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Vulvar cancer: Epidemiology, diagnosis, histopathology, and treatment of rare histologies

Jonathan S Berek, MD, MMS
Amer Karam, MD
Section Editors
Barbara Goff, MD
Don S Dizon, MD, FACP
Deputy Editor
Sandy J Falk, MD, FACOG


Vulvar cancer is the fourth most common gynecologic malignancy in the United States (after uterine, ovarian, and cervical) [1]. 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 (table 1).

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) [1]. 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) [4]. 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].

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Literature review current through: Nov 2017. | This topic last updated: Jul 05, 2017.
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