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
- Director, Stanford Women's Cancer Center
- Stanford Comprehensive Cancer Institute
- Chair, Department of Obstetrics & Gynecology
- Stanford University School of Medicine
- 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 .
Subscribers log in hereLiterature review current through: Feb 2017. | This topic last updated: Tue Sep 08 00:00:00 GMT 2015.References
- Bodelon C, Madeleine MM, Voigt LF, Weiss NS. Is the incidence of invasive vulvar cancer increasing in the United States? Cancer Causes Control 2009; 20:1779.
- Messing MJ, Gallup DG. Carcinoma of the vulva in young women. Obstet Gynecol 1995; 86:51.
- Siegel R, Ma J, Zou Z, Jemal A. Cancer statistics, 2014. CA Cancer J Clin 2014; 64:9.
- Ansink A. Vulvar squamous cell carcinoma. Semin Dermatol 1996; 15:51.
- Madsen BS, Jensen HL, van den Brule AJ, et al. Risk factors for invasive squamous cell carcinoma of the vulva and vagina--population-based case-control study in Denmark. Int J Cancer 2008; 122:2827.
- de Koning MN, Quint WG, Pirog EC. Prevalence of mucosal and cutaneous human papillomaviruses in different histologic subtypes of vulvar carcinoma. Mod Pathol 2008; 21:334.
- Monk BJ, Burger RA, Lin F, et al. Prognostic significance of human papillomavirus DNA in vulvar carcinoma. Obstet Gynecol 1995; 85:709.
- Insinga RP, Liaw KL, Johnson LG, Madeleine MM. A systematic review of the prevalence and attribution of human papillomavirus types among cervical, vaginal, and vulvar precancers and cancers in the United States. Cancer Epidemiol Biomarkers Prev 2008; 17:1611.
- Zacur H, Genadry R, Woodruff JD. The patient-at-risk for development of vulvar cancer. Gynecol Oncol 1980; 9:199.
- Collins CG, Lee FY, Roman-Lopez JJ. Invasive carcinoma of the vulva with lymph node metastasis. Am J Obstet Gynecol 1971; 109:446.
- Hildesheim A, Han CL, Brinton LA, et al. Human papillomavirus type 16 and risk of preinvasive and invasive vulvar cancer: results from a seroepidemiological case-control study. Obstet Gynecol 1997; 90:748.
- Iwasawa A, Nieminen P, Lehtinen M, Paavonen J. Human papillomavirus in squamous cell carcinoma of the vulva by polymerase chain reaction. Obstet Gynecol 1997; 89:81.
- Al-Ghamdi A, Freedman D, Miller D, et al. Vulvar squamous cell carcinoma in young women: a clinicopathologic study of 21 cases. Gynecol Oncol 2002; 84:94.
- Elit L, Voruganti S, Simunovic M. Invasive vulvar cancer in a woman with human immunodeficiency virus: case report and review of the literature. Gynecol Oncol 2005; 98:151.
- Bosch FX, Manos MM, Muñoz N, et al. Prevalence of human papillomavirus in cervical cancer: a worldwide perspective. International biological study on cervical cancer (IBSCC) Study Group. J Natl Cancer Inst 1995; 87:796.
- Vinokurova S, Wentzensen N, Einenkel J, et al. Clonal history of papillomavirus-induced dysplasia in the female lower genital tract. J Natl Cancer Inst 2005; 97:1816.
- Castle PE, Schiffman M, Bratti MC, et al. A population-based study of vaginal human papillomavirus infection in hysterectomized women. J Infect Dis 2004; 190:458.
- Piura B, Rabinovich A, Dgani R. Basal cell carcinoma of the vulva. J Surg Oncol 1999; 70:172.
- Piura B, Egan M, Lopes A, Monaghan JM. Malignant melanoma of the vulva: a clinicopathologic study of 18 cases. J Surg Oncol 1992; 50:234.
- Sugiyama VE, Chan JK, Shin JY, et al. Vulvar melanoma: a multivariable analysis of 644 patients. Obstet Gynecol 2007; 110:296.
- Stang A, Streller B, Eisinger B, Jöckel KH. Population-based incidence rates of malignant melanoma of the vulva in Germany. Gynecol Oncol 2005; 96:216.
- Chang AE, Karnell LH, Menck HR. The National Cancer Data Base report on cutaneous and noncutaneous melanoma: a summary of 84,836 cases from the past decade. The American College of Surgeons Commission on Cancer and the American Cancer Society. Cancer 1998; 83:1664.
- and Practice of Gynecologic Oncology, 2nd, Hoskins WJ, Perez CA, Young RC (Eds), Lippincott-Raven, 1997.
- de Giorgi V, Salvini C, Massi D, et al. Vulvar basal cell carcinoma: retrospective study and review of literature. Gynecol Oncol 2005; 97:192.
- Benedet JL, Miller DM, Ehlen TG, Bertrand MA. Basal cell carcinoma of the vulva: clinical features and treatment results in 28 patients. Obstet Gynecol 1997; 90:765.
- Parker LP, Parker JR, Bodurka-Bevers D, et al. Paget's disease of the vulva: pathology, pattern of involvement, and prognosis. Gynecol Oncol 2000; 77:183.
- Shepherd V, Davidson EJ, Davies-Humphreys J. Extramammary Paget's disease. BJOG 2005; 112:273.
- Fanning J, Lambert HC, Hale TM, et al. Paget's disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision. Am J Obstet Gynecol 1999; 180:24.
- Feuer GA, Shevchuk M, Calanog A. Vulvar Paget's disease: the need to exclude an invasive lesion. Gynecol Oncol 1990; 38:81.
- Felix JC, Cote RJ, Kramer EE, et al. Carcinomas of Bartholin's gland. Histogenesis and the etiological role of human papillomavirus. Am J Pathol 1993; 142:925.
- Copeland LJ, Sneige N, Gershenson DM, et al. Bartholin gland carcinoma. Obstet Gynecol 1986; 67:794.
- Cardosi RJ, Speights A, Fiorica JV, et al. Bartholin's gland carcinoma: a 15-year experience. Gynecol Oncol 2001; 82:247.
- Hacker NF, Nieberg RK, Berek JS, et al. Superficially invasive vulvar cancer with nodal metastases. Gynecol Oncol 1983; 15:65.
- Hacker NF, Berek JS, Lagasse LD, et al. Management of regional lymph nodes and their prognostic influence in vulvar cancer. Obstet Gynecol 1983; 61:408.