Descriptive epidemiology of Ewing tumors analyses of the German patients of(EI)CESS 1980-1997

Background: Due to low absolute morbidity rates a reliable descriptive epid emiology of the Ewing family of tumors (EFT) has been mainly provided by la rge population-based cancer registry reports. To date, only few reports on this topic have been published. Patients: The present report is based on data of 945 German patients who we re enrolled into the (EI)CESS therapy studies between 1980 and 1997. It is assumed that under the age of 15 years an almost complete registration of a ll German patients with Ewing's sarcoma has been achieved. Diagnoses in all patients were ascertained by biopsies. Methods: In this analysis we looked at the associations between year of fir st diagnosis, age at first diagnosis, gender, the histological subtypes and the primary skeletal localisation of the tumours. Results: The majority of patients with EFT was male (60%). More than half o f the patients developed the dis ease between age 10 and 19(57%) while abou t 20% were younger and almost a quarter was diagnosed after age 20 years. T he percentage of patients older than 15 years was higher in men (55%) than in women (45%). The age-standardized annual incidence before age 15 years w as estimated as 3 per million, and the cumulative incidence up to that age as about 46 per million. Long bones of the low er extremities (32%, femur a lone 16%) and the pelvis were the most common sites of primary localisation . While 70 to 80% of all tumours in the long bones of the extremities were composed of Ewing's sarcomas, about one third in the central skeleton and o ver 20% in the pelvic bones were Primitive neuroectodermal tumours (PNET). Atypical Ewing's sarcoma accounted for about 10% of tumours in all sites. Conclusions: The large (EI)CESS database enables the meaningful and reliabl e description of epidemiological characteristics of the rare occurrence of the Ewing family of tumours. Further analyses of this database seem to hold great promise.