Soft tissue tumors

软组织肿瘤
  • 文章类型: Case Reports
    纤维瘤(DT)是一种罕见的局部侵袭性但非转移性间充质软组织肿瘤,主要发生在腹壁,腹腔,和四肢。它在肠系膜的发生相对罕见。
    本文报道了胃肠外科治疗的两例硬纤维瘤,潍坊市人民医院.第一个病例是一名59岁的男性患者,他之前曾接受过食管胃结合部癌的手术。术后,他的腹内肿块在三个月内迅速增大。第二例是一名60岁的男性患者,偶然发现左下腹部有肿块。两名患者都接受了手术治疗,术后病理诊断为肠系膜纤维瘤。
    硬纤维瘤的治疗仍然具有挑战性。简单的手术切除通常会产生不令人满意的结果,辅助放疗和化疗的疗效也有限。进一步的研究和临床实践是必要的,以改善诊断和治疗策略,旨在提高患者的生存和生活质量。
    UNASSIGNED: Desmoid tumor (DT) is a rare locally aggressive but non-metastatic mesenchymal soft tissue neoplasm that predominantly occurs in the abdominal wall, abdominal cavity, and extremities. Its occurrence in the mesentery is relatively uncommon.
    UNASSIGNED: This article reports two cases of desmoid tumor treated at the Department of Gastrointestinal Surgery, Weifang People\'s Hospital. The first case was a 59-year-old male patient who had previously undergone surgery for esophagogastric junction cancer. Postoperatively, he developed an intra-abdominal mass that rapidly increased in size within three months. The second case was a 60-year-old male patient who incidentally discovered a mass in the left lower abdomen. Both patients underwent surgical treatment, and the postoperative pathological diagnosis was mesenteric desmoid tumor.
    UNASSIGNED: The treatment of desmoid tumor remains challenging. Simple surgical resection often yields unsatisfactory outcomes, and the efficacy of adjuvant radiotherapy and chemotherapy is also limited. Further research and clinical practice are necessary to improve diagnostic and therapeutic strategies, aiming to enhance patient survival and quality of life.
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  • 文章类型: Journal Article
    血管肉瘤是一种罕见的侵袭性和未充分研究的软组织肉瘤,由于不同的研究队列和不一致的结果测量,尚待循证治疗指南。目前,广泛切除的手术被认为是管理的基石。在2000年至2017年从丹麦国家卫生登记册中确定的基于人群的队列中,这项研究旨在确定新诊断的软组织血管肉瘤患者的预后因素。Kaplan-Meier生存分析显示5年总生存率为28%。竞争风险分析表明,局部复发的累积发生率为30%,转移的累积发生率为43%。在154名患者中,多变量Cox模型显示年龄超过60岁,转移与较差的总体生存率独立相关。皮肤肿瘤,手术,阴性切缘与总生存率的提高独立相关。辅助肿瘤治疗并不能提高总生存率。转移的风险,或复发。阴性切缘与较低的复发和转移风险无关。我们的结论是,尽管证明了广泛切除手术后生存率的提高,总体生存率仍然很差。
    Angiosarcoma is a rare aggressive and understudied soft tissue sarcoma with pending evidence-based treatment guidelines due to varying study cohorts and inconsistent outcome measures. Surgery with wide resection is currently considered to be the cornerstone in management. In a population-based cohort identified from Danish National Health Registers between 2000 and 2017, this study aimed to define prognostic factors in patients with newly diagnosed soft tissue angiosarcoma. Kaplan-Meier survival analysis demonstrated 5-year overall survival of 28%. Competing risk analysis demonstrated cumulative incidence of local recurrence of 30% and metastasis of 43%. Multivariable Cox models among 154 included patients demonstrated age above 60 years and metastasis to be independently associated with worse overall survival. Cutaneous tumors, surgery, and negative resection margin were independently associated with improved overall survival. Adjuvant oncological treatment did not improve overall survival, risk of metastasis, or recurrence. Negative margin was not associated with lower risk of recurrence and metastasis. We conclude that, despite demonstrated improved survival after surgery with wide resection, overall survival remains poor.
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  • 文章类型: Journal Article
    背景:探讨不同定量动态对比增强(qDCE)-MRI示踪动力学(TK)模型和qDCE参数在区分良性和恶性软组织肿瘤(STT)中的潜力。
    方法:这项研究包括92例患者(41例女性,51名男性;年龄范围16-86岁,平均年龄51.24岁),有STT。qDCE参数(Ktrans,Kep,Ve,Vp,F,PS,通过使用以下TK模型估计STT的感兴趣区域的MTT和E):Tofts(TOFTS),扩展字体(EXTOFTS),绝热组织均匀性(ATH),常规隔室(CC),和分布参数(DP)。我们建立了一个结合形态学特征的综合模型,时间-信号强度曲线形状,和最优qDCE参数。使用曲线下面积(AUC)评估鉴定良性和恶性STT的能力,准确度,和决策曲线的分析。
    结果:TOFTS-Ktrans,EXTOFTS-Ktrans,EXTOFTS-Vp,CC-Vp和DP-Vp在qDCE参数中表现出良好的诊断性能。与其他传统知识模型相比,DP模型具有更高的AUC和更高的准确性。综合模型(AUC,0.936,0.884-0.988)在区分良性和恶性STT方面表现出优势,优于qDCE模型(AUC,0.899-0.915)和传统成像模型(AUC,0.802、0.712-0.891)单独使用。
    结论:各种TK模型成功区分良性和恶性STT。综合模型是一种非侵入性方法,结合了形态学成像方面和qDCE参数,并显示出进一步发展的巨大潜力。
    BACKGROUND: To explore the potential of different quantitative dynamic contrast-enhanced (qDCE)-MRI tracer kinetic (TK) models and qDCE parameters in discriminating benign from malignant soft tissue tumors (STTs).
    METHODS: This research included 92 patients (41females, 51 males; age range 16-86 years, mean age 51.24 years) with STTs. The qDCE parameters (Ktrans, Kep, Ve, Vp, F, PS, MTT and E) for regions of interest of STTs were estimated by using the following TK models: Tofts (TOFTS), Extended Tofts (EXTOFTS), adiabatic tissue homogeneity (ATH), conventional compartmental (CC), and distributed parameter (DP). We established a comprehensive model combining the morphologic features, time-signal intensity curve shape, and optimal qDCE parameters. The capacities to identify benign and malignant STTs was evaluated using the area under the curve (AUC), degree of accuracy, and the analysis of the decision curve.
    RESULTS: TOFTS-Ktrans, EXTOFTS-Ktrans, EXTOFTS-Vp, CC-Vp and DP-Vp demonstrated good diagnostic performance among the qDCE parameters. Compared with the other TK models, the DP model has a higher AUC and a greater level of accuracy. The comprehensive model (AUC, 0.936, 0.884-0.988) demonstrated superiority in discriminating benign and malignant STTs, outperforming the qDCE models (AUC, 0.899-0.915) and the traditional imaging model (AUC, 0.802, 0.712-0.891) alone.
    CONCLUSIONS: Various TK models successfully distinguish benign from malignant STTs. The comprehensive model is a noninvasive approach incorporating morphological imaging aspects and qDCE parameters, and shows significant potential for further development.
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  • 文章类型: Journal Article
    乳腺纤维瘤病(也称为乳腺纤维瘤)是肿瘤学家和外科医生护理乳腺疾病患者的罕见实体。当前的文献高度依赖于病例系列和来自硬纤维瘤相关疾病其他部位的外推。关于病理起源仍不清楚,自然史,以及对这种情况的治疗反应。传统的治疗策略以手术切除为中心,这可能会导致显著毁容的外观和功能结果,经常需要重新手术,和相关的发病率。与药物治疗或观察等待策略相比,支持前期手术切除的优越性的数据有限。当前针对韧带样肿瘤的治疗指南并未将乳房作为疾病部位,并且由于缺乏可用的证据而有目的地模棱两可。我们的目标是回顾有关乳腺纤维瘤病的文献,并根据我们在高容量四元转诊中心的病理学经验,提出一种当前对这种罕见疾病进行循证治疗的算法。
    Desmoid fibromatosis of the breast (also known as desmoid tumor of the breast) is a rare entity infrequently encountered by oncologists and surgeons caring for patients with breast disease. The current body of literature is highly reliant on case series and extrapolations from other sites of desmoid tumor-related disease. Much remains unclear regarding the pathological origins, natural history, and response to treatment of this condition. Traditional treatment strategies have centered on surgical resection, which may result in significantly disfiguring cosmetic and functional outcomes, frequent need for re-operation, and associated morbidity. There are limited data to support the superiority of upfront surgical resection when compared to medical therapy or watchful waiting strategies. Current treatment guidelines for desmoid tumors do not focus on the breast as a site of disease and are purposefully ambiguous due to the paucity of evidence available. We aim to review the literature concerning desmoid fibromatosis of the breast and propose an algorithm for current evidence-based management of this rare disease in the context of our experience with this pathology at a high-volume quaternary referral center.
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  • 文章类型: Journal Article
    背景:2024年,世界卫生组织(WHO)计划发布WHO软组织细胞病理学报告系统(WHORSSTC)。这个系统建立具有明确定义的类别,标准,并估计软组织肿瘤的恶性肿瘤(ROM)风险。对ROM的估计是基于相对较少的已发表研究。尽管报告系统的可重复性非常重要,但报告系统中并未解决观察者间的可重复性。
    方法:在1985年1月1日至2022年12月31日期间,对所有软组织肿瘤的细胞学标本进行了作者个人咨询文件和教学集(L.J.L.)的手动搜索。本研究仅包括有手术病理随访记录的病例。每个病例的载玻片由三名细胞病理学家独立评估,每个病例被分配到WHORSSTC类别之一。计算每个WHORSSTC类别的ROM。通过kappa和加权kappa统计数据评估了观察者之间的协议。
    结果:按类别划分的恶性肿瘤风险为:类别1:0%,类别2:28%,类别3:57%,第四类:47%,第5类:63%,第6类:88%。评估者之间协议的Kappa统计数据从0.2183到0.3465不等,加权kappa从0.3778到0.5217不等。
    结论:WHORSSTC显示恶性风险从“良性”类别(28%)发展到“恶性”类别(88%)。观察员之间的协议是公平的。
    BACKGROUND: In 2024, the World Health Organization (WHO) is scheduled to publish the WHO Reporting System for Soft Tissue Cytopathology (WHORSSTC). This system establishes categories with well-defined definitions, criteria, and estimated risks of malignancy (ROMs) for soft tissue tumors. The estimates of ROM are based on a relatively small number of published studies. Interobserver reproducibility is not addressed in the reporting system even though reproducibility of a reporting system is highly important.
    METHODS: A manual search of one authors personal consultation files and teaching set (L.J.L.) was conducted for all cytologic specimens of soft tissue tumors accessioned between January 1, 1985 and December 31, 2022. Only cases with documented surgical pathology follow-up were included in the study. Slides from each case were evaluated independently by three cytopathologists with each case assigned to one of the WHORSSTC categories. A ROM for each of the WHORSSTC categories was calculated. Interobserver agreement was evaluated by the kappa and weighted kappa statistics.
    RESULTS: Risk for malignancy by category were: Category 1: 0%, Category 2: 28%, Category 3: 57%, Category 4: 47%, Category 5: 63%, and Category 6: 88%. Kappa statistics for agreement between raters varied from 0.2183 to 0.3465 and weighted kappa varied from 0.3778 to 0.5217.
    CONCLUSIONS: The WHORSSTC showed a progression of malignancy risk from the category \"benign\" (28%) to the category \"malignant\" (88%). Interobserver agreement was only fair.
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  • 文章类型: Journal Article
    目的:系统评价影像组学和深度学习(DL)在软组织肿瘤良恶性鉴别诊断中的应用价值。
    方法:对截至2023年12月11日发表的利用影像组学和DL方法诊断STT的研究进行了系统评价。使用Radiomics质量评分(RQS)2.0系统和诊断准确性研究质量评估2(QUADAS-2)工具评估方法学质量和偏倚风险。分别。使用双变量随机效应模型来计算汇总的敏感性和特异性。为了确定导致异质性的因素,进行荟萃回归和亚组分析以评估以下协变量:诊断模式,感兴趣的区域/体积,影像学检查,研究设计,和病理类型。Deeks漏斗图的不对称性用于评估发表偏倚。
    结果:共纳入21项研究,涉及3866名患者,13项使用独立测试/验证集的研究包括在定量统计分析中。平均RQS为21.31,评级者之间达成了实质性或近乎完美的协议。联合的敏感性和特异性分别为0.84(95%CI:0.76-0.89)和0.88(95%CI:0.69-0.96),分别。Meta回归和亚组分析显示,研究设计和感兴趣区域/体积是影响研究异质性的显著因素(P<0.05)。未观察到发表偏倚。
    结论:影像组学和DL可以准确区分良性和恶性STT。未来的研究应该专注于提高研究设计的严谨性,进行多中心前瞻性验证,放大DL模型的可解释性,并整合多模式数据以提高软组织肿瘤评估的诊断准确性和临床实用性。
    OBJECTIVE: To systematically evaluate the application value of radiomics and deep learning (DL) in the differential diagnosis of benign and malignant soft tissue tumors (STTs).
    METHODS: A systematic review was conducted on studies published up to December 11, 2023, that utilized radiomics and DL methods for the diagnosis of STTs. The methodological quality and risk of bias were evaluated using the Radiomics Quality Score (RQS) 2.0 system and Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) tool, respectively. A bivariate random-effects model was used to calculate the summarized sensitivity and specificity. To identify factors contributing to heterogeneity, meta-regression and subgroup analyses were performed to assess the following covariates: diagnostic modality, region/volume of interest, imaging examination, study design, and pathology type. The asymmetry of Deeks\' funnel plot was used to assess publication bias.
    RESULTS: A total of 21 studies involving 3866 patients were included, with 13 studies using independent test/validation sets included in the quantitative statistical analysis. The average RQS was 21.31, with substantial or near-perfect inter-rater agreement. The combined sensitivity and specificity were 0.84 (95% CI: 0.76-0.89) and 0.88 (95% CI: 0.69-0.96), respectively. Meta-regression and subgroup analyses showed that study design and the region/volume of interest were significant factors affecting study heterogeneity (P < 0.05). No publication bias was observed.
    CONCLUSIONS: Radiomics and DL can accurately distinguish between benign and malignant STTs. Future research should concentrate on enhancing the rigor of study designs, conducting multicenter prospective validations, amplifying the interpretability of DL models, and integrating multimodal data to elevate the diagnostic accuracy and clinical utility of soft tissue tumor assessments.
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  • 文章类型: Case Reports
    促纤维化瘤表现出与其他软组织肿瘤相似的程度,即使是黄金标准研究也可能错过。
    这是报告一名47岁男性的胸壁肿块,最初被认为是血管瘤,但后来在活检的帮助下被诊断为增生性纤维母细胞瘤。
    UNASSIGNED: Desmoplastic fibroma presents similar to other soft tissue tumors to such an extent that even a gold standard investigation can miss.
    UNASSIGNED: This is to report a mass in a 47-year-old male arising from the chest wall, which was first thought to be a hemangioma but was later diagnosed as a case of desmoplastic fibroblastoma with the help of a biopsy.
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  • 文章类型: Journal Article
    血管平滑肌瘤并不常见,非癌,主要由血管引起的平滑肌肿瘤。由于缺乏跨学科方法或有限的患者池,以前的研究获得的数据有限。这项研究旨在提供血管平滑肌瘤的全面分析,包括人口统计,临床,放射学,和组织病理学特征,有大量的病人。2005年1月至2023年6月在一个中心进行回顾性调查,该研究涉及142名患者。相关信息是从电子病历中提取的,涵盖临床,放射学,组织学,和人口统计细节。血管平滑肌瘤主要发生在59岁(1-87岁),主要影响女性(53%),通常出现在皮下组织(85%)和下肢(76%)。MRI检查结果揭示了特征性信号,实体组织学类型的患病率很高(65%),经常显示网状征象。平滑肌肌动蛋白普遍存在(n=95/95),而Desmin和Caldesmon在83%(n=71/85)和98%(n=92/94)的病例中呈阳性表达,分别。这项研究提供了血管平滑肌瘤的最新和全面分析。通常表现为四肢明确的结节,这些肿瘤可以使用MRI有效诊断,虽然组织病理学分析通常是必要的确认。治疗主要包括直接切除,并发症和复发率低。
    Angioleiomyomas are uncommon, noncancerous, smooth muscle tumors that primarily arise from blood vessels. Previous studies have yielded limited data due to the lack of interdisciplinary approaches or restricted patient pools. This study aims to provide a comprehensive analysis of angioleiomyomas, including the demographic, clinical, radiological, and histopathological features, with a large number of patients. Conducted as a retrospective investigation at a single center from January 2005 to June 2023, this study involved 142 patients. Relevant information was extracted from electronic medical records, covering clinical, radiological, histological, and demographic details. Angioleiomyomas mostly occurred at age 59 (1-87), predominately affect females (53%) and commonly arise in subcutaneous tissue (85%) and the lower limbs (76%). MRI findings revealed characteristic signals, with a high prevalence of the solid histologic type (65%), often displaying a reticular sign. Smooth muscle Actin was universally present (n = 95/95), while Desmin and Caldesmon showed positive expression in 83% (n = 71/85) and 98% (n = 92/94) of cases, respectively. This study presents an updated and comprehensive analysis of angioleiomyomas. Typically appearing as well-defined nodules in the extremities, these tumors can be effectively diagnosed using MRI, though histopathological analysis is generally essential for confirmation. Treatment primarily involves straightforward excision, with notable low complication and recurrence rates.
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  • 文章类型: Journal Article
    软组织肿瘤(STT)由于其稀有性而提出了诊断和治疗挑战,复杂性,和形态重叠。准确区分良性和恶性STT对于确定治疗方向很重要,然而,这项任务可能很困难。机器学习和人工智能(AI)模型的集成可能有助于对这些肿瘤进行分类。这项研究的目的是研究AI和机器学习工具在将STT分为良性和恶性类别中的应用。这项研究包括三个组成部分:(1)评估整个幻灯片图像(WSI),以将STT分为良性和恶性实体。来自不同医疗中心的五名专业软组织病理学家独立审查了100个WSI,代表100个不同的案例,临床信息有限,没有额外的检查。结果表明,与参考诊断相比,总体符合率为70.4%。(2)鉴定可以区分良性和恶性STT的细胞特异性参数。使用图像分析软件(QuPath)和95例病例的队列,发现几个细胞特异性参数具有统计学意义,最值得注意的是细胞计数,细胞核/细胞面积比,细胞核苏木精密度平均值,和细胞最大卡尺。(3)评估机器学习库(Scikit-learn)在鉴别良性和恶性STT中的作用。总共195例STT病例(训练组156例,验证组39例)达到约70%的敏感性和特异性,AUC为0.68。我们有限的研究表明,在软组织病理学中使用WSI和AI有可能提高诊断准确性并确定可以区分良性和恶性STT的参数。我们设想将人工智能整合为一种支持工具,以增强病理学家的诊断能力。
    Soft tissue tumors (STTs) pose diagnostic and therapeutic challenges due to their rarity, complexity, and morphological overlap. Accurate differentiation between benign and malignant STTs is important to set treatment directions, however, this task can be difficult. The integration of machine learning and artificial intelligence (AI) models can potentially be helpful in classifying these tumors. The aim of this study was to investigate AI and machine learning tools in the classification of STT into benign and malignant categories. This study consisted of three components: (1) Evaluation of whole-slide images (WSIs) to classify STT into benign and malignant entities. Five specialized soft tissue pathologists from different medical centers independently reviewed 100 WSIs, representing 100 different cases, with limited clinical information and no additional workup. The results showed an overall concordance rate of 70.4% compared to the reference diagnosis. (2) Identification of cell-specific parameters that can distinguish benign and malignant STT. Using an image analysis software (QuPath) and a cohort of 95 cases, several cell-specific parameters were found to be statistically significant, most notably cell count, nucleus/cell area ratio, nucleus hematoxylin density mean, and cell max caliper. (3) Evaluation of machine learning library (Scikit-learn) in differentiating benign and malignant STTs. A total of 195 STT cases (156 cases in the training group and 39 cases in the validation group) achieved approximately 70% sensitivity and specificity, and an AUC of 0.68. Our limited study suggests that the use of WSI and AI in soft tissue pathology has the potential to enhance diagnostic accuracy and identify parameters that can differentiate between benign and malignant STTs. We envision the integration of AI as a supportive tool to augment the pathologists\' diagnostic capabilities.
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  • 文章类型: Journal Article
    目的:为了确定基于钆的造影剂是否能增加灵敏度,肌肉骨骼软组织肿瘤恶性潜能的特异性或读者信心。
    方法:来自87例患者的对比前和对比后的MRI研究由三位不同经验的独立放射科医师阅读。根据对比前和对比后的MRI研究,读者注意到其诊断的恶性潜力和信心。统计模型评估了MRI阅读器诊断与病理结果之间的一致性,并分析了对比度对阅读器信心的影响。还计算了观察者之间和观察者之间的恶性潜能变异性。
    结果:评估了87例患者(良性48例,恶性39例;平均[±SD]年龄分别为51±17.9和57.1±17.1)。对于所有的读者,对比前和对比后敏感性分别为68.1%和70.6%,对比前和对比后特异性分别为84.6%和83.8%,分别无显著变化(p=0.88)。在有或没有常驻读者的情况下,与使用对比和预测恶性潜力没有显着关联(p=0.65和p=0.82)。对比的使用与所有读者的较高水平的读者信心(p=0.02)显着相关。观察者之间和观察者内部的差异非常一致(W=0.77和0.70)。
    结论:造影后序列的添加增加了读者对其诊断的信心,而对恶性潜能的准确预测没有相应的显着增加。
    OBJECTIVE: To determine if gadolinium-based contrast agents increase the sensitivity, specificity or reader confidence of malignant potential in musculoskeletal soft tissue tumors.
    METHODS: Pre- and post-contrast MRI studies from 87 patients were read by three independent radiologists of different experience. Readers noted malignant potential and confidence in their diagnosis based on pre-contrast and post-contrast MRI studies. Statistical models assessed for agreement between MRI reader diagnosis and pathologic results as well as analyzing effects of contrast on reader confidence. Inter- and intra-observer variabilities of malignant potential were also calculated.
    RESULTS: 87 patients (48 benign and 39 malignant; mean [± SD] age 51 ± 17.9 and 57.1 ± 17.1, respectively) were evaluated. For all readers, pre-contrast and post-contrast sensitivities were 68.1 % and 70.6 % while pre-contrast and post-contrast specificities were 84.6 % and 83.8 %, respectively without significant change (p=0.88). There was not a significant association with the use of contrast and prediction of malignant potential with or without the resident reader (p=0.65 and p=0.82). Use of contrast was significantly associated with higher levels of reader confidence (p=0.02) for all readers. Inter- and intra-observer variabilities were in good agreement (W = 0.77 and 0.70).
    CONCLUSIONS: The addition of a post-contrast sequence increased reader confidence in their diagnosis without a corresponding significant increase in accurate prediction of malignant potential.
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