Ewing’s Sarcoma

尤因肉瘤
  • 文章类型: Case Reports
    胸壁重建带来了重大挑战。这些挑战之一是选择正确的重建材料。关于使用假体材料与自体组织以及刚性与非刚性材料存在争议。本文展示了阔筋膜在儿童胸壁重建中的新颖用途。
    Chest wall reconstruction poses significant challenges. One of those challenges is choosing the correct material for reconstruction. There is debate on using prosthetic materials versus autologous tissues and rigid versus nonrigid materials. This article showcases the novel use of fascia lata for chest wall reconstruction in children.
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  • 文章类型: Case Reports
    原始神经外胚层肿瘤(PNETs)很少见,由原始神经细胞引起的恶性肿瘤。胸壁的PNET很少见,在儿童和年轻人中观察到。Askin将Askin的肿瘤定义为胸肺区域的PNET。它从胸壁的软组织发展而来,特别是在椎旁区域。这里,我们报告一例Askin肿瘤,发生在一名13岁女孩胸肺区域的罕见肿瘤。她带着发烧的抱怨来了,冷,咳嗽伴有粘液痰,15天气喘吁吁,和三个月的全身无力。还进行了高分辨率计算机断层扫描(HRCT)胸部扫描,提示右侧胸膜基肿块伴椎体转移。通过包括影像学研究和组织病理学检查的勤奋诊断评估,肿瘤被准确识别。
    Primitive neuroectodermal tumours (PNETs) are rare, malignant tumours arising from primitive nerve cells. PNET of the chest wall is rare and is observed in children and young adults. Askin defined Askin\'s tumour as a PNET of the thoracopulmonary area. It develops from the soft tissues of the chest wall, particularly in the paravertebral region. Here, we report a case of Askin\'s tumour, a rare neoplasm occurring in the thoracopulmonary region in a 13-year-old girl. She came with complaints of fever, cold, cough with mucoid expectoration, breathlessness for 15 days, and generalized weakness for three months. A high-resolution computed tomography (HRCT) thorax scan was also done, which suggested a large right pleural-based mass with vertebral metastasis. Through diligent diagnostic evaluation involving imaging studies and histopathological examination, the tumour was accurately identified.
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  • 文章类型: Case Reports
    背景:尤因肉瘤是主要在儿童和青少年中观察到的原发性骨肿瘤,需要多学科治疗方法。虽然局部病例的5年生存率为60-70%,盆腔晚期转移患者的预后明显较差。此外,骨盆尤因肉瘤具有导致术后感染率高的独特问题。
    方法:我们介绍了一个日本14岁男孩,患有左髂尤因肉瘤和多发转移的病例。在初次访问时,影像学检查显示,左髂骨有一个大肿瘤,骨外延伸并转移到多个部位。新辅助化疗导致肿瘤显著减少。在不进行骨盆环重建的情况下进行了手术切除,以使术后早期化疗并最大程度地减少术后感染风险。尽管外展肌完全切除,患者通过使负载轴居中,实现了术后稳定的步态。
    结论:我们的病例强调了左髂尤因肉瘤伴多发转移的成功治疗,专注于功能保存和降低感染风险。未进行骨盆环重建术以避免术后并发症,强调术后早期化疗的重要性。患者术后步态稳定,证明了这种方法在类似情况下的潜在好处。
    BACKGROUND: Ewing\'s sarcoma is a primary bone tumor predominantly observed in children and adolescents, necessitating a multidisciplinary treatment approach. While localized cases have a 5-year survival rate of 60-70%, the prognosis is significantly worse in pelvic advanced cases with metastasis. Moreover, pelvic Ewing\'s sarcoma has the unique problem of leading to high rates of postoperative infection.
    METHODS: We present the case of a Japanese 14-year-old boy with left iliac Ewing\'s sarcoma and multiple metastases. At the initial visit, imaging revealed a large tumor in the left iliac bone with extraosseous extension and metastasis to multiple sites. Neoadjuvant chemotherapy resulted in significant tumor reduction. Surgical resection was performed without pelvic ring reconstruction to enable early postoperative chemotherapy and minimize postoperative infection risk. Despite complete abductor muscle removal, the patient achieved a stable gait postoperatively by centering the load axis.
    CONCLUSIONS: Our case highlights the successful management of a left iliac Ewing\'s sarcoma with multiple metastases, with a focus on functional preservation and infection risk reduction. Pelvic ring reconstruction was not performed to avoid postoperative complications, emphasizing the importance of early postoperative chemotherapy. The patient achieved a stable gait postoperatively, demonstrating the potential benefits of this approach in similar cases.
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  • 文章类型: Journal Article
    角化是铜诱导的细胞死亡。铜代谢相关基因(CMRGs)被证明可用于评估肿瘤的预后。在研究中,检测CMRG对尤文氏肉瘤(ES)中TME细胞浸润的影响。
    GEO和ICGC数据库提供了mRNA表达谱和临床特征以供下载。在GSE17674数据集中,通过单变量回归分析鉴定了22个预后相关的铜代谢相关基因(PR-CMRGs)。随后,为了比较这些PR-CMRGs高表达和低表达组的生存率,实施Kaplan-Meier分析。此外,检查了它们之间的相关性。该研究采用功能富集分析来调查可能的潜在途径,而GSVA用于评估ES(表达集)中的富集途径。通过无监督聚类算法,样本被分为两个集群,揭示了存活率和免疫浸润水平的显著差异。
    使用Lasso和逐步回归方法,五个基因(TFRC,SORD,选择SLC11A2、FKBP4和AANAT)作为风险特征。根据Kaplan-Meier生存分析,高危组的生存率明显低于低危组(p=6.013e-09).受试者工作特征(ROC)曲线的曲线下面积(AUC)值分别为1年、3年和5年的0.876、0.883和0.979,分别。在其他数据集中进一步验证了风险模型,即GSE63155、GSE63156和ICGC数据集。为了帮助预测结果,建立了纳入风险水平和临床特征的列线图.此列线图的性能通过校准曲线得到有效验证。此外,该研究评估了不同风险组之间免疫浸润的差异,以及高表达和低表达组。重要的是,确定了几种显示敏感性的药物,为ES提供潜在的治疗选择。
    上述发现强烈表明,CMRGs在预测ES的预后和免疫状态中起着至关重要的作用。
    UNASSIGNED: Cuproptosis is copper-induced cell death. Copper metabolism related genes (CMRGs) were demonstrated that used to assess the prognosis out of tumors. In the study, CMRGs were tested for their effect on TME cell infiltration in Ewing\'s sarcoma (ES).
    UNASSIGNED: The GEO and ICGC databases provided the mRNA expression profiles and clinical features for downloading. In the GSE17674 dataset, 22prognostic-related copper metabolism related genes (PR-CMRGs) was identified by using univariate regression analysis. Subsequently, in order to compare the survival rates of groups with high and low expression of these PR-CMRGs,Kaplan-Meier analysis was implemented. Additionally, correlations among them were examined. The study employed functional enrichment analysis to investigate probable underlying pathways, while GSVA was applied to evaluate enriched pathways in the ES (Expression Set). Through an unsupervised clustering algorithm, samples were classified into two clusters, revealing significant differences in survival rates and levels of immune infiltration.
    UNASSIGNED: Using Lasso and step regression methods, five genes (TFRC, SORD, SLC11A2, FKBP4, and AANAT) were selected as risk signatures. According to the Kaplan-Meier survival analysis, the high-risk group had considerably lower survival rates than the low-risk group(p=6.013e-09). The area under the curve (AUC) values for the receiver operating characteristic (ROC) curve were 0.876, 0.883, and 0.979 for 1, 3, and 5 years, respectively. The risk model was further validated in additional datasets, namely GSE63155, GSE63156, and the ICGC datasets. To aid in outcome prediction, a nomogram was developed that incorporated risk levels and clinical features. This nomogram\'s performance was effectively validated through calibration curves.Additionally, the study evaluated the variations in immune infiltration across different risk groups, as well as high-expression and low-expression groups. Importantly, several drugs were identified that displayed sensitivity, offering potential therapeutic options for ES.
    UNASSIGNED: The findings above strongly indicate that CMRGs play crucial roles in predicting prognosis and immune status in ES.
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  • 文章类型: Journal Article
    尤因肉瘤(EWS)是一种高度侵袭性的恶性骨肿瘤,主要影响青少年和年轻人。尽管在某些情况下放化疗有效,转移性和复发性疾病患者的治愈率仍然很低。因此,迫切需要创新的治疗方法来应对与EWS治疗相关的挑战.表观遗传调控,生理过程中的一个关键因素,在控制细胞增殖中起着重要作用,保持基因完整性,和调节转录。最近的研究强调了异常表观遗传调控在EWS的启动和进展中的重要性。全面了解EWS与异常表观遗传调控之间的复杂相互作用对于推进临床药物开发至关重要。这篇综述旨在全面概述EWS中涉及的两个表观遗传靶标,整合各种治疗方式,为EWS的临床诊断和治疗提供创新的观点。
    Ewing\'s sarcoma (EWS) is a highly aggressive malignant bone tumor primarily affecting adolescents and young adults. Despite the efficacy of chemoradiotherapy in some cases, the cure rate for patients with metastatic and recurrent disease remains low. Therefore, there is an urgent need for innovative therapeutic approaches to address the challenges associated with EWS treatment. Epigenetic regulation, a crucial factor in physiological processes, plays a significant role in controlling cell proliferation, maintaining gene integrity, and regulating transcription. Recent studies highlight the importance of abnormal epigenetic regulation in the initiation and progression of EWS. A comprehensive understanding of the intricate interactions between EWS and aberrant epigenetic regulation is essential for advancing clinical drug development. This review aims to provide a comprehensive overview of both epigenetic targets implicated in EWS, integrating various therapeutic modalities to offer innovative perspectives for the clinical diagnosis and treatment of EWS.
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  • 文章类型: Journal Article
    目的:冷物理血浆(CPP)通过在各种癌细胞中诱导细胞毒性作用,已成为肿瘤学中的有效疗法,包括软骨肉瘤(CS),尤因肉瘤(ES),骨肉瘤(OS)。当前的研究调查了CPP对CS(CAL-78)中细胞运动的影响,ES(A673),和OS(U2-OS)细胞系,专注于肌动蛋白细胞骨架。
    方法:使用CASY细胞计数器和分析仪研究细胞增殖,并确定胎牛血清的最佳浓度,以维持活力而不刺激细胞增殖。CellTiter-BlueCell活力测定用于确定CPP对骨肉瘤细胞活力的影响。使用Radius测定来确定细胞迁移。脱氧核糖核酸酶Ⅰ染色,G-肌动蛋白,和F-肌动蛋白用于测定对细胞骨架的影响。
    结果:在CPP处理后,在所有细胞系中观察到细胞活力和运动性的降低。CPP诱导肌动蛋白细胞骨架的变化,导致细胞运动性下降。
    结论:CPP通过改变肌动蛋白细胞骨架有效降低骨肉瘤细胞的运动性。这些发现强调了CPP作为骨肉瘤治疗工具的潜力,并强调了该领域进一步研究的必要性。
    OBJECTIVE: Cold physical plasma (CPP) has emerged as an effective therapy in oncology by inducing cytotoxic effects in various cancer cells, including chondrosarcoma (CS), Ewing\'s sarcoma (ES), and osteosarcoma (OS). The current study investigated the impact of CPP on cell motility in CS (CAL-78), ES (A673), and OS (U2-OS) cell lines, focusing on the actin cytoskeleton.
    METHODS: The CASY Cell Counter and Analyzer was used to study cell proliferation and determine the optimal concentrations of fetal calf serum to maintain viability without stimulation of cell proliferation. CellTiter-BlueCell viability assay was used to determine the effects of CPP on the viability of bone sarcoma cells. The Radius assay was used to determine cell migration. Staining for Deoxyribonuclease I, G-actin, and F-actin was used to assay for the effects on the cytoskeleton.
    RESULTS: Reductions in cell viability and motility were observed across all cell lines following CPP treatment. CPP induced changes in the actin cytoskeleton, leading to decreased cell motility.
    CONCLUSIONS: CPP effectively reduces the motility of bone sarcoma cells by altering the actin cytoskeleton. These findings underscore CPP\'s potential as a therapeutic tool for bone sarcomas and highlight the need for further research in this area.
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  • 文章类型: Case Reports
    尤因肉瘤是儿童时期的小圆形蓝色细胞肿瘤之一,通常会影响骨骼。最近,未分化的圆形细胞肉瘤的一个亚组已被遗传鉴定为BCOR(B细胞系6辅抑制因子)改变的肉瘤(BAS)。我们介绍了一例6岁的男性儿童,该儿童以呼吸急促和呼吸急促为主要主诉,并被发现有与肉瘤有关的纵隔肿块。初步活检结果为小圆形蓝色细胞阳性,可能是尤因肉瘤.经过六个周期的化疗,纵隔肿块随后收缩,患者能够进行楔形切除和切除肿块,同时整块切除第五和第六肋骨,保护他的右肺.最终组织病理为BAS阳性。仅有4例报告的胸壁BAS病例和零例报告的肺原发肿瘤表现病例,使这成为这种疾病的罕见表现。
    Ewing sarcoma is one of the small round blue cell tumors of childhood that typically affects bone. Recently, a subgroup of undifferentiated round-cell sarcomas has been genetically identified as BCOR (B-cell Line 6 Corepressor)-altered sarcomas (BAS). We present a case of a six-year-old male child who presented with a chief complaint of shortness of breath and tachypnea and was found to have a large mediastinal mass concerning sarcoma. Preliminary biopsy results were positive for small round blue cells, possibly Ewing sarcoma. After six cycles of chemotherapy, with subsequent shrinkage of mediastinal mass, the patient was able to undergo wedge resection and excision of the mass with en bloc resection of the fifth and sixth rib, preserving his right lung. Final tissue pathology was positive for BAS. There have been only four reported cases of BAS of the chest wall and zero reported cases of primary tumor presentation of the lung, making this a rare presentation of the disease.
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  • 文章类型: Case Reports
    尤因肉瘤是儿童和青少年第二常见的原发性恶性骨肿瘤,骨肉瘤后。这是一种罕见的肿瘤,轴向骨架是发展的首选部位,接着是长骨。诊断通过影像学诱发并通过组织学证实。治疗基于强化化疗和可手术形式的局部手术治疗,在某些情况下,放射治疗。文献中只有少数零星病例描述腓骨远端定位。
    我们报告了一个7岁儿童的病例,该儿童在左脚踝的外侧出现疼痛,没有局部炎症迹象。放射学发现显示左腓骨的干phy端-干phy端溶骨组织过程,组织学得出结论是尤因肉瘤.我们进行了新辅助综合化疗,然后用胫骨前外棒节段性切除腓骨远端,再加上胫骨,和无放射治疗的距骨关节固定术。
    尤因肉瘤的管理在不断发展。它的腓骨远端在生长的肢体中的位置使其变得更加困难。它必须是个性化的,多学科,并在专业中心进行。
    UNASSIGNED: Ewing\'s sarcoma is the second most common primary malignant bone tumor in children and adolescents, after osteosarcoma. It is a rare tumor, with the axial skeleton being the preferred site of development, followed by the long bones. Diagnosis is evoked by imaging and confirmed by histology. Treatment is based on intensive chemotherapy with local surgical treatment in operable forms, and in some cases, radiotherapy. There are only a few sporadic cases in the literature describing distal fibular localization.
    UNASSIGNED: We report the case of a 7-year-old child who presented with pain on the lateral aspect of the left ankle without local inflammatory signs. Radiological findings revealed a metaphyseal-diaphyseal osteolytic tissue process of the left fibula, and histology concluded that it was Ewing\'s sarcoma. We proceeded with neoadjuvant polychemotherapy followed by segmental resection of the distal fibula with an anteroexternal tibial rod, plus tibiotalar, and talocalcaneal arthrodesis without recourse to radiotherapy.
    UNASSIGNED: The management of Ewing\'s sarcoma is constantly evolving. Its distal fibular location in a growing limb makes it even more difficult. It must be personalized, multidisciplinary, and carried out in specialized centers.
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  • 文章类型: Case Reports
    尤因氏肾肉瘤是一种罕见的肿瘤。尽管已知肾癌累及下静脉,肿瘤延伸至右心房并不常见。在大多数情况下,当肿瘤延伸到下腔静脉的肝下部分时,它可以从腹部方法中取出。很少有患者需要使用体外循环来切除下腔静脉和右心房中的肿瘤。需要切除肾肿瘤和右心房肿块的患者的管理更为复杂,需要由肿瘤外科医生组成的团队方法,心脏外科医生,还有心脏麻醉师.切除右心房肿块的肾肿瘤通常同时进行。在这种情况下,需要修改体外循环插管策略。
    Ewing\'s sarcoma of the kidney is a rare tumor. Although renal carcinomas are known to involve the inferior cava, extension of the tumor up to the right atrium is not common. In the majority of cases when the tumor extends into the infrahepatic part of the inferior vena cava, it can be removed from the abdominal approach. Few patients require the use of cardiopulmonary bypass for removal of the tumor in the inferior vena cava and right atrium. The management of patients requiring resection of kidney tumors and right atrial mass is more complicated and requires a team approach consisting of oncosurgeons, cardiac surgeons, and cardiac anesthetists. The resection of the kidney tumor with a mass in the right atrium is usually done concomitantly. The cardiopulmonary bypass cannulation strategy needs to be modified in such cases.
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  • 文章类型: Case Reports
    尤因肉瘤是一种罕见且高度侵袭性的骨肿瘤,主要影响儿童和青少年。它通常存在于骨盆和轴向骨骼中,由于其复杂的解剖位置,骶骨受累带来了独特的挑战。这份报告详细介绍了一名18岁男性的骶骨尤因肉瘤,强调诊断,外科,和管理的重建方面。病人出现下背部疼痛,下肢无力,和尿失禁,这促使了广泛的诊断评估。磁共振成像和计算机断层扫描显示,从S2椎骨延伸到尾骨的大量溶解肿块侵犯了s前空间。活检证实了尤因肉瘤的诊断,以EWS-FLI11型易位为特征。由神经外科医生组成的多学科团队,结直肠外科医生,和整形外科医生制定。在肿瘤的整块切除中,腰骨盆固定术,使用双侧臀大肌推进皮瓣成功进行软组织重建。该程序旨在解决患者病情的肿瘤和功能方面。化疗和放疗作为辅助治疗。在2年的随访中,患者独立行走,影像学检查无残留肿瘤.该病例突出了骶骨尤因肉瘤的复杂性,并强调了多学科方法的重要性。所描述的手术技术,包括创新使用臀大肌推进皮瓣进行软组织重建,有助于减少伤口并发症和促进成功的患者预后。所提出的方法是对这种具有挑战性的恶性肿瘤的治疗方案的宝贵补充。
    Ewing\'s sarcoma is a rare and highly aggressive bone tumor primarily affecting children and adolescents. It commonly presents in the pelvic and axial skeleton, with sacral involvement posing unique challenges due to its intricate anatomical location. This report details the case of an 18-year-old male with sacral Ewing\'s sarcoma, emphasizing the diagnostic, surgical, and reconstructive aspects of management. The patient presented with lower back pain, lower limb weakness, and urinary incontinence, which prompted an extensive diagnostic evaluation. Magnetic resonance imaging and computed tomography scans revealed a large lytic mass extending from the S2 vertebra to the coccyx invading the presacral space. Biopsy confirmed the diagnosis of Ewing\'s sarcoma, characterized by the EWS-FLI1 type 1 translocation. A multidisciplinary team comprising neurosurgeons, colorectal surgeons, and plastic surgeons was formulated. En bloc resection of the tumor, lumbopelvic fixation, and soft-tissue reconstruction using bilateral gluteus maximus advancement flaps were successfully performed. The procedure aimed to address both the oncological and functional aspects of the patient\'s condition. Chemotherapy and radiotherapy were administered as adjuvant therapies. At 2-year follow-up, the patient was ambulating independently with no residual tumor on imaging. This case highlights the complex nature of sacral Ewing\'s sarcoma and underscores the importance of a multidisciplinary approach. The described surgical technique, including the innovative use of gluteus maximus advancement flaps for soft-tissue reconstruction, contributes to reducing wound complications and promoting successful patient outcomes. The presented approach serves as a valuable addition to the armamentarium of treatment options for this challenging malignancy.
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