thymic cancer

胸腺癌
  • 文章类型: Journal Article
    胸腺上皮肿瘤(TET),包括胸腺瘤和胸腺癌,是罕见的纵隔肿瘤.手术切除是可切除的TET的治疗策略,和术后放疗(PORT),以改善复发风险高的患者的局部控制。TET的稀有性导致缺乏随机对照试验,目前PORT的适应症主要依靠回顾性研究。这篇综述分析了有关TET的文献,突出显示端口,指导当前的研究和未来的调查。
    专注于TETS的研究,谈到港口的主题,并且有在线访问的英文摘要有资格纳入我们的审查。我们排除了病例报告或审查文章,用英语以外的语言写的文章,文章发表于30年前,和有关胸腺神经内分泌肿瘤的文章。
    Masaoka或Masaoka-Koga分期,世界卫生组织(WHO)组织学亚型,和切除状态指示切除的TET中的PORT。目前的文献表明,PORT不能改善I-IIA期TET的总体生存率,对于IIB-III阶段的测试结果不一致。风险较高的患者,如癌症或WHOB型,如果他们不发生远处转移,可能会受益于PORT。确定哪些患者将从PORT中受益最多需要进一步调查。对于经常性的TETS,应用PORT的意义尚不清楚,因为现有数据有限.鉴于TET的长期生存,晚期毒性,包括放射性肺炎,辐射诱导的心脏毒性,和继发性恶性肿瘤,必须解决。与传统的光子束放射治疗相比,质子束放射治疗可以通过使器官处于危险中来降低毒性。采用高精度放射治疗,随着新兴的免疫疗法,靶向治疗,和微创手术,可以改善TET结果。
    这篇评论合并了有关台球端口的文献,考虑到Masaoka-Koga分期,WHO组织学亚型,和切除状态。关于PORT功效的不同结果导致IIB-III期TET的未定义策略。尽管先进的放射治疗技术有望减少辐射引起的毒性,需要进一步的研究来研究PORT和联合治疗的疗效.
    UNASSIGNED: Thymic epithelial tumors (TETs), including thymomas and thymic carcinomas, are rare mediastinal tumors. Surgical resection is the treatment strategy for resectable TETs, and postoperative radiotherapy (PORT) is administered to improve local control in patients with a high risk of recurrence. The rarity of TETs has led to a lack of randomized controlled trials, and the current indications for PORT rely largely on retrospective studies. This review analyzes the literature on TETs, highlighting PORT, to guide current research and future investigations.
    UNASSIGNED: Studies that focused on TETs, addressed topics on PORT, and had English abstracts accessible online were eligible for inclusion in our review. We excluded case reports or review articles, articles written in languages other than English, articles published >30 years ago, and articles concerning thymic neuroendocrine tumors.
    UNASSIGNED: Masaoka or Masaoka-Koga staging, World Health Organization (WHO) histological subtype, and resection status indicate PORT in resected TETs. Current literature suggests that PORT does not improve overall survival in stage I-IIA TETs, with inconsistent results for stage IIB-III TETs. Patients with a higher risk, such as carcinomas or WHO type B, might benefit from PORT if they do not develop distant metastasis. Determining which patients will benefit most from PORT requires further investigation. For recurrent TETs, the significance of applying PORT is unclear because available data are limited. Given the long-term survival of TETs, late toxicities, including radiation pneumonitis, radiation-induced cardiotoxicities, and secondary malignancies, must be addressed. Proton beam radiotherapy might reduce toxicities by sparing organs at risk compared to conventional photon beam radiotherapy. The use of high-precision radiation therapy, along with emerging immunotherapy, targeted therapy, and minimally invasive surgery, could improve TET outcomes.
    UNASSIGNED: This review consolidates the literature on PORT for TETs, factoring in the Masaoka-Koga staging, WHO histological subtypes, and resection status. Varying results regarding PORT efficacy have led to an undefined strategy for stage IIB-III TETs. Although advanced radiotherapy techniques promise to reduce radiation-induced toxicities, further research is needed to investigate the efficacy of PORT and combination therapy.
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  • 文章类型: Journal Article
    胸腺上皮肿瘤(TET),包括胸腺瘤和胸腺癌,是源自胸腺的相对罕见的恶性肿瘤。虽然完整的手术切除是治疗这些肿瘤的基石,当地先进病例的最佳管理策略仍然不确定。新辅助疗法,它们有可能提高完全切除的可能性,很有希望,特别是在边缘可操作的情况下。然而,目前缺乏支持这种方法的证据。本文综述了关于诱导治疗后手术切除III期或IV期局部晚期TET的疗效的现有文献,旨在提供最新的观点并为未来的临床研究指明方向。
    使用关键字“手术”搜索PubMed,\"\"生存\",\"胸腺瘤\",“胸腺癌”,和“诱导疗法”。相关文章包括案例系列,回顾性研究,前瞻性研究,并对评论文章进行了评论和选择,以进行全面的叙述性评论。
    本综述包括主要揭示的回顾性研究和有限数量的前瞻性II期试验,这些试验涉及III期或IV期局部晚期TET的诱导治疗和手术治疗。没有确定随机III期研究,这表明尚未对诱导治疗对总生存期(OS)的益处进行综合评估.侵袭性胸腺瘤和胸腺癌的诱导治疗包括化疗,放射治疗,和放化疗,蒽环类药物联合化疗是主要选择。对于唯一的侵袭性胸腺瘤,完整手术切除的中位率和5年OS率分别为76%和85%,分别。有关TET诱导治疗的文献,包括胸腺瘤和胸腺癌,表明完全切除率和5年OS率分别为76%和70%,分别。
    我们对回顾性和前瞻性研究的叙述性综述强调了接受诱导治疗后手术切除的晚期TET患者的长期OS率。这些发现支持选择III期和IV期TET患者的这种多模式治疗策略。
    UNASSIGNED: Thymic epithelial tumors (TETs), including thymomas and thymic cancers, are relatively rare malignancies originating from the thymus. Although complete surgical resection is the cornerstone of treatment for these tumors, the optimal management strategy for locally advanced cases remains uncertain. Neoadjuvant therapies, with their potential to improve the likelihood of complete resection, are promising, particularly in marginally operable cases. However, the current evidence supporting this approach is lacking. This review of the existing literature on the efficacy of induction therapy followed by surgical resection for stage III or IV locally advanced TETs aimed to provide an up-to-date perspective and highlighting directions for future clinical research.
    UNASSIGNED: PubMed was searched using the keywords \"surgery,\" \"survival\", \"thymoma\", \"thymic cancer\", and \"induction therapy\". Relevant articles including case series, retrospective studies, prospective studies, and review articles were reviewed and selected for this comprehensive narrative review.
    UNASSIGNED: This review included primarily revealed retrospective studies and a limited number of prospective phase II trials on induction therapy followed by surgery for stage III or IV locally advanced TETs. No randomized phase III studies were identified, indicating that a comprehensive evaluation of the benefits of induction therapy on overall survival (OS) has not yet been conducted. Induction therapies for both invasive thymoma and thymic cancer included chemotherapy, radiotherapy, and chemoradiotherapy, with anthracycline-based combination chemotherapies being the primary option. For exclusively invasive thymomas, the median rate of complete surgical resection and the 5-year OS rate were reported as 76% and 85%, respectively. Literature focusing on induction therapy for TETs, which includes both thymoma and thymic cancers, indicates that the rates of complete resection and 5-year OS are 76% and 70%, respectively.
    UNASSIGNED: Our narrative review of retrospective and prospective studies highlighted promising long-term OS rates in patients with advanced TETs who underwent induction therapy followed by surgical resection. These findings support this multimodal treatment strategy in selected patients with stage III and IV TETs.
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  • 文章类型: Journal Article
    纵隔肿瘤,尤其是非神经内分泌胸腺上皮肿瘤(TET)相对少见,对广泛的流行病学研究构成挑战。这项研究对1999年至2019年美国(US)和德国(GER)的这些肿瘤进行了全面分析。
    从监测中确定被诊断为前纵隔恶性肿瘤的0-19岁(n=478)和≥20岁(n=17,459)的患者,流行病学,和最终结果注册表(SEER)和ZentrumfürKrebsregisterdaten(ZfKD)数据库。
    在年龄≥20岁的患者中,TET占最常见的前纵隔肿瘤(US/GER:63%/64%),其次是淋巴瘤(14%/8%)。对于<20岁的患者,主要肿瘤包括生殖细胞肿瘤(42%/14%),淋巴瘤(38%/53%),和TET(10%/27%)。胸腺瘤的总年发病率为每百万居民2.2/2.64(US/GER),胸腺癌为0.48/0.42。男女比例为1:1.09/1.03,平均年龄为59.48±14.89/61.33±13.94。患有胸腺瘤的人,但不是胸腺癌,与非胸腺原发性肿瘤对照相比,显示出发生继发性恶性肿瘤的风险显著增加21%/29%.
    这项研究提供了前纵隔肿瘤的比较分析,特别是TET,在过去的二十年里,美国和德国。此外,它强调了胸腺瘤患者继发性恶性肿瘤的发病率显著升高。
    UNASSIGNED: Mediastinal tumors, particularly non-neuroendocrine thymic epithelial tumors (TET) are relatively uncommon, posing challenges for extensive epidemiological studies. This study presents a comprehensive analysis of these tumors in the United States (US) and Germany (GER) from 1999 to 2019.
    UNASSIGNED: Patients aged 0-19 (n=478) and ≥20 years (n=17,459) diagnosed with malignant tumors of the anterior mediastinum were identified from the Surveillance, Epidemiology, and End Results registry (SEER) and the Zentrum für Krebsregisterdaten (ZfKD) databases.
    UNASSIGNED: Among patients aged ≥20 years, TETs accounted for the most prevalent anterior mediastinal tumors (US/GER: 63%/64%), followed by lymphomas (14%/8%). For patients <20 years, predominant tumors included germ cell tumors (42%/14%), lymphomas (38%/53%), and TETs (10%/27%). The overall annual incidence of thymoma was 2.2/2.64 (US/GER) per million inhabitants and for thymic carcinomas 0.48/0.42. The male-to-female ratio was 1:1.09/1.03, and the mean age 59.48 ± 14.89/61.33 ± 13.94. Individuals with thymomas, but not thymic carcinomas, exhibited a 21%/29% significantly heightened risk of developing secondary malignancies compared to controls with non-thymic primary tumors.
    UNASSIGNED: This study provides a comparative analysis of anterior mediastinal tumors, particularly TETs, in the US and GER over the past two decades. Furthermore, it highlights a significantly elevated incidence of secondary malignancies in thymoma patients.
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  • 文章类型: Journal Article
    背景:胸腺癌是一种罕见的疾病,发病率约为每百万0.5例,预后不良。这项研究的目的是评估接受一线化疗的晚期胸腺癌患者的预后。
    方法:在我们的回顾性队列研究中,我们纳入了2013年1月至2019年12月在我院接受转移性胸腺癌治疗的患者.总生存率,无进展生存期(PFS),评估和分析客观缓解率(ORR)和化疗方案.
    结果:对27例患者进行回顾性分析。所有患者均接受以铂(顺铂或卡铂)为基础的方案作为一线化疗(29.6%接受ADOC,11.1%获得PE,40.7%收到CP,14.8%收到CAP)。一线化疗的中位PFS为199天。有效率为40.7%。中位总生存期(OS)为585天。CD5染色阳性与更好的PFS相关。
    结论:我们强调铂类化疗药物作为晚期胸腺癌的主要治疗方式的关键作用,强调铂作为复发性疾病的一线选择的功效,即使在以前用铂治疗的病例中。此外,我们的研究结果表明,CD5阳性可能与改善的PFS有关,提示其作为预后标志物的潜力。
    BACKGROUND: Thymic carcinoma is a rare disease with an incidence of around 0.5 cases per million with a poor prognosis. The aim of this study was to assess patient outcomes with advanced thymic carcinoma receiving first-line chemotherapy.
    METHODS: In our retrospective cohort study, we included patients who underwent treatment for metastatic thymic carcinoma between January 2013 to December 2019 in our hospital. Overall survival, progression-free survival (PFS), objective response rates (ORR) and chemotherapy regimens were assessed and analyzed.
    RESULTS: A total of 27 patients were retrospectively analyzed. All patients received a platinum (cisplatin or carboplatin) based regimen as first-line chemotherapy (29.6% received ADOC, 11.1% received PE, 40.7% received CP, 14.8% received CAP). The median PFS on first-line chemotherapy was 199 days. The response rate was 40.7%. Median overall survival (OS) was 585 days. Positive CD5 staining was associated with better PFS.
    CONCLUSIONS: We highlight the critical role of platinum-based chemotherapy agents as a primary treatment modality in advanced thymic carcinoma, underscoring the efficacy of platinum as a first-line option for recurrent disease, even in cases previously treated with platinum. Additionally, our findings indicate that CD5 positivity could be associated with improved PFS, suggesting its potential as a prognostic marker.
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  • 文章类型: Case Reports
    多房胸腺囊肿(MTC)是由炎症引起的获得性多房囊肿。这种病变的稀有性和缺乏认识使得诊断和治疗困难。在这里,我们介绍了多房性纵隔囊肿的经验,该囊肿在13年的时间内导致了胸腺癌的发展并转移。计算机断层扫描结果显示,一名49岁男性的前纵隔肿块被怀疑是MTC。质量在7年的时间内逐渐缩小;然而,在最初检测后10年观察到生长。在被发现13年后,诊断为胸腺癌伴多发肺转移。在随访期间建议切除,但病人拒绝治疗.多房壁和位置是指示MTC的因素。然而,即使没有做出明确的诊断,由于确定术前诊断困难,应考虑切除多房前纵隔囊肿。然而,我们的病例表明肿瘤与囊肿共存是可能的,并且存在恶性肿瘤发展的潜力。
    Multilocular thymic cysts (MTC) are acquired multilocular cysts caused by inflammation. The rarity of such lesions and a lack of recognition make diagnosis and treatment difficult. Herein, we present our experience with a multilocular mediastinal cyst that resulted in the development of thymic cancer with metastasis over a period of 13 years. Computed tomography findings revealed an anterior mediastinal mass that was suspected to be an MTC in a 49-year-old man. The mass shrank gradually over a period of 7 years; however, growth was observed at 10 years after initial detection. At 13 years after detection, thymic carcinoma with multiple lung metastases was diagnosed. Resection was recommended during the follow-up period, but the patient refused treatment. A multilocular wall and location are factors that indicate MTC. However, even if a definitive diagnosis is not made, resection of multilocular anterior mediastinal cysts should be considered as determining the preoperative diagnosis is difficult. Nevertheless, our case suggests that the coexistence of tumors with cysts is possible, and the potential for malignant tumor development exists.
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  • 文章类型: Journal Article
    背景:评估电影MR特征跟踪技术在评估胸腺上皮肿瘤(TETS)诊断中评估心血管引起的形态变形的可行性。
    方法:我们的研究人群包括43例经病理证实的TET患者,包括10例低级别胸腺瘤,23个高级胸腺瘤,和10个胸腺癌.使用平衡的稳态自由进动序列获取电影MR图像,该序列在具有最大病变横截面积的切片中的轴向和倾斜平面中具有短时屏气。在舒张期手动描绘肿瘤边缘,并自动跟踪所有其他心脏阶段。使用Kruskal-WallisH检验和Mann-WhitneyU检验比较了三个病理组之间与搏动相关的长径比(ΔLSR)和肿瘤面积(Δ面积)的变化率。进行了受试者工作特征(ROC)曲线分析,以评估每个参数区分胸腺癌与胸腺瘤的能力。
    结果:△LSR和△面积在轴向平面(分别为p=0.028和0.006)和在斜平面(分别为p=0.034和0.043)中三组之间存在显着差异。在轴向平面上,胸腺癌的LSR和△面积值显着低于胸腺瘤(对于两者,p=0.012)和在斜平面中(分别为p=0.015和0.011)。诊断胸腺癌的△LSR和△面积的ROC曲线下面积为0.755~0.764。
    结论:使用cine-MR特征追踪分析评估形态学变形可以帮助诊断TET的组织病理学亚型,并在术前识别胸腺癌。
    BACKGROUND: To assess the feasibility of the cine MR feature tracking technique for the evaluation of cardiovascular-induced morphological deformation in the diagnosis of thymic epithelial tumors (TETs).
    METHODS: Our study population consisted of 43 patients with pathologically proven TETs including 10 low-grade thymomas, 23 high-grade thymomas, and 10 thymic carcinomas. Cine MR images were acquired using a balanced steady-state free precession sequence with short periods of breath-hold in the axial and oblique planes in the slice with the largest lesion cross-sectional area. The tumor margin was manually delineated in the diastolic phase and was automatically tracked for all other cardiac phases. The change rates of the long-to-short diameter ratio (∆LSR) and tumor area (∆area) associated with pulsation were compared between the three pathological groups using the Kruskal-Wallis H test and the Mann-Whitney U test. A receiver-operating characteristic (ROC) curve analysis was performed to assess the ability of each parameter to differentiate thymic carcinomas from thymomas.
    RESULTS: ∆LSR and ∆area were significantly different among the three groups in the axial plane (p = 0.028 and 0.006, respectively) and in the oblique plane (p = 0.034 and 0.043, respectively). ∆LSR and ∆area values were significantly lower in thymic carcinomas than in thymomas in the axial plane (for both, p = 0.012) and in the oblique plane (p = 0.015 and 0.011, respectively). The area under the ROC curves for ∆LSR and ∆area for the diagnosis of thymic carcinoma ranged from 0.755 to 0.764.
    CONCLUSIONS: Evaluation of morphological deformation using cine-MR feature tracking analysis can help diagnose histopathological subtypes of TETs and identify thymic carcinomas preoperatively.
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  • 文章类型: Case Reports
    我们在此报告了两例伴有Lynch综合征的胸腺癌,显示出高频率的微卫星不稳定性和错配修复蛋白表达的缺失,而没有MLH1启动子的过度甲基化。在病例1中,一名71岁的男子在MLH1中有致病性种系变异,并接受了肿瘤切除。到目前为止,没有复发的报道。在案例2中,一名43岁的男性接受了基因测试,该测试还显示MLH1中存在致病性种系变异。由于这两个病例有MLH变异,我们怀疑胸腺癌合并Lynch综合征与MLH1的种系致病变异之间可能存在关联.
    We herein report two cases of thymic cancer with Lynch syndrome showing a high frequency of microsatellite instability and loss of mismatch repair protein expression without MLH1 promoter hyper-methylation. In Case 1, a 71-year-old man had a pathogenic germline variant in MLH1 and underwent tumor resection. No relapse has been reported thus far. In Case 2, a 43-year-old man underwent genetic testing that also showed a pathogenic germline variant in MLH1. Since these two cases had MLH variants, we suspect a possible association between thymic cancer with Lynch syndrome and germline pathogenic variants in MLH1.
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  • 文章类型: Journal Article
    目的:胸腺上皮肿瘤(TEN)是一组异质性的罕见胸部恶性肿瘤。我们分析了临床病理特征,生存结果,危险因素,以及切除患者的复发模式。
    方法:回顾了2006-2019年接受TEN切除的成年患者的记录。使用Kaplan-Meier方法评估生存率。使用对数秩检验和Cox比例风险模型进行单变量和多变量分析。
    结果:共分析了100例患者(51例女性,中位年龄58岁)。胸腺瘤是最常见的组织学(n=92),其次是胸腺癌(n=5),和胸腺NET(神经内分泌肿瘤)(n=3)。II期(Masaoka)肿瘤最常见(n=51),其次是阶段I(n=27)。WHOB2/B3是最突出的组织学亚型(n=34)。91例患者实现完全切除(R0):86/92胸腺瘤,4/5胸腺癌和1/3NET。最常见的治疗方式是72例患者单独手术,24例患者接受手术和放射治疗,3例患者接受辅助放化疗。只有一名胸腺癌患者接受了新辅助化疗。10年总生存率和无病生存率分别为86.6%和83.9%,分别。复发在NETs中最常见(3/3)。多变量分析确定的复发危险因素是:R1/2切除(HR9.30,95CI1.82-36.1),TEN亚型(HR8.08,95CI1.24-34.6),和存在淋巴管浸润(LVI)(HR9.56,95CI2.56-25.8)。
    结论:对于TEN患者而言,完全切除仍然至关重要。不完全切除,高危组织学和LVI强调需要有效的辅助治疗方式.鉴于这些疾病的罕见,必须把重点放在对TEN进行的合作研究上。
    OBJECTIVE: Thymic epithelial neoplasms (TEN) represent a heterogeneous group of rare thoracic malignancies. We analyzed the clinicopathological features, survival outcomes, risk factors, and patterns of recurrence in patients undergoing resection.
    METHODS: Records were reviewed for adult patients with TEN who underwent resection from 2006-2019. Survival rates were assessed using the Kaplan-Meier method. Univariable and multivariable analyses were performed using the log-rank test and Cox proportional hazards model.
    RESULTS: A total of 100 patients were analyzed (51 females, median age 58 years). Thymoma was the most common histology (n = 92), followed by thymic carcinoma (n = 5), and thymic NET (neuroendocrine tumor) (n = 3). Stage II (Masaoka) tumours were most common (n = 51), followed by stage I (n = 27). WHO B2/B3 was the most prominent histological subtype (n = 34). Complete resection (R0) was achieved in 91 patients: 86/92 thymoma, 4/5 thymic carcinoma and 1/3 NET. The most common treatment modality was surgery alone in 72 patients, followed by surgery and radiation therapy in 24, and adjuvant chemoradiotherapy in 3 patients. Only one patient with thymic carcinoma received neoadjuvant chemotherapy. The 10-year overall and disease-free survival rates were 86.6% and 83.9%, respectively. Recurrence was most common in NETs (3/3). Risk factors for recurrence identified on multivariable analyses were: R1/2 resection (HR 9.30, 95%CI 1.82-36.1), TEN subtype (HR 8.08, 95%CI 1.24-34.6), and presence of lymphovascular invasion (LVI) (HR 9.56, 95%CI 2.56-25.8).
    CONCLUSIONS: Complete resection remains critical in patients with TEN. Incomplete resection, high-risk histology and LVI highlight the need for effective adjuvant modalities. Given the rarity of these diseases, emphasis must be placed on collaborative research conducted on TEN.
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  • 文章类型: Case Reports
    纵隔肿块相对少见。通常需要手术方法来诊断纵隔肿块。使用支气管内超声经支气管针吸活检术(EBUS-TBNA)接近纵隔肿块尚未建立。这里,我们介绍了一例使用EBUS-TBNA成功诊断胸腺癌的病例。
    Mediastinal masses are relatively uncommon. Surgical approach is often needed to diagnose mediastinal masses. Using endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) toward approaching a mediastinal mass has yet to be established. Here, we present a case of successful diagnosis of thymic cancer with the use of EBUS-TBNA.
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  • 文章类型: Case Reports
    The incidence of pericardial effusion in the U.S. is roughly 3.4% [1]. While most causes of pericardial effusions are indolent and transient, malignancy is a much more insidious cause that cannot be overlooked. Most cases of documented pericardial effusion secondary to malignancy have been due to mass effect from benign thymic tumors, such as thymomas. Our case highlights a 41-year-old male who presented with a dry cough and epigastric pain, found to have a large pericardial effusion and incidental thymic mass. The mass was biopsied and found to be keratinizing squamous cell carcinoma. This case expands our knowledge base as clinicians that pericardial effusions can be caused by malignant extension of tumors, rather than simply by mass effect of benign tumors.
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