Managing problems of DES daughters.

The risk of developing a clear cell adenocarcinoma is estimated to be 0.14-1.4/1000 daughters of women who took diethylstilbestrol (DES) early in their pregnancies. DES related vaginal carcinomas usually arise on the anterior wall in the upper 3rd of the vagina; their size at diagnosis varies from 0.3-10 cm. Careful palpation of the entire cervix and vagina is a necessary part of the examination of DES exposed women. More than half of all patients with DES related vaginal and cervical cancers have had stage 1 lesions at the time of diagnosis. Treatment for most of these early cancers has been radical hysterectomy pelvic lymphadenectomy and partial or complete vaginectomy with vaginal reconstruction. The 5-year survival rate is 88%. Irradiation occasionally has been effective for treating pelvic recurrences. Even though clear cell adenocarcinoma of the vagina and cervix appears to metastasize to the lungs and supraclavicular lymph nodes more often than does squamous cancer the overall 5 year survival for all stages is 78% compared with only 55% for squamous cell cervix cancer and 35% for vaginal squamous cancer. To a great extent the difference is survival results from the greater frequency of early stage cancer in DES exposed patients most of whom have been watched closely and evaluated carefully. The widely publicized DES related vaginal and cervical clear cell cancers are rare but nonmalignant structural and epithelial changes are fairly common. Adenosis -- vaginal glandular epithelium -- is clinically detectable in 35-90% of DES exposed patients. Incidence is greatest in patients exposed early in gestation to high total doses of DES. It is very rare in patients not exposed to DES. Immediate evaluation is very important in a DES exposed woman of any age if she has abnormal bleeding or discharge. Asymptomatic patients should have their 1st pelvic examination at age 14 or 1 year after menarche whichever is earlier. The routine pelvic exam should be modified to evaluate the changes associated with DES exposure. Cytologic specimens should be taken from the cervix and from the vaginal fornices and a biopsy should be taken of any suspicious nodularity. Colposcopic examination is mandatory for further evaluation of the patient with abnormal cytology. Gross structural abnormalities of the cervix and vagina are seen in 20-60% of DES exposed women. At least some of these lower genital tract structural abnormalities appear to regress spontaniously over several years of observaton. Problems with menstrual dysfunction infertility and increased pregnancy looses have become the most frequent sequelae of in utero DES exposure.