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尤文肉瘤患者原发肿瘤部位和转移灶的年龄依赖性。

Age dependency of primary tumor sites and metastases in patients with Ewing sarcoma.

机构信息

Department of Pediatric Hematology/Oncology, University Hospital Muenster, Westfalian Wilhelms University Muenster, Münster, Germany.

University Hospital Essen, Pediatrics III, Sarcoma Centre, West German Cancer, Essen, Germany.

出版信息

Pediatr Blood Cancer. 2018 Sep;65(9):e27251. doi: 10.1002/pbc.27251. Epub 2018 Jun 1.

Abstract

BACKGROUND

The median age of patients with Ewing sarcoma (EwS) at diagnosis is around 14-15 years. Older age is associated with a worse outcome. The correlation of age at diagnosis on sites of disease has not been fully described.

OBJECTIVE

The goal of this study was to evaluate the differences in sites of primary tumor and metastatic tumor involvement according to age groups.

DESIGN/METHOD: EwS data from the Gesellschaft für Pädiatrische Onkologie und Hämatology (GPOH) database of the Cooperative Ewing Sarcoma Study (CESS) 81/86 and the European Intergroup Cooperative Ewing's Sarcoma Study EICESS 92 and the EUROpean Ewing tumor Working Initiative of National Groups-99-Protocol (EURO-E.W.I.N.G.-99) study were analyzed. Patient and tumor characteristics were evaluated statistically using chi square tests.

RESULTS

The study population included 2,635 patients with bone EwS. Sites of primary and metastatic tumors differed according to the age groups of young children (0-9 years), early adolescence (10-14 years), late adolescence (15-19 years), young adults (20-24 years), and adults (more than 24 years). Young children demonstrated the most striking differences in site of disease with a lower proportion of pelvic primary and axial tumors. They presented less often with metastatic disease at diagnosis.

CONCLUSIONS

Site of primary and metastatic tumor involvement in EwS differs according to patient age. The biological and developmental etiology for these differences requires further investigations.

摘要

背景

尤因肉瘤(EwS)患者的中位诊断年龄约为 14-15 岁。年龄较大与预后较差相关。诊断时疾病部位与年龄的相关性尚未完全描述。

目的

本研究旨在评估根据年龄组,肿瘤原发部位和转移瘤受累部位的差异。

设计/方法:分析了 Gesellschaft für Pädiatrische Onkologie und Hämatologie(GPOH)数据库中来自 Cooperative Ewing Sarcoma Study(CESS)81/86 以及 European Intergroup Cooperative Ewing's Sarcoma Study EICESS 92 和 EUROpean Ewing tumor Working Initiative of National Groups-99-Protocol(EURO-E.W.I.N.G.-99)的EwS 数据。使用卡方检验对患者和肿瘤特征进行统计学评估。

结果

本研究人群包括 2635 例骨 EwS 患者。原发和转移瘤的部位因儿童(0-9 岁)、青少年早期(10-14 岁)、青少年晚期(15-19 岁)、青年成年人(20-24 岁)和成年患者(24 岁以上)的年龄组而异。儿童的疾病部位差异最为显著,骨盆原发和轴性肿瘤的比例较低。他们在诊断时转移疾病的发生率较低。

结论

EwS 患者的肿瘤原发部位和转移瘤受累部位与患者年龄有关。这些差异的生物学和发育病因需要进一步研究。

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