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.