Primary carcinoma of the vagina.

In this paper we reviewed the risk factors for primary carcinoma of the vagina (PCV), diagnostic and therapeutic modalities, and principles leading to rational decision-making in the individualized management of vaginal carcinoma patients. The review was based on the recent literature and our own years- long experience with the disease. PCV is a rare gynecologic malignancy accounting for about 2% of all neoplasms of the female genitals. Most of the affected women are over 60 years of age, peaking between 70 and 80 years. Only 10-15% of patients are below 50 years. Histopathologically, most common are squamous cell carcinoma (80-90%) and adenocarcinoma (4-10%). The leading risk factor for vaginal intraepithelial neoplasia (VAIN) and subsequent squamous cell vaginal carcinoma is long-lasting infection with human papillomavirus (HPV) type 16. Prognosis of the disease depends on several factors, the most important of which are age, histologic type, and tumor stage. Survival depends on the disease stage. Five -year survival rates are about 95% for stage 0, 75% for stage I, 60% for stage II, 35% for stage III, 20% for stage IVa, and 0% for IVb stage. Due to its being a rare entity, there are still controversies as to the most optimal treatment. Individualized treatment approaches have been increasingly used. In most centres, standard treatment for this cancer is radiotherapy. Some reports have shown that surgery might also be an option, while in some centres radiation is supplemented by cisplatin-based chemotherapy. The supposed advantage of radiotherapy is the preservation of the anatomy and function of the vagina. We believe that there are certain psychologic benefits with the preservation of the vagina, regardless of its function. However, preservation of the vaginal function after treatment of invasive vaginal cancer is a rare phenomenon, both in the literature and from our own experience.