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Surgical management of thymic tumors: a narrative review with focus on robotic-assisted surgery. 胸腺肿瘤的外科治疗:以机器人辅助手术为重点的综述。
Pub Date : 2024-11-07 eCollection Date: 2024-01-01 DOI: 10.21037/med-24-17
Robin Deckarm, Dominik Valentin Flury, Sarah Deckarm, Sebastian Ott, Gregor Jan Kocher

Background and objective: Thymic epithelial tumors, including thymomas and thymic carcinomas, represent the most common mediastinal tumors and account for up to 50% of all anterior mediastinal tumors. For early stages of these thymic tumors, complete resection of the entire thymus is the recommended treatment. The transition from open surgery to video-assisted thoracoscopic surgery (VATS) and recently to robotic-assisted thoracic surgery (RATS) has fundamentally altered the treatment of thymic tumors. While RATS has been widely implemented due to its many advantages including good visualization with magnification and three-dimensional vision, improved maneuverability and precise instrument control, different techniques have been described. This narrative review focuses on the main approaches and outcomes of RATS thymectomy. It compares the technical, perioperative and clinical outcomes of RATS thymectomy, in particular, with VATS and open thymectomy.

Methods: A non-systematic review for full text studies written in the English language was conducted using the PubMed search engine and literature was summarized.

Key content and findings: We present an overview of robotic-assisted resection for thymomas and review the main approaches and outcomes of RATS thymectomy. Critical points of the RATS approach, including surgical specifics and pitfalls, are presented. Technical advantages and disadvantages of each technique are discussed. The perioperative and clinical outcomes of RATS thymectomy are compared, where possible, to those for VATS and open thymectomy. Currently, retrospective analyses demonstrate comparable or even more favorable outcomes following a RATS approach in comparison to VATS and open approaches in terms of operating time, conversion rates, intraoperative complications, completeness of resection and mortality. Certain analyses also report better outcomes for patients undergoing RATS thymectomy in terms of blood loss, postoperative complications, duration of pleural drainage and length of hospital stay compared to VATS and open thymectomy.

Conclusions: Overall, RATS has shown promising results and could become the preferred technique for resection of thymic tumors. It shows good outcomes compared to VATS and open thymectomy in the current literature. However, especially for extended tumors with the need for extended resection and reconstruction, open thymectomy remains a valuable approach.

背景与目的:胸腺上皮肿瘤,包括胸腺瘤和胸腺癌,是最常见的纵隔肿瘤,占所有前纵隔肿瘤的50%。对于这些胸腺肿瘤的早期阶段,完全切除整个胸腺是推荐的治疗方法。从开放手术到视频辅助胸腔镜手术(VATS)以及最近的机器人辅助胸外科手术(RATS)的转变从根本上改变了胸腺肿瘤的治疗。虽然由于具有良好的可视化放大和三维视觉,改进的可操作性和精确的仪器控制等诸多优点,rat已被广泛应用,但不同的技术已被描述。本文综述了大鼠胸腺切除术的主要方法和结果。它比较了大鼠胸腺切除术的技术、围手术期和临床结果,特别是与VATS和开放式胸腺切除术。方法:使用PubMed搜索引擎对英文全文研究进行非系统综述,并对文献进行总结。主要内容和发现:我们介绍了机器人辅助胸腺瘤切除术的概况,并回顾了RATS胸腺切除术的主要方法和结果。提出了RATS方法的关键点,包括手术细节和陷阱。讨论了每种技术的技术优缺点。在可能的情况下,将rat胸腺切除术的围手术期和临床结果与VATS和开放式胸腺切除术进行比较。目前,回顾性分析表明,与VATS和开放入路相比,RATS入路在手术时间、转归率、术中并发症、切除的完全性和死亡率方面具有相当甚至更有利的结果。某些分析还报道,与VATS和开放式胸腺切除术相比,接受rat胸腺切除术的患者在出血量、术后并发症、胸膜引流时间和住院时间方面的结果更好。结论:总的来说,RATS显示出良好的效果,可能成为胸腺肿瘤切除术的首选技术。在目前的文献中,与VATS和开放胸腺切除术相比,它显示出良好的结果。然而,特别是对于需要扩大切除和重建的肿瘤,开放胸腺切除术仍然是一种有价值的方法。
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引用次数: 0
Current immunotherapy for thymic epithelial tumors: a narrative review. 目前胸腺上皮肿瘤的免疫治疗:叙述性回顾。
Pub Date : 2024-10-11 eCollection Date: 2024-01-01 DOI: 10.21037/med-24-24
Yoko Yamamoto, Kota Iwahori, Yasushi Shintani

Background and objective: Thymic epithelial tumors (TETs) are the most common neoplasm of the prevascular mediastinal compartment and are characterized by their rarity and variable clinical presentation. The present study aimed to explore the current management of patients with TET with a special focus on immunotherapy for advanced disease.

Methods: Relevant studies published between 1981 and 2024 were searched in PubMed using search terms "Thymoma", "Thymic cancer", "Myasthenia gravis", "Radiation therapy", "Surgery", and "Immunotherapy".

Key content and findings: The International Thymic Malignancy Interest Group and the International Association for the Study of Lung Cancer established the tumor-node-metastasis (TNM) staging system for TET based on an overall survival (OS) analysis of a retrospective international database. While complete surgical resection is the mainstay for resectable TET, there are currently no clear guidelines on systemic treatments for advanced TET because of the complexity, rarity, and heterogeneity of this disease and the lack of in vivo and in vitro models. With the development of immunotherapy, the application of the anti-programmed cell death-1 (anti-PD-1) antibody is expanding and includes TET. Clinical trials on immune checkpoint inhibitors (ICIs) are ongoing, and the acceptable clinical efficacy of the anti-PD-1 antibody for TET has been reported. On the other hand, there have been reports of a heightened frequency of severe immune-related adverse events (irAEs) in TET.

Conclusions: ICIs have the potential for patients with TET. The benefit-toxicity ratio of ICI treatment needs to be carefully evaluated for those patients.

背景与目的:胸腺上皮性肿瘤(TETs)是血管前纵隔室最常见的肿瘤,其特点是罕见且临床表现多变。本研究旨在探讨目前TET患者的管理,特别关注晚期疾病的免疫治疗。方法:使用检索词“胸腺瘤”、“胸腺癌”、“重症肌无力”、“放射治疗”、“手术”和“免疫治疗”在PubMed中检索1981年至2024年间发表的相关研究。主要内容和发现:国际胸腺恶性肿瘤兴趣小组和国际肺癌研究协会基于回顾性国际数据库的总生存(OS)分析,建立了TET的肿瘤-淋巴结-转移(TNM)分期系统。虽然完全手术切除是可切除TET的主要方法,但由于该病的复杂性、罕见性和异质性以及缺乏体内和体外模型,目前尚无明确的系统性治疗晚期TET的指导方针。随着免疫疗法的发展,抗程序性细胞死亡-1 (anti-PD-1)抗体的应用范围不断扩大,其中包括TET。免疫检查点抑制剂(ICIs)的临床试验正在进行中,抗pd -1抗体治疗TET的临床疗效已被报道。另一方面,有报道称TET中严重免疫相关不良事件(irAEs)发生率增高。结论:ICIs对TET患者具有潜在的治疗作用。对于这些患者,需要仔细评估ICI治疗的效益-毒性比。
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引用次数: 0
Benign disorders of the mediastinum: a narrative review. 纵隔良性疾病:叙述性回顾。
Pub Date : 2024-09-25 eCollection Date: 2024-01-01 DOI: 10.21037/med-24-14
Saad Farooq, Sabiha Armin, Daniel Ocazionez, Rosa M Estrada-Y-Martin, Sujith V Cherian

Background and objective: There are several benign processes that affect the mediastinum with considerable morbidity that may range from reactive entities to neoplastic disorders. This review article will focus on non-neoplastic benign mediastinal diseases which include large vessel vasculitis such as Takayasu and giant cell arteritis, mediastinal granulomas, fibrosing mediastinitis and mediastinal infections. These diseases can cause significant morbidity and mortality; therefore, we aim to familiarize readers with the pathophysiology, epidemiology and diagnosis of these mediastinal diseases and provide an update on the treatment options available.

Methods: We searched various databases such as PubMed and Google Scholar from August 2023 until January 2024 for the various benign mediastinal disorders we wanted to discuss. Relevant articles that were written in English were shortlisted and used to help write this narrative review.

Key content and findings: We will briefly discuss the anatomy of the mediastinum along with some of the more common benign mediastinal disorders. We will discuss epidemiology, etiology, clinical features, and treatment. Relevant laboratory, and imaging findings important to make the diagnosis will be included as well.

Conclusions: Prompt diagnosis of these diseases is of the utmost importance as delay in care may be associated with increased mortality. Our article aims to provide an up-to-date review and summarize the current literature regarding these diseases.

背景和目的:有几种良性病变可影响纵隔,发病率高,从反应性病变到肿瘤病变不等。本文将重点综述非肿瘤性良性纵隔疾病,包括大血管炎(如高松动脉炎和巨细胞动脉炎)、纵隔肉芽肿、纤维化性纵隔炎和纵隔感染。这些疾病可造成严重的发病率和死亡率;因此,我们的目的是让读者熟悉这些纵隔疾病的病理生理学、流行病学和诊断,并提供最新的治疗方案。方法:从2023年8月至2024年1月,我们检索了PubMed和谷歌Scholar等各种数据库,以获取我们想要讨论的各种良性纵隔疾病。用英语写的相关文章被列入候选名单,并用于撰写这篇叙述性评论。主要内容和发现:我们将简要讨论纵隔解剖以及一些更常见的良性纵隔疾病。我们将讨论流行病学、病因学、临床特征和治疗。对诊断重要的相关实验室和影像学结果也将包括在内。结论:这些疾病的及时诊断是至关重要的,因为延误护理可能与死亡率增加有关。我们的文章旨在提供最新的回顾和总结目前的文献关于这些疾病。
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引用次数: 0
Conservative management of emphysematous esophagitis-a case report. 肺气肿性食管炎的保守治疗1例。
Pub Date : 2024-07-15 eCollection Date: 2024-01-01 DOI: 10.21037/med-24-16
Taekyung Kang, Mi-Jin Kang

Background: Emphysematous esophagitis is a very rare disease and there are only a few previous reports in the literature. Previously reported cases have resulted in emphysematous esophagitis following anterior cervical procedures or ingestion of hydrogen peroxide (HP). In this report, we describe a case in which a patient with emphysematous esophagitis accompanied by gastritis without the above predisposing factors was treated with conservative treatment.

Case description: A 65-year-old woman was admitted to Inje University Sanggye Paik Hospital with general weakness, abdominal discomfort, nausea and chest discomfort. On chest and abdominal radiographs, there were abnormal air density in upper mediastinum and abdomen. Chest and abdomen computed tomography (CT) revealed mural air at entire esophagus and stomach. The patient managed with proton pump inhibitor (PPI), broad spectrum antibiotic therapy, and total parenteral nutrition (TPN).

Conclusions: Emphysematous gastritis occurs mainly along with emphysematous gastritis, with a mortality rate of up to 62%. It is mainly known to be caused by infection of the esophageal wall by gas forming bacteria, but there are also cases where there is no ingestion or exact cause. There is still controversy about treatment methods due to the high death rate, but if detected early like the reported patient, a good outcome can be expected with conservative treatment alone.

背景:肺气肿性食管炎是一种非常罕见的疾病,文献报道很少。以前报道的病例导致肺气肿性食管炎后,颈椎前路手术或摄取过氧化氢(HP)。在这篇报告中,我们描述了一例没有上述易感因素的肺气性食管炎合并胃炎患者接受保守治疗的病例。病例描述:一名65岁妇女因全身无力、腹部不适、恶心和胸部不适被仁济大学Sanggye Paik医院收治。胸腹平片显示上纵隔及腹部空气密度异常。胸部及腹部电脑断层扫描显示整个食道及胃内有壁气。患者接受质子泵抑制剂(PPI)、广谱抗生素治疗和全肠外营养(TPN)治疗。结论:气肿性胃炎主要伴发于气肿性胃炎,病死率高达62%。它主要是由形成气体的细菌感染食管壁引起的,但也有没有摄入或确切原因的病例。由于死亡率高,目前对治疗方法仍有争议,但如果像报道的患者一样早期发现,单纯保守治疗可取得良好效果。
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引用次数: 0
Resection of shrinking secondary thymic cyst during follow-up-a case report. 随访中切除缩小性继发性胸腺囊肿1例。
Pub Date : 2024-07-12 eCollection Date: 2024-01-01 DOI: 10.21037/med-24-13
Taekyung Kang, Mi-Jin Kang

Background: Thymic cysts can be classified as congenital or acquired. Most thymic cysts do not change in size over a short period of time. Although very rare, thymic cyst rupture is associated with serious complications, such as mediastinal hemorrhage and hemothorax. We experienced a case of partial rupture of a secondary thymic cyst, in an asymptomatic patient.

Case description: A 60-year-old woman visited Inje University Sanggye Paik Hospital with left hilar bulging detected on routine chest radiograph. A chest computed tomography (CT) scan revealed a 6 cm well-defined cystic mass with partial septation in the prevascular mediastinum. Thus, secondary thymic cyst was suggested. On the follow-up chest CT scan taken 3 months later, the size of the thymic cyst decreased, while the solid portion increased slightly, suggesting the potential presence of malignancy. Consequently, surgery was conducted. Adhesion to the lung and aorta was observed, but they were relatively well separated. The pathological findings revealed a partially ruptured thymic cyst with fat necrosis and multifocal granulomas.

Conclusions: There are controversies in the treatment of thymic cysts. Some clinicians prefer strict medical supervision to avoid unnecessary surgery, while others advocate immediate excision to avoid complication. However, if any changes are observed during the follow-up of the thymic cyst, it may indicate malignant transformation or rupture, necessitating prompt surgical excision.

背景:胸腺囊肿可分为先天性和后天性。大多数胸腺囊肿在短时间内不会改变大小。虽然非常罕见,但胸腺囊肿破裂常伴有严重的并发症,如纵隔出血和血胸。我们经历了一个病例部分破裂的继发性胸腺囊肿,在一个无症状的病人。病例描述:一名60岁妇女在仁济大学尚溪白医院就诊,常规胸片检查发现左肺门突出。胸部计算机断层扫描(CT)显示在血管前纵隔有一个6厘米明确的囊性肿块,部分分隔。因此,提示继发性胸腺囊肿。3个月后复查胸部CT,胸腺囊肿体积减小,实性部分略有增大,提示可能存在恶性肿瘤。因此,进行了手术。观察到肺和主动脉粘连,但它们相对较好地分离。病理结果显示胸腺囊肿部分破裂伴脂肪坏死及多灶性肉芽肿。结论:胸腺囊肿的治疗存在争议。一些临床医生倾向于严格的医疗监督,以避免不必要的手术,而另一些则主张立即切除,以避免并发症。然而,如果在随访中观察到任何变化,则可能表明胸腺囊肿恶性转化或破裂,需要及时手术切除。
{"title":"Resection of shrinking secondary thymic cyst during follow-up-a case report.","authors":"Taekyung Kang, Mi-Jin Kang","doi":"10.21037/med-24-13","DOIUrl":"https://doi.org/10.21037/med-24-13","url":null,"abstract":"<p><strong>Background: </strong>Thymic cysts can be classified as congenital or acquired. Most thymic cysts do not change in size over a short period of time. Although very rare, thymic cyst rupture is associated with serious complications, such as mediastinal hemorrhage and hemothorax. We experienced a case of partial rupture of a secondary thymic cyst, in an asymptomatic patient.</p><p><strong>Case description: </strong>A 60-year-old woman visited Inje University Sanggye Paik Hospital with left hilar bulging detected on routine chest radiograph. A chest computed tomography (CT) scan revealed a 6 cm well-defined cystic mass with partial septation in the prevascular mediastinum. Thus, secondary thymic cyst was suggested. On the follow-up chest CT scan taken 3 months later, the size of the thymic cyst decreased, while the solid portion increased slightly, suggesting the potential presence of malignancy. Consequently, surgery was conducted. Adhesion to the lung and aorta was observed, but they were relatively well separated. The pathological findings revealed a partially ruptured thymic cyst with fat necrosis and multifocal granulomas.</p><p><strong>Conclusions: </strong>There are controversies in the treatment of thymic cysts. Some clinicians prefer strict medical supervision to avoid unnecessary surgery, while others advocate immediate excision to avoid complication. However, if any changes are observed during the follow-up of the thymic cyst, it may indicate malignant transformation or rupture, necessitating prompt surgical excision.</p>","PeriodicalId":74139,"journal":{"name":"Mediastinum (Hong Kong, China)","volume":"8 ","pages":"52"},"PeriodicalIF":0.0,"publicationDate":"2024-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11707434/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142960019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Imaging of thymic epithelial tumors-a clinical practice review. 胸腺上皮肿瘤的成像--临床实践回顾。
Pub Date : 2024-06-07 eCollection Date: 2024-01-01 DOI: 10.21037/med-23-66
Sho Koyasu

This review article comprehensively examines the diagnostic approach to thymic epithelial tumors (TETs) and other mediastinal masses, focusing on imaging modalities and differential diagnosis. Beginning with a discussion on traditional and contemporary classification systems for mediastinal tumors, including the Japanese Association for Research on the Thymus (JART) and International Thymic Interest Group (ITMIG) classifications, it highlights the shift towards computed tomography (CT)-based categorizations. Emphasis is placed on the importance of distinguishing between solid and cystic lesions in the anterior mediastinum, with detailed insights into imaging characteristics and histological features of various TET subtypes such as thymomas, thymic carcinomas, and thymic neuroendocrine tumors (NETs). The review also elucidates common differential diagnoses, including lymphomas and germ cell tumors, providing guidance on key imaging findings and considerations for accurate diagnosis. Furthermore, it underscores the significance of patient background and blood tests in differential diagnosis, discussing age-related prevalence patterns and tumor marker assessment. After addressing the diagnostic challenges posed by thymic cysts offering insights into their radiological features, management considerations, and potential complications, this review extends to other rare mediastinal lesions highlighting the need for a comprehensive evaluation for accurate identification and management of these tumors. Finally, as illustrative examples, we present six cases highlighting various aspects of anterior mediastinal tumors, including TET. These cases provide valuable insights into the diagnostic challenges, imaging characteristics, and management considerations encountered in clinical practice. The cases presented herein do not all illustrate typical images, courses, and diagnoses. However, they each contain significant implications. Thus, we present them with the belief that they will aid in understanding the intricate nuances of image diagnosis in actual clinical practice.

这篇综述文章全面探讨了胸腺上皮性肿瘤 (TET) 和其他纵隔肿块的诊断方法,重点是成像模式和鉴别诊断。文章首先讨论了纵隔肿瘤的传统和现代分类系统,包括日本胸腺研究协会(JART)和国际胸腺兴趣小组(ITMIG)的分类,然后强调了向基于计算机断层扫描(CT)分类的转变。重点强调了区分前纵隔实性和囊性病变的重要性,并详细介绍了胸腺瘤、胸腺癌和胸腺神经内分泌肿瘤(NET)等各种 TET 亚型的影像学特征和组织学特征。该综述还阐明了常见的鉴别诊断,包括淋巴瘤和生殖细胞瘤,为准确诊断的关键影像学发现和注意事项提供了指导。此外,书中还强调了患者背景和血液检查在鉴别诊断中的重要性,并讨论了与年龄相关的发病模式和肿瘤标志物评估。在探讨了胸腺囊肿给诊断带来的挑战,对其放射学特征、管理注意事项和潜在并发症提出见解后,本综述扩展到其他罕见纵隔病变,强调了对这些肿瘤进行准确识别和管理的全面评估的必要性。最后,作为示例,我们介绍了六个病例,着重说明包括 TET 在内的前纵隔肿瘤的各个方面。这些病例为临床实践中遇到的诊断难题、影像学特征和管理注意事项提供了宝贵的见解。本文介绍的病例并不都是典型的图像、病程和诊断。但是,每个病例都具有重要意义。因此,我们在介绍这些病例时,相信它们有助于理解实际临床实践中影像诊断的复杂细微差别。
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引用次数: 0
Locally advanced thymic epithelial tumors: a foreword to the special series. 局部晚期胸腺上皮肿瘤:特别丛书前言。
Pub Date : 2024-06-07 eCollection Date: 2024-01-01 DOI: 10.21037/med-24-18
Masatsugu Hamaji
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引用次数: 0
Genomic insights into molecular profiling of thymic carcinoma: a narrative review. 胸腺癌分子图谱的基因组学见解:综述。
Pub Date : 2024-06-05 eCollection Date: 2024-01-01 DOI: 10.21037/med-24-5
So Takata

Background and objective: Thymic carcinoma is an exceptionally rare cancer, with an annual incidence of just 0.15-0.29 per 100,000 people. Owing to its rarity, only few proven treatments have been developed. Understanding its genetic profile is crucial for the development of targeted therapies. However, limited studies have exclusively examined thymic carcinoma mutations, with most investigation combining thymomas and thymic carcinomas. This paper reviews findings from genetic studies focusing on thymic carcinoma alone and compares them to those of thymoma.

Methods: We conducted a PubMed search for relevant English studies on thymic carcinoma genomics. Then, key papers utilizing target sequencing or whole-exome sequencing were analyzed.

Key content and findings: The most frequently mutated genes were TP53, CDKN2A, CDKN2B, CYLD, KIT, TET2, SETD2, BAP1, and ASXL1. TP53 and CDKN2A are correlated with poor prognosis. CYLD, which regulates signaling related with proliferation and interacts with AIRE expression and T cell development, might predict the immunotherapy response. KIT mutations might enable targeted therapy. TET2, SETD2, BAP1, and ASXL1 regulate epigenetics, suggesting disruption of these mechanisms. Higher tumor mutational burden (TMB) and 16q loss distinguish thymic carcinoma from thymoma. Although some copy number aberrations are shared, thymic carcinoma exhibits a mutational profile distinct from that of thymoma.

Conclusions: Thymic carcinoma demonstrates a unique genomic landscape, suggesting a molecular pathogenesis distinct from that of thymoma. Our findings revealed prognostic biomarkers such as TP53/CDKN2A and potential therapeutic targets such as KIT. Because thymic carcinoma is extremely rare, sharing molecular profiling data could provide valuable insights into the molecular mechanisms driving the development of these tumors.

背景和目的:胸腺癌是一种极为罕见的癌症,每年的发病率仅为每 10 万人中 0.15-0.29 例。由于其罕见性,目前仅开发出几种行之有效的治疗方法。了解其遗传特征对于开发靶向疗法至关重要。然而,专门研究胸腺癌突变的研究非常有限,大多数研究都是将胸腺瘤和胸腺癌结合起来进行的。本文回顾了单独针对胸腺癌的基因研究结果,并与胸腺瘤的研究结果进行了比较:我们在 PubMed 上搜索了胸腺癌基因组学的相关英文研究。然后,分析了采用靶向测序或全外显子组测序的主要论文:最常见的突变基因是TP53、CDKN2A、CDKN2B、CYLD、KIT、TET2、SETD2、BAP1和ASXL1。TP53和CDKN2A与预后不良有关。CYLD调节与增殖相关的信号转导,并与AIRE表达和T细胞发育相互作用,它可能预测免疫疗法的反应。KIT突变可能有助于靶向治疗。TET2、SETD2、BAP1和ASXL1调节表观遗传学,这表明这些机制会受到破坏。较高的肿瘤突变负荷(TMB)和16q缺失可将胸腺癌与胸腺瘤区分开来。虽然胸腺癌也有一些拷贝数畸变,但其突变特征与胸腺瘤截然不同:结论:胸腺癌显示出独特的基因组图谱,表明其分子发病机制与胸腺瘤不同。我们的研究结果揭示了TP53/CDKN2A等预后生物标志物和KIT等潜在治疗靶点。由于胸腺癌极为罕见,分享分子图谱数据可为了解这些肿瘤的分子机制提供有价值的信息。
{"title":"Genomic insights into molecular profiling of thymic carcinoma: a narrative review.","authors":"So Takata","doi":"10.21037/med-24-5","DOIUrl":"10.21037/med-24-5","url":null,"abstract":"<p><strong>Background and objective: </strong>Thymic carcinoma is an exceptionally rare cancer, with an annual incidence of just 0.15-0.29 per 100,000 people. Owing to its rarity, only few proven treatments have been developed. Understanding its genetic profile is crucial for the development of targeted therapies. However, limited studies have exclusively examined thymic carcinoma mutations, with most investigation combining thymomas and thymic carcinomas. This paper reviews findings from genetic studies focusing on thymic carcinoma alone and compares them to those of thymoma.</p><p><strong>Methods: </strong>We conducted a PubMed search for relevant English studies on thymic carcinoma genomics. Then, key papers utilizing target sequencing or whole-exome sequencing were analyzed.</p><p><strong>Key content and findings: </strong>The most frequently mutated genes were <i>TP53</i>, <i>CDKN2A</i>, <i>CDKN2B</i>, <i>CYLD</i>, <i>KIT</i>, <i>TET2</i>, <i>SETD2</i>, <i>BAP1</i>, and <i>ASXL1</i>. <i>TP53</i> and <i>CDKN2A</i> are correlated with poor prognosis. <i>CYLD</i>, which regulates signaling related with proliferation and interacts with AIRE expression and T cell development, might predict the immunotherapy response. <i>KIT</i> mutations might enable targeted therapy. <i>TET2</i>, <i>SETD2</i>, <i>BAP1</i>, and <i>ASXL1</i> regulate epigenetics, suggesting disruption of these mechanisms. Higher tumor mutational burden (TMB) and 16q loss distinguish thymic carcinoma from thymoma. Although some copy number aberrations are shared, thymic carcinoma exhibits a mutational profile distinct from that of thymoma.</p><p><strong>Conclusions: </strong>Thymic carcinoma demonstrates a unique genomic landscape, suggesting a molecular pathogenesis distinct from that of thymoma. Our findings revealed prognostic biomarkers such as <i>TP53</i>/<i>CDKN2A</i> and potential therapeutic targets such as <i>KIT</i>. Because thymic carcinoma is extremely rare, sharing molecular profiling data could provide valuable insights into the molecular mechanisms driving the development of these tumors.</p>","PeriodicalId":74139,"journal":{"name":"Mediastinum (Hong Kong, China)","volume":"8 ","pages":"39"},"PeriodicalIF":0.0,"publicationDate":"2024-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11330910/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142006092","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Re-evaluation and operative indications after induction therapy for thymic epithelial tumors. 胸腺上皮肿瘤诱导治疗后的重新评估和手术适应症。
Pub Date : 2024-06-04 eCollection Date: 2024-01-01 DOI: 10.21037/med-23-70
Yoshito Yamada, Masatsugu Hamaji, Harutaro Okada, Akihiro Takahagi, Hitomi Ajimizu, Sho Koyasu, Yuichi Sakamori, Akihiro Aoyama

Thymic epithelial tumors (TETs), encompassing thymoma and thymic carcinoma, represent a rare and heterogeneous group of thoracic malignancies with varying prognoses and treatment strategies. Surgical resection is the cornerstone of therapy for localized stages, but the management of locally advanced or unresectable TETs often involves induction therapy, including chemotherapy and/or radiation therapy, as a neoadjuvant approach aimed at downstaging the tumor to facilitate subsequent resection. This review synthesizes current knowledge on the re-evaluation process and operative indications following induction therapy for TETs, highlighting the pivotal role of accurate assessment in guiding surgical decisions and optimizing patient outcomes. Induction therapy's efficacy is contingent upon precise re-evaluation methods to accurately gauge treatment response and assess resectability post-therapy. This review discusses the various modalities employed in re-evaluation, including computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography-CT (PET-CT), and the significance of tumor markers, underlining their strengths and limitations. The adoption of modified RECIST criteria for TETs by the International Thymic Malignancy Interest Group (ITMIG) underscores the necessity for standardized assessment guidelines to ensure consistency and reliability across studies and clinical practices. Furthermore, we explore the implications of induction therapy on surgical decision-making, emphasizing the criteria for determining the suitability of patients for surgical intervention post-therapy. The review addresses the challenges and future perspectives associated with the re-evaluation process, including the potential for advanced imaging techniques and the integration of molecular and genetic markers to enhance the precision of treatment response assessment. In conclusion, the re-evaluation of TETs post-induction therapy is a complex but critical component of the multidisciplinary management approach for these patients. Standardizing re-evaluation methodologies and incorporating novel diagnostic tools could significantly improve the prognostication and treatment stratification, ultimately enhancing the therapeutic outcomes for patients with advanced TETs.

胸腺上皮性肿瘤(TET)包括胸腺瘤和胸腺癌,是一种罕见的异质性胸部恶性肿瘤,其预后和治疗策略各不相同。手术切除是局部分期治疗的基石,但局部晚期或无法切除的 TET 通常采用包括化疗和/或放疗在内的诱导治疗作为新辅助方法,目的是降低肿瘤的分期,以利于随后的切除。本综述综合了目前有关TET诱导治疗后的再评估过程和手术适应症的知识,强调了准确评估在指导手术决策和优化患者预后方面的关键作用。诱导治疗的疗效取决于精确的再评估方法,以准确衡量治疗反应并评估治疗后的可切除性。本综述讨论了再评价中采用的各种方式,包括计算机断层扫描(CT)、磁共振成像(MRI)、正电子发射断层扫描(PET-CT)以及肿瘤标志物的重要性,并强调了它们的优势和局限性。国际胸腺恶性肿瘤兴趣小组(ITMIG)对 TET 采用了修改后的 RECIST 标准,这凸显了制定标准化评估指南的必要性,以确保各项研究和临床实践的一致性和可靠性。此外,我们还探讨了诱导治疗对手术决策的影响,强调了确定患者是否适合在治疗后进行手术干预的标准。综述探讨了与再评价过程相关的挑战和未来展望,包括先进成像技术的潜力以及分子和遗传标记的整合,以提高治疗反应评估的精确度。总之,TETs 诱导治疗后的再评价是这些患者多学科管理方法中复杂但关键的组成部分。规范再评估方法并结合新型诊断工具可显著改善预后和治疗分层,最终提高晚期 TETs 患者的治疗效果。
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引用次数: 0
Narrative review of indication and management of induction therapy for thymic epithelial tumors. 胸腺上皮肿瘤诱导疗法的适应症和管理综述。
Pub Date : 2024-05-31 eCollection Date: 2024-01-01 DOI: 10.21037/med-23-30
Hitomi Ajimizu, Yuichi Sakamori

Background and objective: Thymic epithelial tumors (TETs) are rare and originate from the thymus. Thymomas and thymic carcinomas are the most common types of TETs. Of the two, thymomas tend to have a better prognosis and are typically localized, while thymic carcinomas have a worse prognosis and are more likely to spread. The Masaoka-Koga staging system is commonly used to determine the stage of TETs. Complete resection is the preferred treatment option, but treating locally advanced TETs can be challenging due to the invasion of surrounding structures. In such cases, induction therapy is administered to downstage the tumors and enable complete resection. We conducted this narrative review to evaluate the current progress in induction treatment for locally advanced TETs.

Methods: The literature search was performed using PubMed and Web of Science in June 2023. Prospective and retrospective published trials, systemic and narrative reviews, and meta-analyses were included.

Key content and findings: Induction chemotherapy is often used as a preoperative treatment for advanced TETs. Platinum and anthracycline-based chemotherapy regimens are commonly used for treating thymoma (response rate, 37-100%), and complete resection is highly common. Treatment with cisplatin and etoposide, carboplatin and paclitaxel, docetaxel and cisplatin have also demonstrated effectiveness, particularly in patients with thymic carcinoma or thymoma who cannot tolerate anthracycline regimens. The emergence of immunotherapy and targeted therapies may provide additional options for the treatment of TETs. Induction radiotherapy, as the sole treatment for TETs, is not widely practiced due to concerns about potential damage to surrounding tissues. However, combining modern radiation techniques with surgery has shown promising results in selected patients. Induction chemoradiotherapy, which combines chemotherapy and radiation, is an emerging approach for treating TETs. Despite the lack of randomized trials comparing chemotherapy with chemoradiotherapy, concurrent chemoradiation with radiation doses of 40-50 Gy is often considered the optimal induction therapy for thymic carcinoma patients or in more advanced special situations, such as great vessel invasion.

Conclusions: Overall, the optimal treatment for locally advanced TETs remains controversial. Induction therapy, including chemotherapy, radiotherapy, or chemoradiotherapy, is administered to downstage tumors and improve resectability. The choice of treatment depends on individual factors such as tumor stage, histology, and overall patient condition. However, further research and well-designed studies are needed to determine the most effective treatment strategies for locally advanced TETs.

背景和目的:胸腺上皮肿瘤(TET)非常罕见,起源于胸腺。胸腺瘤和胸腺癌是最常见的 TETs 类型。在这两种肿瘤中,胸腺瘤的预后往往较好,通常是局部性的,而胸腺癌的预后较差,更容易扩散。Masaoka-Koga分期系统通常用于确定TET的分期。完全切除是首选的治疗方案,但由于局部晚期TET会侵犯周围结构,因此治疗具有挑战性。在这种情况下,需要进行诱导治疗以降低肿瘤的分期,从而实现完全切除。我们撰写了这篇叙事性综述,以评估目前局部晚期TET诱导治疗的进展情况:方法:于 2023 年 6 月使用 PubMed 和 Web of Science 进行文献检索。主要内容和研究结果:诱导化疗通常用作晚期TET的术前治疗。铂类和蒽环类化疗方案常用于治疗胸腺瘤(反应率为37%-100%),完全切除非常常见。顺铂和依托泊苷、卡铂和紫杉醇、多西他赛和顺铂治疗也显示出疗效,特别是对不能耐受蒽环类方案的胸腺癌或胸腺瘤患者。免疫疗法和靶向疗法的出现可能会为 TET 的治疗提供更多选择。诱导放疗是治疗 TET 的唯一方法,但由于担心会对周围组织造成潜在损害,这种方法并未得到广泛应用。不过,将现代放射技术与手术相结合已在部分患者中显示出良好的疗效。结合化疗和放疗的诱导化放疗是治疗 TET 的新兴方法。尽管缺乏比较化疗与化学放疗的随机试验,但对于胸腺癌患者或更晚期的特殊情况(如大血管侵犯),放射剂量为40-50 Gy的同步化学放疗通常被认为是最佳的诱导疗法:总体而言,局部晚期TET的最佳治疗方法仍存在争议。包括化疗、放疗或化学放疗在内的诱导治疗可降低肿瘤的分期,提高切除率。治疗方法的选择取决于肿瘤分期、组织学和患者整体状况等个体因素。然而,要确定局部晚期TET最有效的治疗策略,还需要进一步的研究和精心设计的研究。
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Mediastinum (Hong Kong, China)
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