Describe the risk factors, clinical features, spread, staging and management of carcinoma Vulva.

Groin node status is the key prognostic factor.

Definition

Carcinoma of the vulva is a malignant tumour of the vulva, the great majority being squamous-cell carcinomas. It is predominantly a disease of elderly post-menopausal women and most often arises on the labium majus.

Carcinoma Vulva - Spread

Risk Factors

Vulval intraepithelial neoplasia (vin) and HPV infection (in younger women), lichen sclerosus and chronic vulval dystrophy (in older women), smoking, immunosuppression, chronic irritation, and a history of cervical neoplasia.

Premalignant Lesion

Vulval intraepithelial neoplasia (vin) — atypical cells confined to the epithelium — is the precursor, analogous to CIN of the cervix.

Clinical Features

Long-standing vulval pruritus, a lump, ulcer or warty growth, bleeding, discharge, pain and occasionally a groin swelling (nodal disease). The lesion is often neglected, so women present late.

Spread

Direct — to the adjacent vagina, urethra and anus; lymphatic (the main route) — to the inguinal, then femoral, and finally pelvic nodes; blood-borne spread is late.

Investigations

Diagnosis is confirmed by a wedge biopsy of the lesion (with vulvoscopy of the remaining vulva). Spread is assessed by palpation of the groin nodes, cystoscopy/proctoscopy for local extension, and cross-sectional imaging (CT/MRI/pet) of the groin and pelvis; a cervical smear is taken in view of the HPV field association.

Figo Staging

• Stage I — tumour confined to the vulva/perineum (IA ≤ 2 cm with stromal invasion ≤ 1 mm).
• Stage II — extension to adjacent structures (lower urethra, vagina, anus) with negative nodes.
• Stage III — spread to the inguino-femoral lymph nodes.
• Stage IV — invasion of upper urethra/bladder/rectum/bone or distant metastasis.

Management

• Early disease — wide local excision with assessment of the groin nodes (sentinel-node biopsy or inguino-femoral lymphadenectomy).
• Operable invasive disease — radical vulvectomy with bilateral inguino-femoral lymphadenectomy.
• Advanced/inoperable disease — radiotherapy or chemoradiation, with surgery where feasible.
• Treat vin to prevent progression, and counsel on smoking cessation and follow-up.

WHY Vulval Cancer Is Often Diagnosed Late, Despite Being Visible

A striking paradox worth understanding is why carcinoma of the vulva — a cancer on the body's surface, which the woman can see and feel — is so often advanced at diagnosis. The tumour is not hidden; the delay is human. It occurs typically in elderly women, who may be embarrassed to seek help for a genital complaint, may attribute the symptoms to 'old age', or may have limited access to care. Its principal symptom, long-standing pruritus vulvae, is extremely common and is very frequently treated — by the woman herself, by pharmacists, and by doctors — with topical steroids or antifungals for months or years without anyone ever looking properly, or taking a biopsy. Meanwhile the underlying lesion (often arising in a background of lichen sclerosus or vin) progresses to an ulcer or a growth. Hence the cardinal rule: any persistent vulval itching, ulcer, lump or white patch in an older woman must be biopsied, not treated blindly — no vulval lesion should be treated for more than a few weeks without a histological diagnosis. Understanding that the disease is missed not because it is invisible but because it is not looked at explains why the biopsy is the whole key.

WHY the Groin Nodes Dominate the Treatment

Understanding why the surgical treatment of vulval cancer is so extensive, and centred on the groins, follows from its pattern of spread. The vulva has a rich, predictable lymphatic drainage, which passes first to the superficial inguinal nodes, then to the deep inguinal (including Cloquet's node) and thence to the pelvic nodes — and, importantly, the lymphatics of the midline structures (clitoris, perineum) cross, so drainage may be bilateral. Because lymph-node status is by far the most important prognostic factor — survival falls dramatically once the groin nodes are involved — the operation must address them. This is why the classical treatment is a radical vulvectomy with bilateral inguinofemoral lymphadenectomy, and why leaving the nodes untreated is unacceptable except in the earliest lesions. But this surgery is mutilating and carries heavy morbidity — wound breakdown, infection, and lifelong lymphoedema of the legs. Hence the modern trend towards individualisation: a wide local excision with ipsilateral (or sentinel) node assessment for small, unilateral, early tumours, sparing many women the full operation. Understanding that the groin nodes determine both prognosis and the extent of surgery explains the whole surgical strategy.

• Carcinoma vulva: usually squamous cell carcinoma in elderly women; arises from vin or lichen sclerosus (and HPV in younger women).
• Presents with long-standing pruritus vulvae, then an ulcer, lump or bleeding — often treated blindly for months, hence late diagnosis.
• Golden rule: biopsy any persistent vulval ulcer, lump, white patch or itch in an older woman — never treat blindly.
• Spreads to the inguinal nodes (superficial → deep → pelvic; midline lesions drain bilaterally) — node status is the key prognostic factor.
• Treatment: radical vulvectomy with bilateral inguinofemoral lymphadenectomy; wide local excision with sentinel-node biopsy for early unilateral lesions; radiotherapy for advanced disease.