BACKGROUND: Due to low absolute morbidity rates a reliable descriptive epidemiology of the Ewing family of tumors (EFT) has been mainly provided by large 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 were 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 all 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 first 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 of the patients developed the disease between age 10 and 19 (57%) while about 20% were younger and almost a quarter was diagnosed after age 20 years. The 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 was estimated as 3 per million, and the cumulative incidence up to that age as about 46 per million. Long bones of the lower extremities (32%, femur alone 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 over 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 reliable description of epidemiological characteristics of the rare occurrence of the Ewing family of tumours. Further analyses of this database seem to hold great promise.
BACKGROUND: Due to low absolute morbidity rates a reliable descriptive epidemiology of the Ewing family of tumors (EFT) has been mainly provided by large 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 were 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 all 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 first 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 of the patients developed the disease between age 10 and 19 (57%) while about 20% were younger and almost a quarter was diagnosed after age 20 years. The 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 was estimated as 3 per million, and the cumulative incidence up to that age as about 46 per million. Long bones of the lower extremities (32%, femur alone 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 over 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 reliable description of epidemiological characteristics of the rare occurrence of the Ewing family of tumours. Further analyses of this database seem to hold great promise.
Authors: Gerhard M Hobusch; Jakob Bollmann; Stephan E Puchner; Nikolaus W Lang; Jochen G Hofstaetter; Philipp T Funovics; Reinhard Windhager Journal: Clin Orthop Relat Res Date: 2017-03 Impact factor: 4.176