Vulvar cancer is a malignancy of the outer female genitalia that predominantly occurs in postmenopausal women. Major risk factors are HPV infection, smoking, vulvar dystrophy, and vulvar or cervical intraepithelial neoplasia. Clinical manifestations of vulvar cancer include new lumps or lesions, itching, a burning sensation and, less frequently, vulvar bleeding. Suspicious lesions must be biopsied for histological analysis and to rule out other similar conditions, such as vulvar dermatoses or vulvar intraepithelial neoplasia, which both increase the risk of vulvar cancer. Vulvar cancer is staged based on the depth of the lesion and the involvement of neighboring structures. Surgical resection (radical vulvectomy) is the first-line treatment, but advanced stages may require radiotherapy and/or palliative chemotherapy. Vulvar cancer is usually associated with a poor prognosis.
- Incidence: rare 
- Age 
Epidemiological data refers to the US, unless otherwise specified.
- Infection with HPV 16, 18, 31, and 33 (16 and 33 account for 55% of HPV-related cases of vulvar cancer)
- Vulvar dystrophy and vulvar or cervical intraepithelial neoplasia (VIN/CIN)
- Precancerous lesions (e.g., lichen sclerosus)
Paget disease of the vulva
- Adenocarcinoma; carcinoma in situ
- Low risk (< 15%) of underlying invasive Paget disease/invasive adenocarcinoma (unlike which is always associated with underlying carcinoma)
- Clinical features
- May initially be asymptomatic
- Local pruritus, possibly with burning sensation and pain
- Reddish, blackish, and/or whitish patches of discoloration
- Lumps or growths of various shapes, often wart-like lesions or ulcers
- Vulvar bleeding or discharge (less common)
- Dysuria, dyspareunia
- Lymphadenopathy in the groin area
All suspicious lesions must be biopsied for histological analysis.
- Etiology: unclear
- Epidemiology: postmenopausal women and, less commonly, prepubescent girls
- Parchment-like, thin, shiny vulvar skin
- Narrow, atrophic vaginal introitus resulting in dyspareunia
- Burning pain, pruritus, bleeding vulvar ulcers
- Lichen simplex chronicus is characterized by chronic pruritus, which provokes persistent scratching of the vulva and so causes lichenification of the skin.
- Diagnosis: Colposcopy and biopsy of suspicious lesions are required to rule out malignancy.
Vulvar intraepithelial neoplasia (VIN)
- Definition: precancerous lesion caused by dysplasia of squamous cells
- Classification 
- Diagnosis: tissue biopsy
- Treatment: : depending on severity, excision or ablation may become necessary
- Prognosis: may progress to vulvar carcinoma despite treatment (in < 10% of cases)
The differential diagnoses listed here are not exhaustive.
- First-line treatment; : local excision and surgical resection (radical vulvectomy)
- Radiotherapy and/or palliative chemotherapy: when disease metastasizes to peripheral lymph nodes or other organs
- The average 5-year survival rates range from 30–50%.
- Survival rates vary greatly depending on the stage of the disease.
- Localization: The upper third of the posterior vaginal wall is the most common site of vaginal carcinoma.
- Etiology: same as vulvar neoplasia (e.g., HPV 16 and 18)
- Squamous cell carcinoma
- Clear cell adenocarcinoma: seen in daughters of women who received diethylstilbestrol during pregnancy
Sarcoma botryoides 
- Rare, highly malignant embryonal rhabdomyosarcoma that arises most commonly, but not exclusively in the genitourinary system
- Epidemiology: peak incidence in childhood (< 4 years) 
- Vaginal bleeding
- Leukoplakia, vaginal ulceration with contact bleeding
- Malodorous discharge
- Possibly urinary frequency
- Pelvic exam
- Colposcopy: if abnormal cytology results without a clearly visible lesion during pelvic exam
- Biopsy of mass to determine histopathology