Clear-Cell Adenocarcinoma of the Genital Tract in Young Females: Registry Report

Among 91 Registry cases of vaginal and cervical adenocarcinoma predominantly of the clear-cell type, the patients, eight to 25 years of age, were born throughout the United States and in three foreign countries. Pregnancy histories of 66 of the patients' mothers have been obtained; 49 received stilbestrol or related drugs, nine received unidentified drugs administered because of bleeding or prior pregnancy loss, and eight had a negative history. The dosage and duration of therapy varied widely. The frequent coexistence of vaginal adenosis and occasional presence of transverse vaginal or cervical ridges suggested abnormal development of müllerian epithelium in utero. Seventeen of 65 patients have died or had recurrences despite brief follow-up periods in many cases. Although these cancers are rare, the frequency of asymptomatic cases and the unreliability of vaginal cytology for detection support the value of screening postmenarchal females who have been exposed prenatally to stilbestrol or related drugs. RECENTLY, clear-cell adenocarcinomas of the vagina and cervix in young females have been reported with increasing frequency.^{1 2 3 4 5 6 7} The development of most of the vaginal tumors has been linked to the maternal ingestion of diethylstilbestrol or chemically related, nonsteroidal synthetic estrogens during pregnancy.^{8 9 10 11 12 13} Because these tumors are rare and little is known of their behavior, the Registry of Clear-Cell Adenocarcinoma (Mesonephroma) of the Genital Tract in Young Females^{14 15 16} has been established to centralize data and accelerate the acquisition of information. Ninety-one cases had been submitted to the Registry before June 1, 1972, through the generous co-operation of over 200 physicians, to.