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Vulval cancer - Dr Deac Pimp

Definition

  • A carcinoma affecting the external genital organs: the vulva

 

Risk factors

  • Age – majority is seen in older women
  • HPV
  • Lichen sclerosus – has 4% chance of developing into malignancy
  • Paget’s disease – 15% will also have vulval carcinoma
  • VIN (vulval-intraepithelial neoplasia

 

Differential diagnoses

  • Lichen sclerosus
  • Lichen planus
  • Bartholin’s cyst/abscess
  • Paget’s disease
  • STI:
    • Genital warts
    • Genital herpes
    • Syphilis
  • Fungal infection

 

Epidemiology

  • Rare cancer, 20th most common cancer in women
  • Accounts for less than 1% of all cancers
  • Incidence is 3.7 in 100,000 women
  • Generally considered a disease of older women, although young women infected with HPV are accounting for more cases
    • Half of cases over 75, over 75% are over 60
    • Incidence of women in their 40s has doubled, probably as result of HPV

 

Aetiology

  • HPV is increasingly implicated in vulval cancer, especially in younger women
  • VIN is a pre-malignanct state with a risk of developing into carcinoma
    • ‘Usual type’ = HPV dependent (16 + 18) – usually younger women, 2/3 have CIN, appears as warty or plaque like change on the vulva
    • HPV independent – usually older women with precipitating vulval dermatological disorders
  • Melanomas account for a small proportion of vulval cancers, and typically present as an altered/abnormal mole or skin discolouration

 

Clinical features

  • Vulval lump
    • Have high suspicion in new cases of ‘warts’ in women over 50
  • Itching (pruritis) or burning
  • Vulval ulcer
  • Dysuria
  • Thickened/abnormal skin
  • Bleeding/blood-stained discharge
  • 50% of cases occur on the labia majora, 20% on labia minora
  • May also occur on clitoris and bartholin’s glands
  • Most common spread is into vagina and regional lymph nodes

 

Pathophysiology

  • 80% are squamous cell carcinomas

 

Investigations

  • Genital examination, including internal examination to check for spread
  • 2-week referral if:
    • Unexplained vulval lump
    • Unexplained vulval ulceration or bleeding
    • Persistent vulval pain or pruritis (a period of watch and wait is appropriate initially, unless other reasons to suspect malignancy)
  • In small regions, less than 20mm, wide local excision is recommended to send for histology
  • Needle biopsy of larger areas
  • CT and/or MRI for staging and investigation of invasion.
  • Sentinel node biopsy in early stage disease, helps to prevent uneccesary node dissection
  • Staging using FIGO:
    • Stage 1 = confined to vulva
    • Stage 2 = spread to adjacent perineal structures
    • Stage 3 = spread to femoro-inguinal lymph nodes
    • Stage 4 = distant spread (metastasis) including upper vagina and upper urethra

 

Management

  • Surgery:
    • Is standard treatment
    • Wide/radical resection of area
    • Radical vulvectomy if multifocal disease, or other suspicious lesions
      • Butterfly incision
    • Lymph node dissection if suspicion or confirmation of involvement
    • These surgical options can be very distressing, as result in varying degrees of mutilation and sexual difficulties
    • Reconstructive surgery is often required
  • Radiotherapy and chemotherapy:
    • These are often used as adjuvants, especially in advanced disease, for curative treatment and palliation

 

Prognosis

  • Lymph node metastasis is most important prognostic tool
  • 5 year survival if no lymph node involvement (Stage 1 and 2) is >80%
  • 5 year survival with inguinal lymph node involvement (stage 3) is 50%
  • 5 year survival with iliac or pelvic lymph node involvement is 10-15%
  • Recurrence rate is 37%
Vulval cancer.docx
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