Clear-cell adenocarcinoma of the vagina and cervix in girls: Analysis of 170 Registry cases

One hundred cases of vaginal and 70 cervical adenocarcinomas from the Registry of Clear-Cell Adenocarcinoma of the Genital Tract in Young Females have been analyzed. The age range of the patients was 7 to 29 years, and the frequent association with prenatal exposure to diethylstilbestrol and similar nonsteroidal estrogens was confirmed. The hormone administration began prior to the eighteenth week of pregnancy and was continued for periods ranging from 1 week to almost the entire length of the pregnancy. The total dosages ranged from 300 to 18,200 mg. Although most patients had vaginal bleeding or discharge, 16 per cent were asymptomatic. Abnormal cytology was the first clue to the diagnosis of cancer in 11 patients, but 21 per cent of the smears were negative. The larger and more deeply invasive tumors were often complicated by lymph node metastases, but these were also encountered in 1 case in which the tumor had an area of only 3 sq. cm. and with another tumor that invaded less than 3 mm. These findings suggest that local treatment of the primary tumor alone may be inadequate in some cases. Recurrences developed in 37 of the patients and 24 of them died, although the follow-up in one third of the cases has been less than 2 years. The recurrences frequently involved the lungs and supraclavicular lymph nodes as well as the pelvis. The very common association of vaginal adenosis and the occasional coexistence of transverse vaginal or cervical ridges provide morphologic evidence of a stilbestrol-related disturbance in the development of the lower Müllerian tract. The results of intravenous pyelography suggest that the development of the urinary tract is not affected. The fact that all the asymptomatic patients with carcinoma have been successfully treated thus far underscores the importance of screening exposed asymptomatic patients in search of early cases. The rarity (9 per cent) of these cancers prior to the age of 12 years suggests that the inclusion of a large population of girls in this age group in a screening program would uncover very few cases. However, such individuals should certainly be examined at any time abnormal vaginal bleeding or discharge develops. © 1974.

Duke Scholars

Author Stanley J. Robboy Pathology