Vulvar cancer: Epidemiology, diagnosis, histopathology, and treatment of rare histologies
- 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
- Amer Karam, MD
Amer Karam, MD
- Clinical Associate Professor
- Associate Director and Director of Outreach
- Division of Gynecologic Oncology
- Department of Obstetrics and Gynecology
- Stanford University School of Medicine
- Section Editors
- Barbara Goff, MD
Barbara Goff, MD
- Section Editor — Gynecologic Oncology
- Director, Gynecologic Oncology
- University of Washington Medical Center
- Don S Dizon, MD, FACP
Don S Dizon, MD, FACP
- Section Editor – Gynecologic Oncology
- Head of Women's Cancers, Lifespan Cancer Institute
- Director of Medical Oncology, Rhode Island Hospital
- Associate Professor of Medicine, Warren Alpert Medical School of Brown University
Vulvar cancer is the fourth most common gynecologic malignancy in the United States (after uterine, ovarian, and cervical) . Squamous cell carcinoma is the most common histologic type of vulvar cancer, comprising at least 75 percent or more of cases [2,3]. Other histologies include melanoma, basal cell carcinoma, Bartholin gland adenocarcinoma, sarcoma, and Paget disease.
Human papillomavirus infection is associated with the majority of vulvar squamous cell carcinomas. In addition, vulvar lichen sclerosus is associated with an increased risk of vulvar cancers.
The epidemiology, clinical presentation, diagnosis, and histology of women with vulvar cancer will be reviewed here. Treatment of rare histologies is discussed here. Staging, treatment, and prognosis of squamous cell vulvar carcinoma are discussed separately. (See "Squamous cell carcinoma of the vulva: Staging and surgical treatment".)
In the United States (US), vulvar cancer is the fourth most common gynecologic malignancy (after uterine, ovarian, and cervical) . There are approximately 6000 cases and 1000 deaths from the disease each year.
Incidence rates by race or ethnicity in the US from 2010 to 2014 were: white (2.7 per 100,000 persons), black (1.8), Asian/Pacific Islander (0.9), Hispanic (1.8), and non-Hispanic (2.6) . In the United States, some data suggest that black women present with vulvar cancer at a younger age and have an increased probability of distant spread, which is a consistent finding across the different tumor models [5,6].To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- RISK FACTORS AND ETIOLOGY
- CLINICAL PRESENTATION
- Vulvar lesion
- Vulvar pruritus
- Other presentations
- DIAGNOSTIC EVALUATION
- DIFFERENTIAL DIAGNOSIS
- HISTOLOGIC TYPES
- Squamous cell carcinoma
- - Verrucous carcinoma
- Basal cell carcinoma
- Paget disease of the vulva
- Bartholin gland carcinoma
- TREATMENT OF RARE HISTOLOGIES
- Verrucous carcinoma
- Basal cell carcinoma
- Paget disease of the vulva
- Bartholin gland carcinoma
- SUMMARY AND RECOMMENDATIONS