Ewing’s Sarcoma

尤因肉瘤
  • 文章类型: Journal Article
    这项研究旨在评估生存结果,预后因素,骨肉瘤和尤因肉瘤化疗后的不良事件。这项回顾性观察研究收集了2008年至2019年接受化疗的骨肉瘤或尤因肉瘤患者的资料。采用灵活的参数生存模型来探讨调整后的生存概率和预后因素。共纳入102例患者(骨肉瘤79例,尤因肉瘤23例)。骨肉瘤患者可切除疾病的估计5年无病生存期(DFS)和5年总生存期(OS)概率分别为60.9%和63.3%。尤因肉瘤占54.4%和88.3%,分别,而不可切除/转移性疾病患者的5年DFS和5年OS仍低于25%.骨肉瘤的两个预后因素包括对新辅助化疗的反应和女性。年龄在25岁及以上的尤因肉瘤患者与较差的生存结果显著相关。在181个化疗周期中,常见的自我报告不良症状包括肿瘤疼痛(n=32,17.7%),发烧(n=21,11.6%),和疲劳(n=16,8.8%),而常见的III级不良事件包括发热性中性粒细胞减少症(n=13,7.3%)和中性粒细胞减少症(n=9,5.1%).没有化疗相关的死亡(V级)或过敏反应事件。
    This study aimed to assess survival outcomes, prognostic factors, and adverse events following chemotherapy treatment for osteosarcoma and Ewing\'s sarcoma. This retrospective observational study was conducted to collect the data of the patients with osteosarcoma or Ewing\'s sarcoma who received chemotherapy treatment between 2008 and 2019. The flexible parametric survival model was performed to explore the adjusted survival probability and the prognostic factors. A total of 102 patients (79 with osteosarcoma and 23 with Ewing\'s sarcoma) were included. The estimated 5-year disease-free survival (DFS) and 5-year overall survival (OS) probabilities in patients with resectable disease were 60.9% and 63.3% for osteosarcoma, and 54.4% and 88.3% for Ewing\'s sarcoma, respectively, whereas the 5-year DFS and 5-year OS for those with unresectable/metastatic disease remained below 25%. Two prognostic factors for osteosarcoma included a response to neoadjuvant chemotherapy and female gender. Ewing\'s sarcoma patients aged 25 years and older were significantly associated with poorer survival outcomes. Of 181 chemotherapy treatment cycles, common self-reported adverse symptoms included tumor pain (n = 32, 17.7%), fever (n = 21, 11.6%), and fatigue (n = 16, 8.8%), while common grade III adverse events included febrile neutropenia (n = 13, 7.3%) and neutropenia (n = 9, 5.1%). There was no chemotherapy-related mortality (grade V) or anaphylaxis events.
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  • 文章类型: Journal Article
    背景:病理/发炎的细胞微环境状态是任何癌症类型的额外风险因素。已经分析了慢性炎症状态在大多数弥漫性肿瘤中的重要性,除了Ewing的肉瘤.这是一种高度恶性的蓝色圆形细胞肿瘤,90%的病例发生在5至25岁之间的患者中。全球,每年1,000,000名儿童中有2.9名受到这种恶性肿瘤的影响。这项回顾性研究的目的是分析C反应蛋白(CRP)作为尤文氏肉瘤的预后因素的作用。
    方法:这项在KlinikumrechtsderIsar进行的回顾性研究包括2004年至2019年期间治疗的82例确诊尤因肉瘤患者。术前CRP测定以mg/dL为单位进行评估,正常值低于0.5mg/dL。无疾病生存时间计算为初始诊断和诸如局部复发或转移的事件之间的时间。随访状态被描述为疾病死亡(DOD),没有疾病的证据(NED)或活着的疾病(AWD)。这项研究的排除标准包括实验室值不足和缺乏有关随访状态或非肿瘤切除的信息。
    结果:预后较差(DOD)的患者和存在远处转移的患者的血清CRP水平存在显着差异(分别为p=0.0016和p=0.009),而局部复发患者的CRP水平无显著差异(p=0.02).预测预后的最佳断点为0.5mg/dL,灵敏度为0.76,特异性为0.74(AUC0.81)。单变量CRP分析水平>0.5mg/dL显示风险比为9.5(95%CI3.5-25.5)。
    结论:在尤因肉瘤病例中,我们认为CRP预处理值>0.5mg/dL是远处转移和预后不良的敏感预后危险因素。需要使用更多数据进行进一步研究,以确定更敏感的截止水平。
    Background: A pathological/inflamed cellular microenvironment state is an additional risk factor for any cancer type. The importance of a chronic inflammation state in most diffuse types of tumour has already been analysed, except for in Ewing’s sarcoma. It is a highly malignant blue round cell tumour, with 90% of cases occurring in patients aged between 5 and 25 years. Worldwide, 2.9 out of 1,000,000 children per year are affected by this malignancy. The aim of this retrospective study was to analyse the role of C-reactive protein (CRP) as a prognostic factor for Ewing’s sarcomas. Methods: This retrospective study at Klinikum rechts der Isar included 82 patients with a confirmed Ewing’s sarcoma diagnosis treated between 2004 and 2019. Preoperative CRP determination was assessed in mg/dL with a normal value established as below 0.5 mg/dL. Disease-free survival time was calculated as the time between the initial diagnosis and an event such as local recurrence or metastasis. Follow-up status was described as death of disease (DOD), no evidence of disease (NED) or alive with disease (AWD). The exclusion criteria of this study included insufficient laboratory values and a lack of information regarding the follow-up status or non-oncological resection. Results: Serum CRP levels were significantly different in patients with a poorer prognosis (DOD) and in patients who presented distant metastasis (p = 0.0016 and p = 0.009, respectively), whereas CRP levels were not significantly different in patients with local recurrence (p = 0.02). The optimal breakpoint that predicted prognosis was 0.5 mg/dL, with a sensitivity of 0.76 and a specificity of 0.74 (AUC 0.81). Univariate CRP analysis level >0.5 mg/dL revealed a hazard ratio of 9.5 (95% CI 3.5−25.5). Conclusions: In Ewing’s sarcoma cases, we consider a CRP pretreatment value >0.5 mg/dL as a sensitive prognostic risk factor indication for distant metastasis and poor prognosis. Further research with more data is required to determine more sensitive cutoff levels.
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  • 文章类型: Journal Article
    旋转成形术是一种已建立的技术,对于某些患有膝关节周围原发性骨肿瘤并接受肿瘤切除的患者,它是手术重建的一部分。外科医生应用旋转成形术以及可能是该手术候选人的患者对手术的接受程度存在很大差异。我们通过使用半结构化访谈对4名患者及其家人进行访谈,定性研究了接受旋转成形术的患者家属的决策过程。专题分析确定了以下在决策过程中很重要的主题:(1)对良好信息来源的渴望,(2)发现与其他面临类似决定的患者会面的价值,(3)将功能优先于宇宙,(4)希望限制修改手术的需要,(5)接受恢复正常不是手术的选择。面对类似决定的医生和患者可以从对该过程的更好理解中受益。以及他们可能经历的焦虑和担忧的正常化。
    Rotationplasty is an established technique that is indicated as part of the surgical reconstruction for certain patients with primary bone tumors around the knee who undergo tumor resection. There is considerable variation in the application of rotationplasty by surgeons as well as acceptance of the procedure by patients who may be candidates for this procedure. We qualitatively studied the decision-making process of families of patients who had undergone rotationplasty by interviewing 4 patients and their families using semi-structured interviews. Thematic analysis identified the following themes that were important in the decision-making process: (1) the desire for good information sources, (2) finding value in meeting with other patients who had been faced with a similar decision, (3) prioritizing function over cosmesis, (4) a desire to limit the need for revision surgeries, and (5) accepting that a return to normalcy is not an option with a surgery. Physicians and patients faced with a similar decision can benefit from a better understanding of the process, and by the normalization of anxieties and concerns that they may experience.
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  • 文章类型: Journal Article
    UNASSIGNED: Ewing\'s sarcoma is a disease of children and young adults and occurs most often in bone and soft tissues. The intracranial and spinal manifestation of the disease is rare and reported incidence is 1%-6%.
    UNASSIGNED: We conducted this study to determine the surgical outcome of children with skull and spine Ewing\'s sarcoma (SSES).
    UNASSIGNED: This is a prospective analysis of 13 patients of SSES who reported to the Department of Neurosurgery, Nizam\'s Institute of Medical Sciences, Hyderabad, Telangana, India, between 2014 and 2016. All cases after detailed examination, magnetic resonance imaging, and computed tomography scan were subjected to surgery followed by adjuvant therapy comprising chemotherapy and radiotherapy. Outcome was analyzed at 6 months as well at latest follow-up. Neurological function, local recurrence, primary or secondary nature of the disease, distant relapse, and treatment-related complications were analyzed in this study.
    UNASSIGNED: There were eight female and five male patients with a mean age of 12 years (ranging from 4 to 8 years). Pain was the common presenting feature in all cases. Focal neurological deficits corresponding to the anatomical location was seen in six patients. These 13 cases were distributed anatomically as four cases involving the cloves, two cases with occipital bone and lobe involvement, one case of parietal bone and lobe involvement, and six cases of spinal involvement. Surgery was performed in all cases where gross total excision (Ozge C, Calikoglu M, Cinel L, Apaydin FD, Ozgür ES. Massive pleural effusion in an 18-year-old girl with Ewing sarcoma. Can Respir J 2004;11:363-5), near-total excision, and subtotal excision was achieved in these 13 cases (Steinbok P, Flodmark O, Norman MG, Chan KW, Fryer CJ. Primary Ewing\'s sarcoma of the base of the skull. Neurosurgery 1986;19:104-7). Subsequently all cases underwent multiagent chemoradiotherapy. Postsurgery pain subsided in 12 (92%) of patients. Ten patients maintained or improved motor function. In seven cranial cases and in six spinal cases, four cases showed improvement whereas three (23%) had deterioration of motor function.
    UNASSIGNED: Surgical outcome of SSES in short-term follow-up is good with current recommended management regimen of maximum excision followed by chemo and radiotherapy. However, metastasis is not uncommon.
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  • 文章类型: Journal Article
    Alterations in DNA methylation patterns are a hallmark of malignancy. However, the majority of epigenetic studies of Ewing\'s sarcoma have focused on the analysis of only a few candidate genes. Comprehensive studies are thus lacking and are required. The aim of the present study was to identify novel methylation markers in Ewing\'s sarcoma using microarray analysis. The current study reports the microarray-based DNA methylation study of 1,505 CpG sites of 807 cancer-related genes from 69 Ewing\'s sarcoma samples. The Illumina GoldenGate Methylation Cancer Panel I microarray was used, and with the appropriate controls (n=14), a total of 92 hypermethylated genes were identified in the Ewing\'s sarcoma samples. The majority of the hypermethylated genes were associated with cell adhesion, cell regulation, development and signal transduction. The overall methylation mean values were compared between patients who survived and those that did not. The overall methylation mean was significantly higher in the patients who did not survive (0.25±0.03) than in those who did (0.22±0.05) (P=0.0322). However, the overall methylation mean was not found to significantly correlate with age, gender or tumor location. GDF10, OSM, APC and HOXA11 were the most significant differentially-methylated genes, however, their methylation levels were not found to significantly correlate with the survival rate. The DNA methylation profile of Ewing\'s sarcoma was characterized and 92 genes that were significantly hypermethylated were detected. A trend towards a more aggressive behavior was identified in the methylated group. The results of this study indicated that methylation may be significant in the development of Ewing\'s sarcoma.
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