Snapshot A 62-year-old woman presents with vaginal itching. She denies any trauma, any abnormal odors, or vaginal discharges. She noted a lump on her vulva. She has a history of cigarette smoking and has not quit. Pelvic examination is notable for ulcerative lesion in the left labia majora. A biopsy is performed, which is positive for squamous cell carcinoma. Introduction Overview cancer affecting the vulva squamous cell carcinoma is the most common histologic type (~75% of cases) other histologic types include melanoma basal cell carcinoma Bartholin gland adenocarcinoma sarcoma Paget disease most common cause is human papilloma virus (HPV) infection Epidemiology Incidence most frequently occurs between 65-75 years of age fourth most common gynecologic malignancy after uterine, ovarian, and cervical Risk factors HPV tobacco use high risk sexual activity vulvar intraepithelial neoplasia (VIN) cervical intraepithelial neoplasia (CIN) vulvar lichen sclerosis Protective factors HPV vaccination smoking cessation ETIOLOGY Pathophysiology vulvar squamous cell adenocarcinoma can be secondary to HPV infection chronic inflammatory or autoimmune process Presentation Symptoms pruritus patients may report vulvar bleeding or pain Physical exam vulvar lesion plaque, ulcer, or mass most commonly on the labia major lesions in squamous cell carcinoma are firm white, red, or skin-colored Studies Invasive studies biopsy indication confirms the diagnosis colposcopy indication used to visualize the vulva if lesions are not appreciated grossly Differential Vaginal cancer differentiating factors malignant cells arising from the vagina Treatment Surgical excision indication treatment of choice for local disease Complications Surgical complications sexual dysfunction urinary and fecal incontinence introital stenosis