General Information About Vaginal Cancer
Incidence and Mortality
Estimated new cases and deaths from vaginal (and other female genital) cancer in the United States in 2013:
- New cases: 2,890.
- Deaths: 840.
Early stage tumors are often curable with local modality therapies, but there is no standard treatment of proven efficacy for metastatic disease. A large proportion (30%–50%) of women with vaginal carcinomas have had a prior hysterectomy for benign, pre-malignant, or malignant disease.[3,4]
The American Joint Committee on Cancer (AJCC) staging system indicates that tumors in the vagina that involve the cervix of women with an intact uterus are classified as cervical cancers. Therefore, tumors that may have actually originated in the apical vagina but extend to the cervix would be classified as cervical cancers.
Squamous cell cancer (SCC) accounts for approximately 85% of vaginal cancer cases. SCC initially spreads superficially within the vaginal wall and later invades the paravaginal tissues and the parametria. Distant hematogenous metastases occur most commonly in the lungs, and less frequently in liver, bone, or other sites. SCC of the vagina is associated with a high rate of infection with oncogenic strains of human papillomavirus (HPV) and has many risk factors in common with SCC of the cervix.[6-8] HPV infection has also been described in a case of vaginal adenocarcinoma.
Approximately 5% to 10% of cases of vaginal cancers are adenocarcinomas. A rare form of adenocarcinoma (clear cell carcinoma, described below) occurs in association with in utero exposure to diethylstilbestrol (DES), with a peak incidence at young ages (less than 30 years). However, adenocarcinomas that are not associated with DES exposure occur primarily during postmenopausal years.
The association between the clear cell carcinomas and in utero exposure to DES was first reported in 1971. The incidence of this disease, which is highest for those exposed during the first trimester, peaked in the mid-1970s, reflecting the use of DES in the 1950s. It is extremely rare now. However, women with a known history of in utero DES exposure should be carefully followed for this tumor.
Vaginal adenosis is most commonly found in young women who had in utero exposure to DES and may coexist with a clear cell adenocarcinoma, though it rarely progresses to adenocarcinoma. Adenosis is replaced by squamous metaplasia, which occurs naturally, and requires follow-up but not removal.
Rarely, melanomas (often nonpigmented), sarcomas, or small-cell carcinomas have been described as primary vaginal cancers.Prognostic Factors
Patient prognosis depends primarily on the stage of disease, but survival is reduced among those who are older than 60 years, are symptomatic at the time of diagnosis, have lesions of the middle and lower third of the vagina, or have poorly differentiated tumors.
In addition, the length of vaginal wall involvement has been found to be associated with survival and stage of disease in vaginal SCC patients.
Non–DES-associated adenocarcinomas generally have a worse prognosis than SCC tumors, but DES-associated clear cell tumors have a relatively good prognosis. The natural history, prognosis, and treatment of other primary vaginal cancers (i.e., sarcoma, melanoma, lymphoma, and carcinoid tumors) are different and are not covered in this summary.
Therapeutic options depend on tumor stage; surgery and radiation therapy are highly effective in early stages, whereas radiation therapy is the primary treatment of more advanced stages. Chemotherapy has not been shown to be curative for advanced vaginal cancer, and there are no standard drug regimens.References
- American Cancer Society.: Cancer Facts and Figures 2013. Atlanta, Ga: American Cancer Society, 2013. Available online. Last accessed January 10, 2014.
- Eifel PJ, Berek JS, Markman MA: Cancer of the cervix, vagina, and vulva. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 1311-44.
- Eifel P, Berek J, Markman M: Cancer of the cervix, vagina, and vulva. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. Vols. 1 & 2. 8th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2008, pp 1496-1543.
- Stock RG, Chen AS, Seski J: A 30-year experience in the management of primary carcinoma of the vagina: analysis of prognostic factors and treatment modalities. Gynecol Oncol 56 (1): 45-52, 1995. [PUBMED Abstract]
- Vagina. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 387-9.
- Daling JR, Madeleine MM, Schwartz SM, et al.: A population-based study of squamous cell vaginal cancer: HPV and cofactors. Gynecol Oncol 84 (2): 263-70, 2002. [PUBMED Abstract]
- Parkin DM: The global health burden of infection-associated cancers in the year 2002. Int J Cancer 118 (12): 3030-44, 2006. [PUBMED Abstract]
- Ikenberg H, Runge M, Göppinger A, et al.: Human papillomavirus DNA in invasive carcinoma of the vagina. Obstet Gynecol 76 (3 Pt 1): 432-8, 1990. [PUBMED Abstract]
- Herbst AL, Ulfelder H, Poskanzer DC: Adenocarcinoma of the vagina. Association of maternal stilbestrol therapy with tumor appearance in young women. N Engl J Med 284 (15): 878-81, 1971. [PUBMED Abstract]