Vulvar and Vaginal Cancer

Vulvar and vaginal cancers are uncommon gynaecological malignancies, predominantly of squamous cell histology, arising from the vulvar or vaginal epithelium.

Two distinct aetiological pathways are recognised for vulvar cancer. The first is human papillomavirus-associated, typically affecting younger women. It arises in the context of vulvar intraepithelial neoplasia and shares epidemiological features with cervical cancer.

The second is human papillomavirus-independent, typically affecting older women. It arises in the context of chronic vulvar dermatological conditions, particularly lichen sclerosus.

Vaginal cancer is rarer still. It frequently needs careful distinction from direct vaginal extension of a primary cervical or vulvar malignancy, given the anatomical proximity.

Pathophysiology and risk factors

• Human papillomavirus-associated vulvar cancer arises through a progressive dysplastic pathway analogous to that of cervical cancer. Usual-type vulvar intraepithelial neoplasia is the recognised precursor lesion.
• It shares risk factors with cervical and other human papillomavirus-associated anogenital malignancies, including smoking and immunosuppression.
• Human papillomavirus-independent vulvar cancer arises in chronic vulvar dermatological conditions, most notably lichen sclerosus. This chronic inflammatory condition carries a modest overall lifetime risk of malignant transformation.
• Differentiated-type vulvar intraepithelial neoplasia is the precursor lesion in this pathway. It typically affects older women and has a somewhat different, generally more aggressive natural history than the human papillomavirus-associated pathway.
• Primary vaginal cancer is rare. Most malignancy found in the vagina is direct extension or metastatic spread from an adjacent primary site, most commonly cervical or vulvar cancer.
• Full assessment to exclude these more common explanations is essential before a primary vaginal cancer diagnosis is confirmed.

• Vulvar cancer typically presents with a persistent vulvar lump, ulcer or area of skin change (including thickening, colour change or a non-healing lesion). Pruritus, pain or bleeding may accompany it.
• Symptoms are frequently present for a considerable period before diagnosis. Patients may be reluctant to present with genital symptoms, and symptoms may initially be attributed to a more common, benign dermatological or infective cause.
• A background of chronic vulvar symptoms, including longstanding pruritus or a known diagnosis of lichen sclerosus or vulvar intraepithelial neoplasia, raises malignancy risk.
• Any new, persistent or changing lesion in this background should prompt prompt further evaluation.
• Vaginal cancer presents with abnormal vaginal bleeding (including postcoital or postmenopausal bleeding) or discharge. A vaginal mass may be palpable in some cases.
• Symptoms frequently overlap substantially with those of cervical cancer, given the anatomical proximity and shared risk factors.
• Any persistent, unexplained vulvar or vaginal symptom warrants biopsy of any identified lesion to exclude malignancy. This applies particularly with background risk factors or failure to respond to an appropriate trial of treatment for a more common, benign diagnosis.
• Do not continue extended empirical treatment without histological confirmation.

• The essential diagnostic approach for both conditions is careful vulvar and vaginal examination, with colposcopic assessment where indicated, and biopsy of any suspicious lesion. Biopsy gives definitive histological diagnosis and characterisation.
• Staging assessment informs treatment planning. It includes magnetic resonance imaging of the pelvis and, where relevant to regional or distant spread, further cross-sectional imaging.
• It also includes assessment of regional lymph nodes. For vulvar cancer these are the inguinofemoral nodes, given the characteristic regional lymphatic drainage from this site.
• For suspected primary vaginal cancer, careful clinical and radiological assessment must exclude direct extension or metastatic spread from an adjacent primary site (cervical or vulvar) as the explanation for an apparent vaginal malignancy.
• This is essential to accurate diagnostic classification.

• Vulvar cancer management is individualised by tumour size, depth of invasion and regional lymph node involvement.
• Surgery is the standard primary treatment for the great majority of cases. It is wide local excision or, for more extensive disease, radical vulvectomy. Inguinofemoral lymphadenectomy or sentinel lymph node biopsy is chosen according to tumour characteristics and depth of invasion.
• Radiotherapy, with or without concurrent chemotherapy, is adjuvant treatment for higher-risk disease, including significant nodal involvement or close surgical margins. It is primary treatment for more locally advanced disease not amenable to resection.
• Radiotherapy is also the standard approach for confirmed regional nodal involvement.
• Vaginal cancer management similarly uses surgery, radiotherapy or both, individualised by tumour stage, location and extent. Radiotherapy includes brachytherapy, as the site is anatomically accessible.
• Treatment frequently parallels that of cervical cancer, given the anatomical and biological similarity.
• Manage longer-term treatment consequences as an integral part of long-term care. These include sexual and psychological impact, given the sensitive, intimate location of both conditions, and lymphoedema after inguinofemoral lymphadenectomy where performed.
• Structured long-term follow-up continues surveillance and management of any background dermatological condition, including lichen sclerosus in the human papillomavirus-independent vulvar cancer pathway.
• Care is delivered through specialist gynaecological oncology services, given the rarity of these cancers and the specialised expertise required.