Vulvar cancer

Classification

Lymphatic Drainage

• Drainage pathways: The lymphatic drainage of the vulva proceeds first to the superficial and deep inguinofemoral lymph nodes, then onwards to the pelvic nodes.
• Lateral tumours: For laterally located tumours with a distance of at least 1 cm from the midline, ipsilateral assessment is sufficient.

Staging

• FIGO I: Stage FIGO I means a tumour confined to the vulva or perineum without lymph node metastases.
• FIGO IA: Stage FIGO IA is defined as a tumour up to 2 cm with a depth of invasion up to 1 mm.
• FIGO II: Stage FIGO II means spread to the lower parts of the urethra, vagina, or anus without lymph node metastases.
• FIGO III: Stage FIGO III means lymph node metastases in the inguinofemoral lymph nodes.
• FIGO IV: Stage FIGO IV means involvement of upper urethral or vaginal parts, bladder or rectal mucosa, pelvic bone, fixed or ulcerated inguinal lymph nodes, or distant metastases.

Occurrence & epidemiology

Epidemiology and Aetiology

• Histology: The most common histological type of vulvar carcinoma is squamous cell carcinoma.
• Pathways of development: Two main pathways of development are distinguished, namely the HPV-dependent pathway via HSIL in younger women and the HPV-independent pathway via lichen sclerosus and dVIN in older women.
• Incidence: The incidence of vulvar carcinoma has increased in recent decades, including in younger women.

Aetiopathogenesis

Risk Factors

• HPV-associated risks: HPV-associated risk factors include persistent infection with high-risk types, smoking, immunosuppression, and concurrent cervical neoplasia.
• HPV-independent risk: The most important HPV-independent risk factor is lichen sclerosus.

Clinical features

Clinical Presentation and Predilection Sites

• Further symptoms: Other complaints include a non-healing sore, bleeding, burning, pain, and a palpable lump.
• Predilection sites: Typical predilection sites are the labia majora, followed by the labia minora, clitoral region, and posterior commissure.

Histology

Other Histologies

• Rare entities: Other rare entities are basal cell carcinoma, adenocarcinoma of the Bartholin's gland, and sarcoma.

Diagnosis

Diagnosis and Staging

• Diagnostic confirmation: The diagnosis is confirmed by a punch biopsy from the edge of the lesion.
• Histopathology: Information from the biopsy regarding histological type, grading, depth of invasion, and the HPV or p16 and p53 status is crucial.
• Clinical examination: The clinical examination includes the entire vulva with vulvoscopy, vagina, and cervix, as well as the inguinal lymph nodes.
• Staging investigations: Staging includes ultrasound of the groins, and in advanced cases, cross-sectional imaging of the pelvis and abdomen, cystoscopy and rectoscopy as needed.

Typical imaging findings

• Clinical finding: A rounded, firm, exophytic nodule with an irregular, partly keratotic and eroded surface lies on the labium. The margin is raised and the lesion is barely mobile against the underlying tissue; the surrounding skin appears atrophic.
• Clinical finding: A firm, yellowish-brown horn-like structure protrudes from a reddened, thickened base. The base itself is raised and indurated; the surrounding skin is pale, atrophic and shows signs of a chronic underlying disease.

Keep learning in the app

Further reading (selection)

1. StatPearls: Vulvar Cancer (NCBI Bookshelf)
2. DocCheck Flexikon, Vulvakarzinom

Cross-references