Rare (only 1–2% of all gynecologic malignancies). Twenty percent of vaginal tumors are detected incidentally as a result of Pap smear cytologic screening for cervical cancer. Eighty to ninety percent of cases are squamous cell carcinomas; most common location is the upper posterior 1/3 of the vagina. Lymph node drainage from the upper 2/3 of the vagina is to the pelvic nodes and from the lower 1/3 of the vagina is to the inguinal/femoral nodes. VAIN (vaginal intraepithelial neoplasia) associated with human papilloma virus (HPV); frequently multifocal, and progresses to invasive disease. Melanoma comprises 5% and most frequently occur in the lower 1/3 of the vagina. Adenocarcinoma comprises 5–15% and frequently presents in the Bartholin’s or Skene’s glands. Verrucous carcinomas tend to recur locally, but rarely metastasize. Rare histologies include papillary serous adenocarcinoma, small cell carcinoma, botryoid variant of embryonal rhabdomyosarcoma, lymphoma, and clear cell adenocarcinoma (which is associated with in utero exposure to diethylstilbestrol (DES)). Pelvic disease control worse in primary non-DES–associated adenocarcinoma of the vagina compared to squamous cell carcinoma (31 vs. 81%; p < 0.01) (Frank et al. 2007). Risk factors: carcinoma in situ, HPV, chronic vaginal irritation, previous abnormal Pap smears, early hysterectomy, multiple lifetime sex partners, early age at first intercourse, current smoker, in utero exposure to DES, partner with penile cancer. Most significant prognostic factor is FIGO stage; other adverse prognostic factors: age >60 years, middle or lower 1/3 location, poorly differentiated, size, and anemia (Chyle et al. 1996; Perez et al. 1999; Tran et al. 2006). External beam radiotherapy in combination with brachytherapy has been shown to improve survival compared to external beam treatment alone. The role for chemotherapy (usually cisplatin-based) is based on small phase I and II studies and extrapolated from cervical cancer literature (Morris et al. 2004; Samant et al. 2007; National Cancer Institute, 2009). Combined analysis of three randomized clinical trials of the FDA-approved human papillomavirus quadrivalent (Types 6, 11, 16, 18) vaccine shows 50% efficacy (95% CI) for HPV18-related VAIN2/3 in the intention-to-treat population (Joura et al. 2007).
KeywordsHuman Papilloma Virus Small Cell Carcinoma Inguinal Node Urethral Stricture Penile Cancer
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