The precise causes of vulvar carcinoma vary by histologic subtype. Squamous cell carcinoma of the vulva, the most common subtype, is associated in many cases with persistent infection by high-risk strains of human papillomavirus. A separate pathway involves vulvar intraepithelial neoplasia arising in the context of chronic inflammatory skin conditions such as lichen sclerosus, which is not HPV-driven. Risk factors include a history of HPV infection, prior cervical or vaginal intraepithelial neoplasia, immunosuppression, smoking, and chronic vulvar inflammatory conditions. Adenocarcinoma and other rarer subtypes arise through distinct mechanisms related to the glandular or specialized cell populations of the region. No specific germline gene mutations are listed in the current data packet for this condition.

Diagnosis of vulvar carcinoma is established through biopsy of the suspicious lesion, with histopathologic examination confirming the presence and type of malignancy. Clinical evaluation includes thorough inspection of the vulva and surrounding structures. Imaging studies including computed tomography, magnetic resonance imaging, and positron emission tomography may be used for staging to assess regional lymph node involvement and distant spread. Surgical staging, including sentinel lymph node biopsy or inguinofemoral lymphadenectomy depending on lesion characteristics, provides additional staging information. The differential diagnosis includes benign vulvar conditions such as lichen sclerosus, contact dermatitis, vulvar intraepithelial neoplasia, Paget disease of the vulva, and other infectious or inflammatory conditions. Accurate staging according to established gynecologic oncology criteria is essential for treatment planning.

Treatment of vulvar carcinoma is individualized based on histologic subtype, tumor size, location, depth of invasion, and the presence or absence of lymph node involvement. Surgical resection is the foundational treatment for early-stage disease and may involve wide local excision or more extensive procedures depending on tumor extent. Assessment and management of regional inguinofemoral lymph nodes is an important component of surgical planning. Radiation therapy, delivered externally or through brachytherapy, may be used as primary treatment for patients who are not surgical candidates, as adjuvant therapy after surgery, or for locally advanced disease. Concurrent chemotherapy is often administered alongside radiation to enhance local control. Systemic therapy, including immune checkpoint inhibitors and targeted agents, is under active investigation for recurrent, persistent, or metastatic disease not amenable to surgery or radiation. A multidisciplinary team including gynecologic oncology, radiation oncology, and medical oncology is central to treatment planning.

Prognosis in vulvar carcinoma depends substantially on the stage at diagnosis and the extent of lymph node involvement. Individuals diagnosed with early-stage, node-negative disease generally have more favorable outcomes following surgical treatment. The presence of regional lymph node metastases is among the most significant factors associated with a less favorable outlook. Recurrence after primary treatment can occur and may involve the primary site, regional lymph nodes, or distant organs. Ongoing surveillance following treatment is important for early detection of recurrence. Research into systemic therapies for advanced and recurrent disease continues to evolve.

Several clinical trials are evaluating treatment approaches for vulvar carcinoma and related gynecologic malignancies. Studies include investigation of immune checkpoint inhibitor combinations, such as pembrolizumab with lenvatinib, for recurrent, persistent, metastatic, or locally advanced disease. Other trials are examining novel biologic agents in advanced solid tumor settings that include vulvar carcinoma. Supportive care studies are also underway, including evaluation of integrative therapies to reduce treatment-associated symptoms in patients with gynecologic malignancies receiving brachytherapy. These investigations reflect an active research environment aimed at expanding options for patients with advanced or treatment-resistant disease.