Clear-cell adenocarcinoma of the vagina and cervix in young females: (Analysis of 37 tumors that persisted or recurred after primary therapy)

Persistent or recurrent tumor complicated 37 of 154 cases submitted to the Registry of Clear-Cell Adenocarcinoma of the Genital Tract in Young Females. The maternal histories were available in 32 cases, and at least 82% were positive for the treatment of a high-risk pregnancy by diethylstilbestrol. Seventeen tumors were vaginal Stage I or cervical Stage I or IIA; abdominal exploration of 13 of these cases revealed involvement of the pelvic lymph nodes in 8. The remaining 20 cancers were more advanced (vaginal stages II–IV, cervical stages IIB–IV). Tumors that were large, close to the resection margin, or penetrated more than 3 mm into the vaginal or cervical wall persisted or recurred frequently. The first reappearance of tumor was encountered on an average of 17 months after the initial therapy, while the second was observed after an additional average interval of 9 months. The sites of disease reappearance were mainly the pelvis (53%) and the lungs and/or supraclavicular lymph nodes (35%); this distribution is unlike that of recurrent squamous cell carcinoma of the cervix or vagina, which is clinically apparent in the lungs or supraclavicular areas only infrequently. Effective therapy of the recurrent tumor was accomplished in some cases by radical surgical procedures or cancerocidal dosages of radiation. Six patients are alive and free of tumor 6 to 69 months after the first recurrence was treated; 5 of these patients had recurrences localized to the vagina, while the 6th had a metastasis to an inguinal lymph node. Twenty-four of the 37 patients died; the survival rate was 23% 3 years after the initial reappearance of tumor. These data indicate that clear-cell adenocarcinoma of the cervix and vagina in young females can be an aggressive and lethal form of cancer, but on occasion persistent and recurrent tumor can be treated successfully.