparent genital warts in a post-menopausal patient)
    • Ulceration or bleeding
• Refer patients with abnormal examination findings, e.g. suspicious vulval lesion, to a gynaecologist for further assessment which is likely to include vulvoscopy and/or biopsy. Urgently refer patients with a fungating mass or palpable inguinal nodes as these indicate more advanced cancer.

Manage according to vulval biopsy results

• Vulval cancer: the patient will usually be managed in a Gynaecological Oncology centre
• Lichen sclerosus: manage with topical corticosteroids (click here). Place a recall for annual review, and advise patients to return for examination if there are any changes in symptoms or to the feel/appearance of the lesion arise within the year. Consider referral for an additional biopsy if there is inadequate response to treatment. Long-term or indefinite follow-up is required.
• Paget disease of the vulva: investigate for co-existing malignancies, e.g. at the rectum, bladder, urethra, cervix. Patients will usually require treatment, e.g. surgery, imiquimod cream (unapproved indication), to reduce the risk of progression to invasive adenocarcinoma. Long-term follow-up is recommended.
• Low-grade squamous intraepithelial lesion (LSIL): consider periodic follow-up until the lesion resolves
• Differentiated VIN: the patient will usually undergo surgical resection in secondary care. Treat any underlying lichen sclerosus with ultrapotent topical corticosteroids to reduce the risk of patients developing recurrence of differentiated VIN and subsequent vulval squamous cell carcinoma.
  • European guidelines recommend ongoing follow-up at least every six months, depending on the severity of any associated lichen sclerosus
• HSIL: the patient will usually undergo surgical resection in secondary care. Topical application of imiquimod cream (unapproved indication) is a non-surgical treatment option.
  • Vulval HSIL often co-exists with other HPV-dependent cancers or pre-cancerous lesions. As the incidence of anal cancer is higher in people with a history of pre-cancerous vulval lesions or vulval cancer, consider investigations for anal cancer, e.g. digital anorectal examination, anal cytology, as indicated.
  • There are no New Zealand-specific cervical screening recommendations for people treated for vulval HSIL; international guidelines recommend annual cervical screening. In practice, decisions about cervical screening for people with a history of vulval HSIL will be made on a case-by-case basis in conjunction with a gynaecologist.