[1] Risk factors include vulvar intraepithelial neoplasia (VIN), HPV infection, genital warts, smoking, and many sexual partners.

Chronic inflammatory conditions of the vulva that may be precursors to vulvar cancer include lichen sclerosus, which can predispose to differentiated VIN.

[17][18] Risk factors for vulvar cancer are largely related to the causal pathways above, involving exposure or infection with the HPV virus and/or acquired or innate auto-immunity.

Additional evaluation may include a chest X-ray, an intravenous pyelogram, cystoscopy or proctoscopy, as well as blood counts and metabolic assessment.

Depending on the cellular origin, different histologic cancer subtypes may arise in vulvar structures.

The lymphatics of the labia drain to the upper vulva and mons pubis, then to both superficial and deep inguinal and femoral lymph nodes.

A verrucous carcinoma of the vulva is a rare subtype of squamous cell cancer and tends to appear as a slowly growing wart.

Verrucous vulvar cancers tend to have a good overall prognosis, as these lesions hardly ever spread to regional lymph nodes or metastasize.

Other forms of vulvar cancer include invasive Extramammary Paget's disease, adenocarcinoma (of the Bartholin glands, for example) and sarcoma.

FIGO's revised TNM classification system uses tumor size (T), lymph node involvement (N) and presence or absence of metastasis (M) as criteria for staging.

Stage III cancers include greater disease extension to neighboring tissues and inguinal lymph nodes on one side.

Stage IV indicates metastatic disease to inguinal nodes on both sides or distant metastases.

[31] Other cancerous lesions in the differential diagnosis include Paget's disease of the vulva and vulvar intraepithelial neoplasia (VIN).

A number of diseases cause infectious lesions including herpes genitalis, human papillomavirus, syphilis, chancroid, granuloma inguinale, and lymphogranuloma venereum.

Surgery is a mainstay of therapy depending on anatomical staging and is usually reserved for cancers that have not spread beyond the vulva.

[31] Surgery may involve a wide local excision (excision of the tumor with a safety-margin of healthy tissue, that ensures complete removal of the tumor), radical partial vulvectomy, or radical complete vulvectomy with removal of vulvar tissue, inguinal and femoral lymph nodes.

[22][26] In cases of early vulvar cancer, the surgery may be less extensive and consist of wide excision or a simple vulvectomy.

Surgery is significantly more extensive when the cancer has spread to nearby organs such as the urethra, vagina, or rectum.

Complications of surgery include wound infection, sexual dysfunction, edema and thrombosis, as well as lymphedema secondary to dissected lymph nodes.

[33] Radiation therapy may be used in more advanced vulvar cancer cases when disease has spread to the lymph nodes and/or pelvis.

[37] Women with vulvar cancer should have routine follow-up and exams with their oncologist, often every three months for the first 2–3 years after treatment.

[38] Imaging without these indications is discouraged because it is unlikely to detect a recurrence or improve survival and is associated with its own side effects and financial costs.

Five-year survival is greater than 90% for patients with stage I lesions but decreases to 20% when pelvic lymph nodes are involved.

This is most common in older women, due to the increased risk for developing cellular atypia which in turn leads to cancer.