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

被引:30
作者
Hense, HW
Ahrens, S
Paulussen, M
Lehnert, M
Jürgens, H
机构
[1] Univ Munster, Inst Epidemiol & Sozialmed, Bereich Klin Epidemiol, D-48129 Munster, Germany
[2] Univ Munster, Klin & Poliklin Kinderheilkunde Padiat Hamatol On, D-48129 Munster, Germany
[3] Epidemiol Krebsregister Regierungsbezirk Munster, Munster, Germany
来源
KLINISCHE PADIATRIE | 1999年 / 211卷 / 04期
关键词
Ewing's sarcoma; epidemiology; EICESS; incidence; primary sites; histological subtypes;
D O I
10.1055/s-2008-1043799
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
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 dis ease 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 low er 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.
引用
收藏
页码:271 / 275
页数:5
相关论文
共 23 条
[1]   CHILDHOOD-CANCER INCIDENCE IN THE SOUTH-EAST OF FRANCE - A REPORT OF THE PROVENCE-ALPES-COTE DAZUR AND CORSICA REGIONS PEDIATRIC CANCER REGISTRY, 1984-1991 [J].
BERNARD, JL ;
BERNARDCOUTERET, E ;
COSTE, D ;
THYSS, A ;
SCHEINER, C ;
PERRIMOND, H ;
MARIANI, R ;
DEVILLE, A ;
MICHEL, G ;
GENTET, JC ;
RAYBAUD, C .
EUROPEAN JOURNAL OF CANCER, 1993, 29A (16) :2284-2291
[2]  
DEHNER LP, 1994, ARCH PATHOL LAB MED, V118, P606
[3]   THE EWING FAMILY OF TUMORS - A SUBGROUP OF SMALL-ROUND-CELL TUMORS DEFINED BY SPECIFIC CHIMERIC TRANSCRIPTS [J].
DELATTRE, O ;
ZUCMAN, J ;
MELOT, T ;
GARAU, XS ;
ZUCKER, JM ;
LENOIR, GM ;
AMBROS, PF ;
SHEER, D ;
TURCCAREL, C ;
TRICHE, TJ ;
AURIAS, A ;
THOMAS, G .
NEW ENGLAND JOURNAL OF MEDICINE, 1994, 331 (05) :294-299
[4]  
DUNST J, 1991, CANCER, V67, P2818, DOI 10.1002/1097-0142(19910601)67:11<2818::AID-CNCR2820671118>3.0.CO
[5]  
2-Y
[6]   RADIATION-THERAPY IN EWINGS-SARCOMA - AN UPDATE OF THE CESS-86 TRIAL [J].
DUNST, J ;
JURGENS, H ;
SAUER, R ;
PAPE, H ;
PAULUSSEN, M ;
WINKELMANN, W ;
RUBE, C .
INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS, 1995, 32 (04) :919-930
[7]   The Ewing family of tumors - Ewing's sarcoma and primitive neuroectodermal tumors [J].
Grier, HE .
PEDIATRIC CLINICS OF NORTH AMERICA, 1997, 44 (04) :991-+
[8]  
HARTMAN KR, 1991, CANCER-AM CANCER SOC, V67, P163, DOI 10.1002/1097-0142(19910101)67:1<163::AID-CNCR2820670128>3.0.CO
[9]  
2-Y
[10]   THE GPO COOPERATIVE EWINGS-SARCOMA STUDIES CESS-81-86 - REPORT AFTER 61/2 YEARS [J].
JURGENS, H ;
BIER, V ;
DUNST, J ;
HARMS, D ;
JOBKE, A ;
KOTZ, R ;
KUHL, J ;
MULLERWEIHRICH, S ;
RITTER, J ;
SALZERKUNTSCHIK, M ;
SAUER, R ;
STERNSCHULTE, W ;
TREUNER, J ;
VOUTE, PA ;
WEINEL, P ;
WINKELMANN, W ;
WINKLER, K ;
GOBEL, U .
KLINISCHE PADIATRIE, 1988, 200 (03) :243-252