Childhood nonovarian female genital tract cancers in Britain, 1962-1978. Descriptive epidemiology and long-term survival.
The files of the Childhood Cancer Research Group ( CCRG ) and of the Oxford Survey of Childhood Cancers ( OSCC ) were scrutinized for all malignant genital neoplasms other than ovarian tumor registered in England, Scotland, and Wales in the period 1962 to 1978. After a review of the records and of the available pathologic reports, a total of 55 cases were confirmed, indicating an average incidence of about 0.5 cases per million female children per year, with no obvious trend over the study period. These included 9 (16.4%) cases originating from the vulva, 28 (50.9%) from the vagina, 3 (5.5%) from the cervix, 12 (21.8%) from the corpus uteri, and also 3 (5.5%) cancers of unspecified pelvic sites. A total of 45 (81.8%) cases were sarcomas, most frequently of the botryoid type, 7 (11.7%) were carcinomas, and 3 (5.5%) were germ cell neoplasms. Fifteen cases (27.3%) occurred in children younger than 1 year of age, and incidence showed a declining trend over age. Long-term survival was more common in tumors of the external genitalia, vagina, or cervix, and significantly better in carcinomas (100%) than in germ cell neoplasms (67%), or sarcomas (34%). A significant improvement in survival was noted over the period of time considered (5-year survival rates were 23% in the cases diagnosed in the 1960s, but 52% for those diagnosed in the 1970s), suggesting that a more rational integrated therapeutic approach may appreciably improve the prognosis of these rare neoplasms.
D000293:Adolescent; D002277:Carcinoma / Q000401:mortality / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D005833:Genital Neoplasms, Female / Q000401:mortality* / Q000601:surgery; D006801:Humans; D007223:Infant; D009373:Neoplasms, Germ Cell and Embryonal / Q000401:mortality / Q000601:surgery; D012509:Sarcoma / Q000401:mortality / Q000601:surgery; D006113:United Kingdom; D002583:Uterine Cervical Neoplasms / Q000401:mortality / Q000601:surgery; D014594:Uterine Neoplasms / Q000401:mortality / Q000601:surgery; D014625:Vaginal Neoplasms / Q000401:mortality / Q000601:surgery; D014846:Vulvar Neoplasms / Q000401:mortality / Q000601:surgery