Clear cell adenocarcinomas have been seen more frequently in young women since 1970 because of the association with intrauterine exposure to diethylstilbestrol. Three predominant histologic patterns are found with clear cell carcinoma; they have been described as tubulocystic, solid, and papillary patterns. Most clear cell carcinomas of the vagina are polypoid or nodular, with a reddish color.
Clear cell carcinomas can spread locally and by the lymphatic and hematogenous routes. Metastases to regional pelvic nodes have been found in approximately one-sixth of stage I cases. Spread to regional pelvic nodes becomes more frequent in higher-stage tumors.
Clear cell adenocarcinomas are staged as other carcinomas of the vagina are by the FIGO. Some 80% have been diagnosed as stage I or II.
Several prognostic factors have been identified. Older patients (ie, > 19 years of age) have a more favorable prognosis than younger patients.86 This difference has been associated with the presence of a more favorable tubulocystic pattern of clear cell adenocarcinoma, which is the most frequent histologic pattern found in older patients. In addition, smaller tumor diameter and superficial depth of invasion correlate with improved patient survival.
Survival also depends on the stage of the disease. In 547 patients treated for clear cell adenocarcinoma of the vagina, the 5-year survival rate for those in stages I, II, III, and IV has been 93%, 83%, 37%, and 0%, respectively (
Because of the young age of these patients, surgery often is the primary therapy. For stage I and early stage II disease (
Figure 100-8), radical hysterectomy, partial or complete vaginectomy, pelvic lymphadenectomy, and replacement of the vagina with a split-thickness skin graft have been the approaches most frequently used.
In patients with small stage I tumors of the vagina, efforts have been made to preserve fertility. The tumor has been excised with retroperitoneal lymph node dissection, followed by local radiation. Senekjian and colleagues reported that the survival rate of patients with small vaginal tumors treated with such an approach compares favorably with that of patients treated with conventional therapy. In their series, eight pregnancies were reported in five patients who were treated locally.
Larger tumors have been treated with whole-pelvis radiation in addition to intracavitary implant. For tumors greater than 2 cm, whole-pelvis radiation of 4,000 to 5,000 cGy has been given, with an additional implant of 3,000 to 4,000 cGy. In a few instances, exenterative surgery has been performed for larger tumors; however, this procedure usually has been applied to central recurrences following primary radiation therapy.
If there is a recurrence, therapy consists of additional radical surgery, often requiring exenteration or extensive radiation localized to the pelvis. Systemic chemotherapy has been used in cases of metastatic disease. Cisplatin (75-100 mg/m2) with a continuous infusion of 5-FU (1 g/m2 for 3 to 5 days every 3 to 4 weeks) is currently recommended. However, no single agent or combination of chemotherapeutic agents has emerged as the most effective. Prolonged follow-up is necessary because recurrences, especially in the lungs and supraclavicular areas, have been reported as long as 19 years after primary therapy.
Woodruff JD, Genadry R, Poliakoff S. Treatment of dyspareunia and vaginal outlet distortions by perineoplasty. Obstet Gynecol 1981;57:750.