Clear-cell adenocarcinoma of the cervix in young women.

Abstract

This report describes 2 cases of clear-cell adenocarcinoma of the cervix in adolescent girls in whom a history of maternal administration of diethylstilbestrol (DES) during pregnancy was obtained. In 1 case other pathology was also present in other portions of the endocervix. The 1st case was a 16-year-old girl with a history of vaginal spotting and irregular periods for over a year. Examination revealed a large mass replacing the cervix and parametrial involvement. 1 month after completion of radiation therapy pelvic examination revealed no clinical evidence of remaining tumor. 4 months later a radical hysterectomy and salpingo-oophorectomy with pelvic node dissection showed no residual visable malignancy. 4 years later she shows no evidence of recurrent tumor. The 2nd case was a 15-year-old girl who had noted vaginal discharge for 5 months. Pelvic examination showed an eroded tumor covering most of the cervix. A radical hysterectomy was done with hemivaginectomy deep pelvic node dissection and an incidental appendectomy. When last seen 3 years later there was no evidence of recurrence on pelvic examination. The patients mother gave a history of 5 previous miscarriages and had been given DES during the first trimester of the pregnancy at risk because of vaginal bleeding. Most reported cases of vaginal adenocarcinoma in young women have been associated with intrauterine exposure to DES or other synthetic carcinogenic substances and with vaginal adenosis. Preliminary data record an 18% mortality rate with an additional 8% recurrence. Since cases have mostly occurred after puberty following a long latent period it is possible that the endogenous steroid hormones play a role in acting on epithelium previously primed during the first trimester of fetal life. Failure of the primitive mullerian epithelium to transform to a low stratified squamous type which normally occurs toward the end of the first trimester of pregnancy may represent the initial step in the later development of vaginal aadenosis and adenocarcinoma.