Vulvar malignancies: an interdisciplinary perspective

Overview

This CME review, published in the Journal der Deutschen Dermatologischen Gesellschaft in 2019, combines gynaecologic oncology and dermatology perspectives. Vulvar cancer is the fourth most common gynaecologic malignancy. Most cases occur later in life, but every subtype has been described in young women, and diagnosis is often delayed. Because these tumours are rare, the authors recommend referral to dedicated centres and interdisciplinary care. Histology is required for any suspicious lesion. Precise mapping of biopsy sites guides the surgical approach.

Squamous cell carcinoma

Squamous cell carcinoma is the most common type, and its incidence is rising. It develops through two pathways. One is HPV-related usual-type vulvar intraepithelial neoplasia. The other is HPV-independent differentiated neoplasia, which is associated with lichen sclerosus and lichen planus. Surgery is the primary treatment for early disease. Local excision appears to give survival comparable to radical vulvectomy. Sentinel node biopsy spares selected patients from full groin dissection. Locally advanced disease is usually treated with chemoradiation. Lymph node status is the key prognostic factor, and recurrences are common, so close follow-up is needed.

Other tumour types

Verrucous carcinoma is now considered a separate entity. It grows locally with little risk of nodal spread. Coexisting squamous carcinoma must be excluded with adequate biopsies, and local excision without routine node dissection is standard. Vulvar melanoma differs biologically from skin melanoma and has a worse prognosis. It is staged with the melanoma system rather than the gynaecologic one. It is treated with local excision, because more radical surgery has shown no benefit. Immunotherapy and targeted therapy are evolving, with lower responses than in skin melanoma.

Extramammary Paget disease mimics common skin conditions, which delays diagnosis. It has high rates of involved margins and recurrence, and it requires work-up to exclude an underlying cancer. Basal cell carcinoma has a good prognosis after surgery. Sarcomas and Bartholin gland carcinomas are rare and best managed in specialised centres.

Clinical implications

Across all types, treatment aims for disease control while preserving continence and sexual function wherever possible.