Primary vaginal cancer is a rare occurrence. Most cases are metastatic from other sites. Common sites of primary cancer that metastasize to the vagina include the cervix, ovary, breast, rectum, kidney, and uterus. Primary vaginal cancer most commonly is squamous cell cancer (SCC). Other types of primary vaginal cancer include adenocarcinoma, melanoma, and sarcoma.

The cause of vaginal cancer is unknown, but human papillomavirus (HPV) infection can increase the risk of SCC. About 60% of vaginal cancers are related to HPV.

Incidence of vaginal cancer is 0.5-1 in 100 000, and it makes up about 3% of all gynecologic malignancies. The average age of diagnosis is 60 years for SCC but can be much younger for other types.

Most often, these lesions are asymptomatic early in the disease course, but patients may present with postcoital bleeding, pain with intercourse, a vaginal lesion, a feeling of fullness / pressure, constipation, vaginal pain, painless abnormal uterine bleeding, or vaginal discharge.

A special separate population to consider for vaginal cancer is individuals who had in utero exposure to diethylstilbestrol (DES). This medicine was used between 1940 and 1971 to help prevent complications of pregnancy but was eventually found to be an endocrine disruptor, leading to changes in the fetuses who were exposed. Given the time frame, these patients are now in their mid-40s to early 80s. DES exposure in utero carries a 40-times increased risk of developing vaginal clear cell adenocarcinoma. Most cases of vaginal cancer in these patients were diagnosed at an early age (as early as 8 years old), but cases up to age 50 have been reported. 

Another type of vaginal cancer that may be seen in the pediatric population is sarcoma botryoides. This is a form of embryonal rhabdomyosarcoma that arises from the vaginal wall in infants and girls younger than age 5.