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.