Factors associated with tumor volume and primary metastases in Ewing tumors:: Results from the (EI)CESS studies

Factors associated with tumor volume and primary metastases in Ewing tumors:: Results from the (EI)CESS studies
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DOI:
10.1023/a:1008357018737
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发表时间:
1999-09-01
期刊:
影响因子:
50.5
通讯作者:
Jürgens, H
Jürgens, H
中科院分区:
医学1区
文献类型:
--
作者:
Hense, HW;Ahrens, S;Jürgens, H

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背景资料:肿瘤体积超过100 ml和原发性转移的存在已被确定为尤文瘤患者预后不良的决定因素。我们试图评估流行的临界肿瘤大小和原发性转移的患者在一个大的国家样本的首次诊断的时间,并确定与他们的occurrence.Patients相关的因素:本报告是基于数据的945名德国患者谁被纳入(EI)1980年和1997年之间的CESS治疗研究。据推测,自1985年左右以来,德国15岁以下尤文瘤患者的登记几乎完成。原发性肿瘤的诊断仅通过活检确定。分析仅限于与尤文瘤的骨患者,由于发生在软组织。方法:肿瘤体积数据进行评估,通过X线摄影,计算机断层扫描或核磁共振成像为821例。936例原发性转移瘤的诊断基于胸部计算机断层扫描或全身骨扫描。可疑病变必须通过骨髓活检证实。我们探讨了如何年的首次诊断,首次诊断年龄,性别,组织学亚型和原发肿瘤的部位相关的肿瘤大小和存在的转移由单变量和多变量statistical techniques.Results:68%的患者(n = 559)有一个体积超过100毫升与较小的肿瘤更常见的儿童比在青春期后期和成年早期。股骨和骨盆肿瘤体积较大者占90%,其他部位肿瘤体积较小(P < 0.001)。平均而言,所有患者中有26%出现临床明显的原发性转移。1991年以后确诊的肺癌转移灶检出率明显高于1991年以后确诊的肺癌(P < 0.001)。原发性转移在盆腔肿瘤和外周神经外胚层肿瘤中也更常见(PNET; P < 0.01)。大于100 ml的肿瘤与转移性疾病呈正相关(P < 0.001)。多变量分析,同时包括所有单变量预测因素的逻辑回归模型,表明大多数观察到的关联基本上是无混杂的。肿瘤体积大于或等于100 ml的校正比值比(OR)为:年龄每增加10岁,OR = 1.5,骨盆OR = 5.8,股骨OR = 7.1(均P < 0.001)。转移灶的存在与诊断年份显著相关(OR = 1.9,1991年后vs. 1986年前),骨盆为原发肿瘤部位(OR = 1.8),PNET(OR = 1.5),肿瘤大小≥ 100 ml结论:总的来说,我们发现在诊断为尤文瘤的患者中,不利预后的既定因素的患病率高得令人不安。影像学技术的最新进展似乎是1991年后转移瘤检出率上升的主要原因。我们确定年龄,特别是骨盆和股骨部位作为局部肿瘤扩展的主要决定因素。原发转移的发生与肿瘤大小、盆腔部位和PNET独立相关。
Background: Tumor volumes of more than 100 ml and the presence of primary metastases have been identified as determinants of poor prognosis in patients with Ewing tumors. We sought to assess the prevalence of critical tumor size and primary metastases in a large national sample of patients at the time of first diagnosis and to identify factors that are associated with their occurrence.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 registration of German patients with Ewing tumors under the age of 15 years was almost complete since around 1985. Diagnoses of primary tumors were ascertained exclusively by biopsies. Analyses were restricted to patients with Ewing tumors of bone due to the few occurrences in soft tissues.Methods: Tumor volume data as assessed by radiography, computed tomography or nuclear magnetic imaging were available for 821 patients. The diagnosis of primary metastases was based on thoracic computed tomography or on whole body bone scans in 936 patients. Suspicious lesions had to be confirmed by bone marrow biopsies. We explored how year of first diagnosis, age at first diagnosis, sex, histological subtype and site of the primary tumor related to tumor size and presence of metastases by univariate and multivariate statistical techniques.Results: Sixty-eight percent of the patients (n = 559) had a volume above 100 ml with smaller tumors being more common in childhood than in late adolescence and early adulthood. Extensive volumes were observed in almost 90% of the tumors located in femur and pelvis while they were less common in other sites (P < 0.001). On average, 26% of all patients presented with clinically apparent primary metastases. The detection rate of metastases was markedly higher in patients diagnosed after 1991 (P < 0.001). Primary metastases were also significantly more common for tumors originating in the pelvis and for peripheral neuroectodermal tumors (PNET; P < 0.01). Tumors greater than 100 ml were positively associated with metastatic disease (P < 0.001). Multivariate analyses, which included simultaneously all univariate predictors in a logistic regression model, indicated that most of the observed associations were essentially unconfounded. The adjusted odds ratios (OR) for the presence of tumor volumes greater than or equal to 100 ml were OR = 1.5 per age rise of 10 years, and OR = 5.8 for pelvis and OR = 7.1 for femur as primary tumor site (all P < 0.001). The presence of metastases was significantly associated with the year of diagnosis (OR = 1.9, after 1991 vs. before 1986), pelvis as site of the primary tumor (OR = 1.8), a PNET (OR = 1.5), and tumor size greater than or equal to 100 ml (OR = 1.6).Conclusions: In conclusion, we find that the prevalence of established factors for an unfavorable prognosis is disturbingly high among patients diagnosed with Ewing tumors. Recent progress in imaging techniques seems to account for much of the rise in the detection rate of metastases after 1991. We identify age and, in particular, pelvic and femoral site as the major determinants of local tumor extension. Occurrence of primary metastases is independently related to tumor size, pelvic site, and PNET.