Note: Estimated new cases and deaths from vaginal (and other female genital) cancer in the United States in 2008:[1]

• New cases: 2,210.
• Deaths: 760.

Carcinomas of the vagina are uncommon tumors comprising 1% to 2% of gynecologic malignancies. They can be effectively treated, and when found in early stages, are often curable. The histologic distinction between squamous cell carcinoma and adenocarcinoma is important because the two types represent distinct diseases, each with a different pathogenesis and natural history. Squamous cell vaginal cancer (approximately 85% of cases) initially spreads superficially within the vaginal wall and later invades the paravaginal tissues and the parametria. Distant metastases occur most commonly in the lungs and liver.[2] Adenocarcinoma (approximately 15% of cases) has a peak incidence between 17 and 21 years of age and differs from squamous cell carcinoma by an increase in pulmonary metastases and supraclavicular and pelvic node involvement.[3] Rarely, melanoma and sarcoma are described as primary vaginal cancers. Adenosquamous carcinoma is a rare and aggressive mixed epithelial tumor comprising approximately 1% to 2% of cases.

Prognosis depends primarily on the stage of disease, but survival is reduced in patients who are greater than 60 years of age, are symptomatic at the time of diagnosis, have lesions of the middle and lower third of the vagina, or have poorly differentiated tumors.[4,5] In addition, the length of vaginal wall involvement has been found to be significantly correlated to survival and stage of disease in squamous cell carcinoma patients.[6]

Therapeutic alternatives depend on stage; surgery or radiation therapy is highly effective in early stages, while radiation therapy is the primary treatment of more advanced stages.[7,8] Chemotherapy has not been shown to be curative for advanced vaginal cancer, and there are no standard drug regimens.

Clear cell adenocarcinomas are rare and occur most often in patients less than 30 years of age who have a history of in utero exposure to diethylstilbestrol (DES). 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.[3] Young women with a history of in utero DES exposure should prospectively be followed carefully to diagnose this disease at an early stage. In women who have been carefully followed and well-managed, the disease is highly curable.

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. The natural history, prognosis, and treatment of other primary vaginal cancers (sarcoma, melanoma, lymphoma, and carcinoid tumors) may be different, and specific references should be sought.[9]

References

1. American Cancer Society.: Cancer Facts and Figures 2008. Atlanta, Ga: American Cancer Society, 2008. Also available online. Last accessed October 1, 2008. 
   
   
2. Gallup DG, Talledo OE, Shah KJ, et al.: Invasive squamous cell carcinoma of the vagina: a 14-year study. Obstet Gynecol 69 (5): 782-5, 1987.  [PUBMED Abstract]
   
   
3. Herbst AL, Robboy SJ, Scully RE, et al.: Clear-cell adenocarcinoma of the vagina and cervix in girls: analysis of 170 registry cases. Am J Obstet Gynecol 119 (5): 713-24, 1974.  [PUBMED Abstract]
   
   
4. Kucera H, Vavra N: Radiation management of primary carcinoma of the vagina: clinical and histopathological variables associated with survival. Gynecol Oncol 40 (1): 12-6, 1991.  [PUBMED Abstract]
   
   
5. Eddy GL, Marks RD Jr, Miller MC 3rd, et al.: Primary invasive vaginal carcinoma. Am J Obstet Gynecol 165 (2): 292-6; discussion 296-8, 1991.  [PUBMED Abstract]
   
   
6. Dixit S, Singhal S, Baboo HA: Squamous cell carcinoma of the vagina: a review of 70 cases. Gynecol Oncol 48 (1): 80-7, 1993.  [PUBMED Abstract]
   
   
7. Perez CA, Camel HM, Galakatos AE, et al.: Definitive irradiation in carcinoma of the vagina: long-term evaluation of results. Int J Radiat Oncol Biol Phys 15 (6): 1283-90, 1988.  [PUBMED Abstract]
   
   
8. Pride GL, Schultz AE, Chuprevich TW, et al.: Primary invasive squamous carcinoma of the vagina. Obstet Gynecol 53 (2): 218-25, 1979.  [PUBMED Abstract]
   
   
9. Sulak P, Barnhill D, Heller P, et al.: Nonsquamous cancer of the vagina. Gynecol Oncol 29 (3): 309-20, 1988.  [PUBMED Abstract]
   
   

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