Soft tissue neoplasms

软组织肿瘤
  • 文章类型: Journal Article
    背景:人工智能(AI)的集成,特别是,在肿瘤学中,显著改变了医学诊断和治疗计划的范式。然而,AI的效用,特别是OpenAI的ChatGPT,在软组织肉瘤治疗中,尚不清楚。
    方法:我们评估了ChatGPT与日本骨科协会(JOA)2020年软组织肿瘤治疗临床实践指南的一致性。制定了22个临床问题(CQ)以涵盖肉瘤诊断的各个方面,治疗,和管理。ChatGPT的响应被分类为“完全对齐,\"\"部分对齐,\"或\"不对齐\"基于建议和证据的强度。
    结果:ChatGPT与JOA指南一致率为86%。AI提供了两个完全对齐的实例和17个部分对齐的实例,表明有很强的能力来匹配大多数问题的指导标准。然而,在非典型脂肪瘤的治疗领域发现了三个差异,滑膜肉瘤的围手术期化疗,老年恶性软组织肿瘤的治疗策略。用指导方针输入重新评估导致了一些调整,揭示AI在复杂肉瘤治疗中的潜力和局限性。
    结论:我们的研究表明,特别是ChatGPT,可以符合软组织肉瘤治疗的临床指南。它还强调了在医疗决策中需要不断改进和谨慎地整合人工智能,特别是在软组织肉瘤的治疗方面。
    BACKGROUND: The integration of artificial intelligence (AI), particularly, in oncology, has significantly shifted the paradigms of medical diagnostics and treatment planning. However, the utility of AI, specifically OpenAI\'s ChatGPT, in soft tissue sarcoma treatment, remains unclear.
    METHODS: We evaluated ChatGPT\'s alignment with the Japanese Orthopaedic Association (JOA) clinical practice guidelines on the management of soft tissue tumors 2020. Twenty-two clinical questions (CQs) were formulated to encompass various aspects of sarcoma diagnosis, treatment, and management. ChatGPT\'s responses were classified into \"Complete Alignment,\" \"Partial Alignment,\" or \"Nonalignment\" based on the recommendation and strength of evidence.
    RESULTS: ChatGPT demonstrated an 86% alignment rate with the JOA guidelines. The AI provided two instances of complete alignment and 17 instances of partial alignment, indicating a strong capability to match guideline criteria for most questions. However, three discrepancies were identified in areas concerning the treatment of atypical lipomatous tumors, perioperative chemotherapy for synovial sarcoma, and treatment strategies for elderly patients with malignant soft tissue tumors. Reassessment with guideline input led to some adjustments, revealing both the potential and limitations of AI in complex sarcoma care.
    CONCLUSIONS: Our study demonstrates that AI, specifically ChatGPT, can align with clinical guidelines for soft tissue sarcoma treatment. It also underscores the need for continuous refinement and cautious integration of AI in medical decision-making, particularly in the context of treatment for soft tissue sarcoma.
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  • 文章类型: Journal Article
    背景:根据软组织肉瘤(STS)的NCCN指南,四肢STS(ESTS)的治疗包括放射治疗(RT)和手术切除高度和>5cm的肿瘤。这项研究的目的是描述邻里社会经济地位(NSES)之间的关联,符合NCCN指南建议,和ESTS患者的结局。
    方法:在SEER登记处确定了2006年至2018年诊断为ESTS的患者。分析队列仅限于接受保肢手术的高级别肿瘤>5cm无淋巴结或远处转移的患者。使用调整回归分析分析与接受RT相关的患者人口统计学和肿瘤特征。使用Kaplan-Meier曲线和调整的加速失败时间模型来检查癌症特异性存活的差异。
    结果:在2,249名患者中,29.0%(n=648)接受新辅助RT,49.7%(n=1,111)接受了辅助治疗或术中放疗,21.3%(n=476)未接受RT。在调整后的分析中,较低的NSES与较低的接受RT的可能性相关(比值比,0.70[95%CI,0.57-0.87];P<.001)。低nSES与较差的癌症特异性生存率相关(风险比,1.19[95%CI,1.01-1.40];P=.04)。在完全调整的模型中,种族和种族不是接受RT或癌症特异性生存的重要预测因素。
    结论:来自较低nSES区域的患者不太可能接受NCCN指南推荐的ESTSRT,并且癌症特异性生存率较差。有必要努力更好地定义和解决ESTS患者治疗和生存方面的差异。
    Based on the NCCN Guidelines for Soft Tissue Sarcoma (STS), treatment of extremity STS (ESTS) includes radiation therapy (RT) and surgical resection for tumors that are high-grade and >5 cm. ​​The aim of this study was to describe the association between neighborhood socioeconomic status (nSES), concordance with NCCN Guidelines recommendations, and outcomes in patients with ESTS.
    Patients with ESTS diagnosed from 2006 through 2018 were identified in SEER registries. The analytic cohort was restricted to patients with high-grade tumors >5 cm without nodal or distant metastases who received limb-sparing surgery. Patient demographics and tumor characteristics associated with receipt of RT were analyzed using adjusted regression analyses. Kaplan-Meier curves and adjusted accelerated failure time models were used to examine disparities in cancer-specific survival.
    Of 2,249 patients, 29.0% (n=648) received neoadjuvant RT, 49.7% (n=1,111) received adjuvant or intraoperative RT, and 21.3% (n=476) did not receive RT. In adjusted analyses, lower nSES was associated with lower likelihood of receiving RT (odds ratio, 0.70 [95% CI, 0.57-0.87]; P<.001). Low nSES was associated with worse cancer-specific survival (hazard ratio, 1.19 [95% CI, 1.01-1.40]; P=.04). Race and ethnicity were not significant predictors of receipt of RT or cancer-specific survival in the fully adjusted models.
    Patients from lower nSES areas were less likely to receive NCCN Guideline-recommended RT for their ESTS and had worse cancer-specific survival. Efforts to better define and resolve disparities in the treatment and survival of patients with ESTS are warranted.
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  • 文章类型: Journal Article
    目的:子宫肉瘤是一种罕见的肿瘤,预后较差。他们的诊断通常是偶然的,手术后。我们的目标是研究子宫肉瘤的早期管理策略,评估指南依从性和专家中心转诊对子宫肉瘤患者治疗方法和临床结局的影响.
    方法:我们回顾性分析了转诊至居里研究所并在法国NETSARC网络数据库中注册的子宫肉瘤患者的医疗记录。
    结果:总计,100名患者,平均年龄为54岁,包括在分析中。MRI扫描(n=36),所有患者至少有两个提示恶性肿瘤的体征,和77.8%有四个或更多的迹象。65.6%的病例未进行术前活检。只有14.1%的患者在专家中心接受了初始手术。在网络外进行的手术与碎裂显着相关(32.9%vs.0%;p=0.036),更少的负利润率(R0利润率52.4%与100%;p=0.006),和对手术指南的依从性差(28.3vs.72.7%;p=0.013)。多变量分析显示,不坚持手术建议与无复发生存率无显著相关(HR=0.54;95%CI[0.21-1.38])。但却是总生存期较差的独立预测因子(HR=0.12;95%CI[0.03-0.52];p=0.005).
    结论:尽管可疑的临床和放射学体征频率很高,大部分接受肉瘤手术的女性在专家网络之外接受治疗。我们提供指导方针,整合临床背景和放射学体征,以鼓励早期转诊到肉瘤参考中心。
    OBJECTIVE: Uterine sarcomas are rare tumors with a poor prognosis. Their diagnosis is often incidental, following surgery. Our goal was to examine the early management strategies for uterine sarcomas, and to assess the impact of guideline adherence and expert center referral on both the management approaches and the clinical outcomes in patients with uterine sarcomas.
    METHODS: We retrospectively analyzed medical records from patients with uterine sarcoma referred to the Institut Curie and registered in the database of the French NETSARC network.
    RESULTS: In total, 100 patients, with a median age of 54 years, were included in the analyses. On MRI scans (n = 36), all patients had at least two signs suggestive of malignancy, and 77.8 % had four or more signs. No preoperative biopsy was performed in 65.6 % of cases. Only 14.1 % of patients underwent initial surgery at an expert center. Surgery performed outside the network was significantly associated with morcellation (32.9 % vs. 0 %; p = 0.036), fewer negative margins (R0 margins 52.4 % vs. 100 %; p = 0.006), and poor adherence to surgical guidelines (28.3 vs. 72.7 %; p = 0.013). Multivariate analysis showed that non-adherence to surgical recommendations was not significantly associated with relapse-free survival (HR = 0.54; 95 % CI [0.21-1.38]), but was an independent predictor of poor overall survival (HR = 0.12; 95 % CI [0.03-0.52]; p = 0.005).
    CONCLUSIONS: Despite a high frequency of suspicious clinical and radiological signs, a large proportion of women undergoing sarcoma surgery are treated outside of expert networks. We provide guidelines, integrating the clinical context and radiological signs to encourage early referral to reference centers for sarcoma.
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  • 文章类型: Journal Article
    目标:早期,准确的诊断对软组织肉瘤患者的预后至关重要。为此,成像算法的标准化,技术要求,因此,报告是一个先决条件。自2015年第一次欧洲肌肉骨骼放射学学会(ESSR)共识以来,技术成就,对特定实体的进一步洞察,修订后的WHO分类(2020年)和AJCC分期系统(2017年)进行了必要的更新。该指南旨在支持放射科医师的决策,并为跨学科肿瘤委员会的讨论做出贡献。
    方法:使用基于同行评审文献的经过验证的Delphi方法,在来自12个欧洲国家的46名专业肌肉骨骼放射科医师小组中达成共识。在两轮迭代中,报表按协议级别(0到10)在线评分。要么“团体共识”,\"\"组协议,实现了\"或\"缺乏协议\"。
    结果:定义了八个部分,最终包含145个带有注释的语句。总的来说,达到95.9%的群体共识,集团协议占4.1%。该通讯包含第一部分,包括可疑软组织肿瘤的成像算法,局部成像方法,以及肿瘤中心的作用。
    结论:超声代表可接近和小肿瘤的初始分诊成像模式。MRI是大多数软组织肿瘤的表征和局部分期的首选方式。CT在特殊情况下显示。在可疑或可能的恶性肿瘤中,应联系专科肿瘤中心,以获得转诊或远程放射学第二意见。这应该在进行活检之前完成,无一例外。
    结论:更新的ESSR软组织肿瘤成像指南旨在为标准化成像提供最佳实践专家共识,支持放射科医生的决策,并提高个体患者和未来个性化策略研究中的检查可比性。
    结论:•超声仍然是可接近的和疑似小的软组织肿瘤的最佳初始分诊成像方式。•在大多数情况下,MRI是软组织肿瘤的表征和局部分期的首选方式;在特殊情况下需要CT。可疑或可能的恶性肿瘤应行活检。•在大型患者中,不确定或可疑的肿瘤,应联系肿瘤参考中心以获得转诊或远程放射学第二意见;这必须在活检前完成。
    OBJECTIVE: Early, accurate diagnosis is crucial for the prognosis of patients with soft tissue sarcomas. To this end, standardization of imaging algorithms, technical requirements, and reporting is therefore a prerequisite. Since the first European Society of Musculoskeletal Radiology (ESSR) consensus in 2015, technical achievements, further insights into specific entities, and the revised WHO-classification (2020) and AJCC staging system (2017) made an update necessary. The guidelines are intended to support radiologists in their decision-making and contribute to interdisciplinary tumor board discussions.
    METHODS: A validated Delphi method based on peer-reviewed literature was used to derive consensus among a panel of 46 specialized musculoskeletal radiologists from 12 European countries. Statements were scored online by level of agreement (0 to 10) during two iterative rounds. Either \"group consensus,\" \"group agreement,\" or \"lack of agreement\" was achieved.
    RESULTS: Eight sections were defined that finally contained 145 statements with comments. Overall, group consensus was reached in 95.9%, and group agreement in 4.1%. This communication contains the first part consisting of the imaging algorithm for suspected soft tissue tumors, methods for local imaging, and the role of tumor centers.
    CONCLUSIONS: Ultrasound represents the initial triage imaging modality for accessible and small tumors. MRI is the modality of choice for the characterization and local staging of most soft tissue tumors. CT is indicated in special situations. In suspicious or likely malignant tumors, a specialist tumor center should be contacted for referral or teleradiologic second opinion. This should be done before performing a biopsy, without exception.
    CONCLUSIONS: The updated ESSR soft tissue tumor imaging guidelines aim to provide best practice expert consensus for standardized imaging, to support radiologists in their decision-making, and to improve examination comparability both in individual patients and in future studies on individualized strategies.
    CONCLUSIONS: • Ultrasound remains the best initial triage imaging modality for accessible and small suspected soft tissue tumors. • MRI is the modality of choice for the characterization and local staging of soft tissue tumors in most cases; CT is indicated in special situations. Suspicious or likely malignant tumors should undergo biopsy. • In patients with large, indeterminate or suspicious tumors, a tumor reference center should be contacted for referral or teleradiologic second opinion; this must be done before a biopsy.
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  • 文章类型: English Abstract
    随着与复发性驱动分子改变相关的新实体的描述,子宫肉瘤的景观变得越来越复杂。子宫肉瘤,类似于软组织肉瘤,分为复杂基因组肉瘤和简单基因组肉瘤。平滑肌肉瘤和未分化子宫肉瘤属于复杂基因组肉瘤组。低级和高级子宫内膜间质肉瘤,其他与融合转录本相关的罕见肿瘤(如NTRK,PDGFB,ALK,RETROS1)和SMARCA4缺陷型子宫肉瘤被认为是简单的基因组肉瘤。最常见的子宫肉瘤首先是平滑肌肉瘤,其次是子宫内膜间质肉瘤。平滑肌肉瘤的三种不同组织学亚型(梭形,粘液样,上皮样)被识别,粘液样和上皮样平滑肌肉瘤比梭形平滑肌肉瘤更具侵袭性。低级和高级子宫内膜间质肉瘤之间的区别主要是形态学和免疫组织化学,融合转录本的检测可以帮助诊断。子宫PEComa是一种罕见的肿瘤,分为边缘和恶性,根据风险评估算法。子宫颈的胚胎性横纹肌肉瘤在儿童中更常见,但也可发生在成年女性中。子宫颈的胚胎性横纹肌肉瘤几乎总是DICER1突变,与野生型DICER1的阴道和DICER1突变但频率较低的腺肉瘤不同。在新兴实体中,与涉及NTRK的融合转录本相关的肉瘤,ALK,PDGFB基因受益于靶向治疗。分子数据与组织学和临床数据的整合可以更好地鉴定子宫肉瘤,以便更好地治疗它们。
    The landscape of uterine sarcomas is becoming more complex with the description of new entities associated with recurrent driver molecular alterations. Uterine sarcomas, in analogy with soft tissue sarcomas, are distinguished into complex genomic and simple genomic sarcomas. Leiomyosarcomas and undifferentiated uterine sarcomas belong to complex genomic sarcomas group. Low-grade and high-grade endometrial stromal sarcomas, other rare tumors associated with fusion transcripts (such as NTRK, PDGFB, ALK, RET ROS1) and SMARCA4-deficient uterine sarcoma are considered simple genomic sarcomas. The most common uterine sarcoma are first leiomyosarcoma and secondly endometrial stromal sarcomas. Three different histological subtypes of leiomyosarcoma (fusiform, myxoid, epithelioid) are identified, myxoid and epithelioid leiomyosarcoma being more aggressive than fusiform leiomyosarcoma. The distinction between low-grade and high-grade endometrial stromal sarcoma is primarily morphological and immunohistochemical and the detection of fusion transcripts can help the diagnosis. Uterine PEComa is a rare tumor, which is distinguished into borderline and malignant, according to a risk assessment algorithm. Embryonal rhabdomyosarcoma of the uterine cervix is more common in children but can also occur in adult women. Embryonal rhabdomyosarcoma of the uterine cervix is almost always DICER1 mutated, unlike that of the vagina which is wild-type DICER1, and adenosarcoma which can be DICER1 mutated but with less frequency. Among the emerging entities, sarcomas associated with fusion transcripts involving the NTRK, ALK, PDGFB genes benefit from targeted therapy. The integration of molecular data with histology and clinical data allows better identification of uterine sarcomas in order to better treat them.
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  • 文章类型: Journal Article
    横纹肌肉瘤是儿科人群中最常见的软组织肿瘤。在过去的25年中,患有横纹肌肉瘤的儿童的生存率仅略有改善,对于患有转移性疾病的儿童来说仍然很差。横纹肌肉瘤治疗进展的一个重大挑战是这种疾病的相对罕见,完成临床试验需要数年时间。可以通过国际合作和分享国家经验来加速进展。这需要就描述患者队列的共同语言和指导诊断的共识标准达成一致。治疗,和反应评估。这些目标为在2017年创建国际软组织肉瘤(INSTRuCT)奠定了前提。此后,该财团的多学科成员就该诊断制定了国际共识声明,治疗,和小儿软组织肉瘤的管理。在这里,INSTRuCT诊断成像工作组成员就横纹肌肉瘤患者的诊断成像提出了国际共识建议,在分期时,治疗期间和结束后。目的是为患有这种恶性肿瘤的儿科患者推广标准化的成像方法,以创建更可靠的国际临床试验结果比较,从而加速治疗横纹肌肉瘤和提高生存率的进展。
    Rhabdomyosarcoma is the most common soft-tissue neoplasm in the pediatric population. The survival of children with rhabdomyosarcoma has only marginally improved over the past 25 years and remains poor for those with metastatic disease. A significant challenge to advances in treatment of rhabdomyosarcoma is the relative rarity of this disease, necessitating years to complete clinical trials. Progress can be accelerated by international cooperation and sharing national experiences. This necessitates agreement on a common language to describe patient cohorts and consensus standards to guide diagnosis, treatment, and response assessment. These goals formed the premise for creating the INternational Soft Tissue saRcoma ConsorTium (INSTRuCT) in 2017. Multidisciplinary members of this consortium have since developed international consensus statements on the diagnosis, treatment, and management of pediatric soft-tissue sarcomas. Herein, members of the INSTRuCT Diagnostic Imaging Working Group present international consensus recommendations for imaging of patients with rhabdomyosarcoma at diagnosis, at staging, and during and after completion of therapy. The intent is to promote a standardized imaging approach to pediatric patients with this malignancy to create more-reliable comparisons of results of clinical trials internationally, thereby accelerating progress in managing rhabdomyosarcoma and improving survival.
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  • 文章类型: Journal Article
    目的:系统评价用于正电子发射断层扫描(PET)成像指南的方法,并比较这些建议的一致性。
    方法:我们搜索了PubMed,EMBASE,四个指南数据库,和谷歌学者确定有关使用PET的循证临床实践指南,PET/计算机断层扫描(CT),或常规实践中的PET/磁共振。我们使用研究和评估II评估指南评估了每个指南的质量,并比较了有关18F-氟代脱氧葡萄糖(FDG)PET/CT适应症的建议。
    结果:35个PET成像指南,包括在2008年至2021年之间发布的。这些指南在范围和目的领域表现良好(中位数为80.6%,四分位数间距[IQR]77.8-83.3%)和显示清晰度(中位数75%,IQR69.4-83.3%),但适用性较差(中位数为27.1%,IQR22.9-37.5%)。比较了13种癌症中48种适应症的建议。在与8种癌症类型有关的10种(20.1%)适应症中,在是否支持使用FDGPET/CT的方向上观察到相当大的不一致:头颈部癌(治疗反应评估),结直肠癌(I-III期患者的分期),食管癌(分期),乳腺癌(重新分类和治疗反应评估),宫颈癌(IB2期患者的分期和治疗反应评估),卵巢癌(复述),胰腺癌(诊断),和肉瘤(治疗反应评估)。
    结论:目前的PET成像指南在方法学质量上存在差异,并提供了相当不一致的建议。需要努力提高对指导方针制定方法的遵守,合成高质量的证据,并采用标准术语。
    PROSPEROCRD42020184965。
    结论:PET成像指南提供了相当不一致的建议,方法学质量也各不相同。建议临床医生在实践中应用这些建议时要对这些建议持批评态度,指南开发人员采用更严格的开发方法,研究人员优先考虑当前指南确定的研究差距。
    结论:•PET指南的方法学质量各不相同,并提供了不一致的建议。需要努力改进方法,合成高质量的证据,规范术语。·在AGREEII工具评估的六个方法学质量领域中,PET成像指南在范围和目的上表现良好(中位数为80.6%,四分位数间距77.8-83.3%)和表示清晰度(75%,69.4-83.3%),但适用性较差(27.1%,22.9-37.5%)。•在比较的48项建议(针对13种癌症类型)中,在是否支持FDGPET/CT使用的方向上的冲突在10个(20.1%)中观察到,对于8种癌症类型(即,头部和颈部,结直肠,食道,乳房,子宫颈,卵巢,胰腺,和肉瘤)。
    OBJECTIVE: To systematically appraise the methodologies used for guidelines for positron emission tomography (PET) imaging and to compare the consistency of these recommendations.
    METHODS: We searched PubMed, EMBASE, four guideline databases, and Google Scholar to identify evidence-based clinical practice guidelines pertaining to the use of PET, PET/computed tomography (CT), or PET/magnetic resonance in routine practice. We assessed the quality of each guideline using the Appraisal of Guidelines for Research and Evaluation II instrument and compared recommendations regarding indications for 18F-fluorodeoxyglucose (FDG) PET/CT.
    RESULTS: Thirty-five guidelines for PET imaging, published between 2008 and 2021, were included. These guidelines performed well in the domains of scope and purpose (median 80.6%, inter-quartile range [IQR] 77.8-83.3%) and clarity of presentation (median 75%, IQR 69.4-83.3%), but poorly in applicability (median 27.1%, IQR 22.9-37.5%). Recommendations for 48 indications in 13 cancers were compared. Considerable inconsistencies in the direction of whether to support the use of FDG PET/CT were observed in 10 (20.1%) indications pertaining to 8 cancer types: head and neck cancer (treatment response assessment), colorectal cancer (staging in patients with stages I-III disease), esophageal cancer (staging), breast cancer (restaging and treatment response assessment), cervical cancer (staging in patients with stage < IB2 disease and treatment response assessment), ovarian cancer (restaging), pancreatic cancer (diagnosis), and sarcoma (treatment response assessment).
    CONCLUSIONS: Current guidelines for PET imaging vary in methodological quality and provided considerably inconsistent recommendations. Efforts are needed to improve adherence to guideline development methodologies, to synthesis high-quality evidence, and to adopt standard terminologies.
    UNASSIGNED: PROSPERO CRD42020184965.
    CONCLUSIONS: Guidelines for PET imaging provide considerably inconsistent recommendations and vary in methodological quality. It is suggested that clinicians be critical of these recommendations when applying them in practice, that guideline developers adopt more rigorous development methodologies, and that researchers prioritize research gaps identified by current guidelines.
    CONCLUSIONS: • PET guidelines vary in methodological quality and provided inconsistent recommendations. Efforts are needed to improve methodologies, synthesize high-quality evidence, and standardize terminologies. • Among six domains of methodological quality assessed by the AGREE II tool, guidelines for PET imaging performed well in scope and purpose (median 80.6%, inter-quartile range 77.8-83.3%) and clarity of presentation (75%, 69.4-83.3%), but poorly in applicability (27.1%, 22.9-37.5%). • Among the 48 recommendations (for 13 cancer types) compared, conflicts in the direction of whether to support FDG PET/CT use were observed in 10 (20.1%), for 8 cancer types (i.e., head and neck, colorectal, esophageal, breast, cervical, ovarian, pancreatic, and sarcoma).
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  • 文章类型: Systematic Review
    背景:肉瘤的最佳管理需要在整个诊断过程中多学科的团队投入,治疗和随访。这项系统评价旨在评估在专门的肉瘤中心进行的手术对结局的影响。
    方法:对人群进行了系统评价,干预,比较和结果(PICO)模型。Medline,Embase,在CochraneCentral数据库中查询了评估本地控制的出版物,保肢率,30天和90天手术死亡率,与非专科中心相比,在专科肉瘤中心接受手术的患者的总生存率。每个研究都由两名独立的审阅者筛选适合性。对结果进行定性合成。
    结果:确定了66项研究。大多数研究是NHMRC证据等级评估的III-3级,而超过一半的研究质量很好。在专门的肉瘤中心进行的明确手术与改善的局部控制相关,如局部复发率较低所定义。手术切缘阴性率较高,提高局部无复发生存率和较高的保肢率。现有证据显示30天和90天死亡率较低的有利模式,与非专业中心相比,在专科肉瘤中心进行手术的总生存率更高。
    结论:在专门的肉瘤中心进行手术时,证据支持更好的肿瘤学结果。疑似肉瘤的病人应及早转介至专设的肉瘤中心进行多学科治疗,其中包括计划的活检和明确的手术。
    Optimal management of sarcoma requires multidisciplinary team input throughout the process of diagnosis, treatment and follow up. This systematic review aimed to evaluate the impact of surgery performed at specialised sarcoma centres on outcomes.
    A systematic review was conducted using the population, intervention, comparison and outcome (PICO) model. Medline, Embase, Cochrane Central databases were queried for publications that evaluated the local control, limb salvage rate, 30-day and 90-day surgical mortality, and overall survival in patients undergoing surgery in a specialist sarcoma centre compared with non-specialist centre. Each study was screened by two independent reviewers for suitability. A qualitative synthesis of the results was performed.
    Sixty-six studies were identified. The majority of studies were Level III-3 as assessed by the NHMRC Evidence Hierarchy, whilst just over half of the studies were of good quality. Definitive surgery performed at specialised sarcoma centres was associated with improved local control as defined by lower rate of local relapse, higher rate of negative surgical margins, improved local recurrence free survival and higher limb conservation rate. Available evidences show a favourable pattern of lower 30-day and 90-day mortality rates, and greater overall survival when surgery was performed in specialist sarcoma centres compared with non-specialised centres.
    Evidences support better oncological outcomes when surgery is performed at specialised sarcoma centre. Patients with suspected sarcoma should be referred early to a specialised sarcoma centre for multidisciplinary management, which includes planned biopsy and definitive surgery.
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  • 文章类型: English Abstract
    Soft tissue sarcomas are rare, heterogeneous tumors that are frequently in the extremities. Treatment includes surgical resection, combination chemotherapy and/or radiotherapy, as well as supplementary procedures such as isolated limb perfusion and regional deep hyperthermia. The prognosis depends on the tumor stage and the approximately 70 histological subtypes, with specific treatment approaches existing only for some subtypes. This review summarizes the recommendations of the German S3 guideline \"Adult Soft Tissue Sarcomas\" and the European Society for Medical Oncology (ESMO) guideline \"Soft tissue and visceral sarcomas\" regarding the diagnostic workup and therapy of soft tissue sarcomas of the extremities.
    UNASSIGNED: Weichgewebesarkome sind seltene, heterogene Tumore, welche häufig an den Extremitäten lokalisiert sind. Die Therapie beinhaltet die chirurgische Resektion, Kombinationschemotherapie und/oder Strahlentherapie sowie ergänzende Verfahren wie isolierte Extremitätenperfusion und regionale Tiefenhyperthermie. Die Prognose ist abhängig vom Tumorstadium und den etwa 70 histologischen Subtypen, wobei nur für einige Subtypen spezifische Therapieansätze existieren. Diese Übersichtsarbeit fasst die Empfehlungen der deutschen S3-Leitlinie „Adulte Weichgewebesarkome“ und der Leitlinie der European Society for Medical Oncology (ESMO) „Soft tissue and visceral sarcomas“ zur Diagnostik und Therapie lokalisierter Weichgewebesarkome der Extremitäten zusammen.
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  • 文章类型: Journal Article
    背景:儿童手术后的边缘状态,青少年,和年轻的成年人软组织肉瘤是有争议的,并已被不同的专业定义,定义随着时间和合作小组的推移而变化。国际软组织肉瘤协会(INSTRuCT)是儿童肿瘤学组织(COG)软组织肉瘤委员会的合作,欧洲小儿软组织肉瘤研究组(EpSSG),和欧洲合作WeichteilsarkomStudiengruppe(CWS)致力于通过汇集和挖掘合作小组的临床试验数据来改善患者的预后。
    方法:INSTRuCT非横纹肌肉瘤软组织肉瘤(NRSTS)工作组旨在制定有关儿童和青少年软组织肿瘤手术切缘评估和定义的国际统一建议。
    结论:本综述讨论了公认的原则和争议领域,包括外科医生的观点,病理学家,放射肿瘤学家,儿科肿瘤学家,制定一个框架,为未来的研究建立共同的指导方针。
    Margin status following surgery in children, adolescents, and young adults with soft tissue sarcomas is controversial and has been defined differently by various specialties, with definitions changing over time and by cooperative group. The International Soft Tissue Sarcoma Consortium (INSTRuCT) is a collaboration of the Children\'s Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft Tissue sarcoma Study Group (EpSSG), and the European Cooperative Weichteilsarkom Studiengruppe (CWS) devoted to improving patient outcomes by pooling and mining cooperative group clinical trial data.
    The INSTRuCT non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) working group aimed to develop international harmonized recommendations regarding surgical margin assessment and definitions in children and adolescents with soft tissue tumors.
    This review addresses accepted principles and areas of controversy, including the perspectives of surgeons, pathologists, radiation oncologists, and pediatric oncologists, to develop a framework for building common guidelines for future research.
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