Vulvar cancer: Clinical manifestations, diagnosis, and pathology
- John C Elkas, MD, JD
John C Elkas, MD, JD
- Associate Professor
- Inova Teaching Faculty
- Department of Obstetrics and Gynecology
- Virginia Commonwealth University
- Jonathan S Berek, MD, MMS
Jonathan S Berek, MD, MMS
- Laurie Kraus Lacob Professor
- Stanford University School of Medicine
- Fellow, Stanford Distinguished Careers Institute
- Director, Stanford Women's Cancer Center
- Senior Scientific Advisor, Stanford Cancer Institute
- Section Editors
- Barbara Goff, MD
Barbara Goff, MD
- Section Editor — Gynecologic Oncology
- Professor of Gynecologic Oncology
- University of Washington
- Arno J Mundt, MD
Arno J Mundt, MD
- Section Editor — Radiation Therapy
- Chairman of Radiation Oncology
- University of California, San Diego
- Don S Dizon, MD, FACP
Don S Dizon, MD, FACP
- Section Editor – Gynecologic Oncology
- Clinical Co-Director, Gynecologic Oncology
- Founder and Director, The Oncology Sexual Health Clinic
- Massachusetts General Hospital Cancer Center
- Associate Professor of Medicine
- Harvard Medical School
Vulvar cancer is the fourth most common gynecologic cancer (following cancer of the uterine corpus, ovary, and cervix) and comprises 5 percent of malignancies of the female genital tract. Although the rate of invasive vulvar carcinoma has remained stable over the past two decades, the incidence of in situ disease (vulvar intraepithelial neoplasia) has more than doubled.
The clinical presentation, diagnosis, histology, and staging evaluation of women with vulvar cancer will be reviewed here. Treatment and prognosis are discussed separately. (See "Squamous cell carcinoma of the vulva: Staging and surgical treatment".)
EPIDEMIOLOGY AND RISK FACTORS
The age-adjusted incidence of vulvar cancer in the United States was 2.5 per 100,000 women, based upon 1997 to 2004 data from a national cancer database . Vulvar carcinoma is encountered most frequently in postmenopausal women. The mean age at diagnosis is 65, but may be falling. This was illustrated in a study of 78 women diagnosed with vulvar cancer between 1979 and 1993 in which the average age at presentation dropped from 69 to 55 during this interval . Within the United States, almost 4900 cases are diagnosed each year, with over 1000 of those women expected to succumb to their disease .
Risk factors for vulvar cancer include cigarette smoking, vulvar dystrophy (eg, lichen sclerosus), vulvar or cervical intraepithelial neoplasia, human papillomavirus (HPV) infection, immunodeficiency syndromes, a prior history of cervical cancer, and northern European ancestry [4,5].
Two independent pathways of vulvar carcinogenesis are felt to currently exist, the first related to mucosal HPV infection and the second related to chronic inflammatory (vulvar dystrophy) or autoimmune processes . HPV has been shown to be responsible for 60 percent of vulvar cancers . Specifically, HPV 16 and 33 are the predominant subtypes accounting for 55.5 percent of all HPV-related vulvar cancers .
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