1-2% of all gyn cancers.

1. Sarcoma botryoides: embryonal rhabdomyosarcoma. 90% occur before 5y of age. Appearance of red-tan grape clusters protruding from vagina. Frequent lymphatic mets to groin and pelvis. Hematogenous mets occur as well.

2. Endodermal sinus tumor: germ cell origin (see ovary chapter). Occurs in infants. Can be mistaken for sarcoma botryoides.

1. Features of in-utero DES exposure: Risk of CCC (Vagina >> cervix) is 1:1000. History of in-utero DES exposure present in 67% of vaginal CCC, 33% of cervical CCC. Mean age of DES associated CCC 19y (range 7-42). Three histologic subtypes: tubulocystic, papillary, solid. Tubulocystic has better prognosis (88% vs 73% 5y survival) and usually occurs after age 19. Adenosis present in 33%. Squamous metaplasia in adenosis makes colposcopic evaluation of dysplasia difficult, but causes eventual regression of adenosis. Lifetime risk of vaginal dysplasia increased. Probably no significant increased risk of breast cancer (controversial). DES associated uterine anomalies (80%) and infertility widely reported.

2. Recommended routine DES exam: Initial colposcopy, pap smear, and palpation of vagina. Take separate pap from vagina and cervix. If adenosis present, re-examine q6 months with colposcopy at least every 4th visit.

1. Squamous carcinoma: Mean age 60-65y. Most common in upper 1/3 of vagina, and on posterior wall. Lymphatic drainage mimics cervix for upper vaginal lesions, and vulva for lower vaginal lesions. Frequently associated with HPV (usually HPV 16, see cervix chapter).

2. Malignant melanoma: Average age 55y (range 22-83). Usually in lower 1/3 of vagina, and on anterior wall. Pigmented in 95%, amelanotic in 5%.

3. Rarely, tumors develop in Gartner's duct cysts (Wolffian system) and Müllerian duct cysts. They tend to be located anterolaterally, and anteriorly, respectively. Lymphoma and sarcoma (adult type) also occur rarely.