Highlights
- Early detection of vulvar cancer significantly impacts prognosis and treatment effectiveness.
- HPV vaccination and lifestyle modifications are crucial in preventing vulvar cancer risk factors.
Overview and Epidemiology
Vulvar cancer is a rare malignancy of the external female genitalia, mainly affecting the labia majora and minora, with rising incidence and mortality worldwide. It primarily affects postmenopausal women but can occur at any age. About half of the cases are linked to persistent high-risk human papillomavirus (HPV) infection, especially types 16 and 18, typically in younger women. HPV-negative cancers often occur in older women and are associated with chronic inflammatory conditions like lichen sclerosus and genetic mutations such as p53 abnormalities.
In the United States, vulvar cancer rates have increased annually by about 0.7% for incidence and 2.1% for mortality over the past decade. HPV infection is a major factor, with approximately 40% of vulvar cancers HPV-related. Other risk factors include smoking, alcohol use, and sexual behavior. Epidemiological data are sourced from cancer registries and databases like Cancer Incidence in Five Continents Plus (CI5 Plus).
Signs, Symptoms, and Diagnosis
Symptoms vary by disease stage and may include persistent itching, burning, pain, sores, lumps, red or scaly patches, and abnormal bleeding. Early lesions can be asymptomatic and mimic benign conditions. High-risk HPV infections may cause precancerous changes with symptoms like lumps and bleeding.
Diagnosis involves physical examination, biopsy, and imaging studies such as MRI, CT, and PET/CT to determine disease extent and staging. Colposcopy or vulvoscopy with acetic acid or toluidine blue helps visualize abnormal cells. Sentinel lymph node mapping using indocyanine green fluorescence improves detection of lymphatic spread. Additional procedures like cystoscopy or proctoscopy assess involvement of adjacent organs. Cancer staging follows the FIGO system to guide treatment.
Causes and Risk Factors
Vulvar cancer develops via HPV-related and HPV-independent pathways. High-risk HPV types 16 and 18 cause about half of vulvar squamous cell carcinomas by disrupting cell regulation. HPV-negative cancers, more common in older women, are linked to lichen sclerosus and p53 mutations. Smoking synergistically increases risk in HPV-infected individuals. Environmental exposures and a history of cervical cancer also elevate risk.
Prevention
Prevention strategies include smoking cessation, limiting alcohol use, and practicing safe sex to reduce HPV exposure. HPV vaccination (bivalent, quadrivalent, and nonavalent vaccines) significantly lowers the risk of HPV-associated vulvar precancers and cancers, especially when given before age 17. Regular medical checkups aid early detection, while managing chronic vulvar conditions like lichen sclerosus with topical corticosteroids may reduce cancer risk.
Treatment
Treatment depends on cancer stage and spread. Early-stage disease is usually treated with surgery, such as radical vulvectomy and lymph node removal, sometimes followed by radiation. Radiation therapy may be used pre- or post-surgery or as primary treatment if surgery is contraindicated. Chemotherapy is combined with radiation to enhance effectiveness and used for metastatic or palliative care. Clinical staging guides therapy planning, with cystoscopy evaluating bladder involvement when needed.
Prognosis and Support
Prognosis varies by cancer type, stage, age, and overall health, with early detection improving outcomes. Support services provide 24/7 access to specialists, counseling, and resources for patients and caregivers. Palliative care focuses on symptom relief and quality of life in advanced cases. Evidence-based guidelines from professional organizations support optimal prevention, diagnosis, and treatment.
Research and Advances
Recent studies clarify HPV’s role in vulvar carcinogenesis through viral oncogenes E6 and E7 and differences between HPV-associated and independent cancers. Molecular insights inform targeted therapies and prognosis. Reviews of clinical guidelines emphasize standardized, evidence-based care. Treatment continues evolving with combined surgery, radiation, chemotherapy, and symptom management, including cautious use of topical corticosteroids for relief.
The content is provided by Avery Redwood, Scopewires
