Carcinoid

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  • 文章类型: Case Reports
    背景:中央气道肿瘤偶尔会被误诊为慢性疾病。我们介绍了一个被误认为是慢性哮喘多年的中央气道类癌的病例。
    方法:一名29岁的男性健美运动员因呼吸急促和咯血出现在急诊科。他是一位狂热的健美运动员,参加了比赛。他被诊断患有哮喘多年,并长期使用沙丁胺醇吸入器。胸部计算机断层扫描显示左半胸部弥漫性混浊,多个空气-液体水平和近端左主支气管的4厘米肿块,并伴有管腔内钙化。支气管镜检查显示巨大的支气管内肿块,活检为典型类癌。通过刚性支气管镜检查和部分支气管内清创术可实现稳定,以使左肺通畅。稳定后,他随后接受了左肺切除术。他恢复良好,并在术后第2天出院回家。在全肺切除术后2.5年的监测中,他已经恢复健美,没有疾病复发的证据。
    结论:近端气道肿瘤可以模拟哮喘。即使在非常复杂的情况下,谨慎的管理也可以取得成功。应该增加对其他诊断的怀疑,特别是在年轻和健康的哮喘患者中,这种哮喘难以治疗。
    结论:气道近端肿瘤可以模拟慢性疾病,如哮喘。应该考虑其他诊断,特别是在年轻和健康的人有哮喘症状,对传统疗法没有反应。
    BACKGROUND: Central airway tumors can occasionally be misdiagnosed as a chronic disease. We present a case of a central airway carcinoid tumor that was mistaken as chronic asthma for many years.
    METHODS: A 29-year-old male bodybuilder presented to our emergency department with shortness of breath and hemoptysis. He was an avid bodybuilder who participated in competitions. He had been diagnosed with asthma for years and used an albuterol inhaler chronically. Computed tomography of the chest showed diffuse opacification of the left hemithorax, multiple air-fluid levels and a 4-cm mass of the proximal left mainstem bronchus with intraluminal calcifications. Bronchoscopy demonstrated a large endobronchial mass, and biopsy was positive for typical carcinoid tumor. Stabilization was achieved with rigid bronchoscopy and partial endobronchial debridement of the tumor to allow some patency to the left lung. After stabilization, he subsequently underwent left pneumonectomy. He recovered well and was discharged home on postoperative day 2. On surveillance 2.5 years after pneumonectomy, he has resumed bodybuilding and has no evidence of recurrent disease.
    CONCLUSIONS: Proximal airway tumors can mimic asthma. Careful management can achieve successful results even in very complex cases. There should be an increased level of suspicion for other diagnoses, especially in young and healthy individuals with asthma that is refractory to medical treatment.
    CONCLUSIONS: Proximal airway tumors can mimic chronic diseases such as asthma. Other diagnoses should be considered, especially in young and health individuals with asthma symptoms that do not respond to conventional therapies.
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  • 文章类型: Journal Article
    胃神经内分泌肿瘤(G-NENs)是一组异质性肿瘤,大致分为两组。第一组,由胃泌素过度分泌驱动,通常是多焦点的,小,并且表现得懒惰,进展和转移扩散的风险较低(但非零)。它们通常分为1型,内源性胃泌素过量产生,和2型G-NEN,胃外胃泌素分泌肿瘤过度产生胃泌素。第二组,称为3型G-NEN,自发发生,并且可能更具攻击性,具有类似于其他胃肠道神经内分泌肿瘤的临床过程。1型G-NEN可以通过内镜监测和切除可见病变,取得了巨大的成功。为罕见的高危病变保留手术,而在2型G-NEN中,对致病性胃泌素分泌肿瘤的手术切除通常是治愈性的。3型G-NEN通常通过正式的手术切除来管理,但越来越多的证据表明,在适当选择的低风险患者中,有限的手术甚至内镜切除是安全有效的。一种新的G-NEN亚型,与长期使用质子泵抑制剂有关,发病率正在增加。病理生理学似乎与1型G-NEN平行。在转移性疾病的背景下,它可以发生在任何亚型中,但在3型G-NEN中最常见,由于缺乏G-NEN特有的试验数据,因此需要外推治疗非胃神经内分泌疾病的策略和药物.该领域的快速发展也可能使转移性G-NEN患者受益。由于治疗是基于G-NEN的类型,建立病变的病因学至关重要,但对G-NEN病理生理学的认识和病理学家之间的密切合作,胃肠病学家,放射科医生,外科医生,和肿瘤学家已经实现了逐步降级和侵入性较小的治疗范式的增长趋势。
    UNASSIGNED: Gastric neuroendocrine neoplasms (G-NENs) are a heterogeneous group of tumors that broadly fall into two groups. The first group, driven by oversecretion of gastrin, are generally multifocal, small, and behave indolently with a low (but non-zero) risk of progression and metastatic spread. They are conventionally categorized into type 1, with endogenous gastric-based overproduction of gastrin, and type 2 G-NEN, with overproduction of gastrin from an extra-gastric gastrin-secreting tumor. The second group, termed type 3 G-NEN, occur spontaneously and are potentially more aggressive, having a clinical course analogous to other neuroendocrine tumors of the gastrointestinal tract. Type 1 G-NEN can be managed with endoscopic surveillance and resection of visible lesions with great success, reserving surgery for the rare high-risk lesion, whereas surgical resection of the causative gastrin-secreting tumor in type 2 G-NEN is usually curative. Type 3 G-NEN is usually managed with formal surgical resection but there is growing evidence that limited surgery or even endoscopic resection in appropriately selected patients with low risk is both safe and effective. A novel subtype of G-NEN, associated with long-term proton pump inhibitor usage, is increasing in incidence. The pathophysiology seems to parallel type 1 G-NEN. In the setting of metastatic disease, which can occur in any subtype but is most common by far in type 3 G-NEN, the lack of trial data unique to G-NEN results in extrapolation of strategies and agents for treatment of non-gastric neuroendocrine disease. The rapid pace of development in this area is likely to benefit the metastatic G-NEN patient as well. As treatment is predicate on type of G-NEN, establishing the etiology of the lesion is crucial but growing knowledge of G-NEN pathophysiology and close collaboration between pathologists, gastroenterologists, radiologists, surgeons, and oncologists have enabled a growing trend towards de-escalation and less-invasive treatment paradigms.
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  • 文章类型: Journal Article
    神经内分泌肿瘤,也被称为神经内分泌肿瘤(NET),是罕见的肿瘤,来源于具有神经和内分泌细胞特征的细胞。临床表现,诊断,NEN的治疗方法因类型而异,location,肿瘤是否有激素功能,它有多咄咄逼人,以及它是否已经转移到身体的其他部位。本文概述了特定类型的NET,临床表现和相关综合征,诊断,和常见NENs的管理方法。
    Neuroendocrine neoplasms (NENs), also known as neuroendocrine tumors (NETs), are rare tumors derived from cells with characteristics of both nerve and endocrine cells. The clinical presentation, diagnosis, and treatment of NENs vary significantly depending on the type, location, whether the neoplasm is hormonally functional, how aggressive it is, and whether it has metastasized to other parts of the body. This article provides an overview of specific types of NETs, clinical presentations and related syndromes, diagnosis, and approach to management of common NENs.
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  • 文章类型: Journal Article
    背景。纵隔畸胎瘤引起的神经内分泌病变是罕见的肿瘤,文献报道的患者数量很少。这些病变的行为似乎与传统的神经内分泌肿瘤不同。全面审查对于类似病例的组织学评估和治疗计划将是有价值的。案例介绍。我们提供了一个57岁男子咳嗽的例子。随后的检查显示,计算机断层扫描显示前纵隔肿块为2.1cm。患者接受了机器人辅助的胸腔镜胸腺切除术。组织学检查显示,成熟的囊性畸胎瘤具有神经内分泌成分,由肿瘤细胞簇组成,具有圆形至椭圆形的核和“盐和胡椒”染色质模式。肿瘤细胞对细胞角蛋白具有免疫反应性,突触素,嗜铬粒蛋白,和INSM1,Ki-67增殖指数为4%。组织学诊断为成熟畸胎瘤,伴有分化良好的低度神经内分泌肿瘤(类癌)。在10个月完成手术切除后,患者情况良好,没有疾病。文献综述。文献回顾了13例纵隔畸胎瘤引起的神经内分泌病变。没有疾病相关死亡率的报告,即使在有高级神经内分泌的病变中,癌,或未成熟的畸胎瘤成分。Conclusions.手术切除是治疗这些病变的主要手段,并且神经内分泌成分的存在似乎不会对预后产生负面影响。
    Background. Neuroendocrine lesions arising from mediastinal teratomas are rare tumors with only small number of patients reported in literature. The behavior of these lesions appears to be different from traditional neuroendocrine neoplasms. A comprehensive review will be valuable for histologic assessment and treatment planning for similar cases. Case presentation. We present an example of a 57-year-old man who presented with cough. Subsequent work-up revealed an anterior mediastinal mass of 2.1 cm on computed tomography. The patient underwent robot-assisted thoracoscopic thymectomy. Histological examination revealed a mature cystic teratoma with a neuroendocrine component consisting of clusters of tumor cells with round to oval nuclei and a \"salt-and-pepper\" chromatin pattern. The tumor cells were immunoreactive to cytokeratin, synaptophysin, chromogranin, and INSM1, with a Ki-67 proliferative index of 4%. A histological diagnosis was mature teratoma with well-differentiated low-grade neuroendocrine tumor (carcinoid) was made. The patient was well and without disease after complete surgical excision at 10 months. Literature review. Literature reviewed yielded 13 examples of neuroendocrine lesions arising from mediastinal teratomas. No disease-related mortality was reported, even in lesions with high-grade neuroendocrine, carcinomatous, or immature teratomatous components. Conclusions. Surgical removal is the mainstay of treatment of these lesions, and the presence of a neuroendocrine component does not appear to negatively affect prognosis.
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  • 文章类型: Journal Article
    背景:中央气道阻塞(CAO),见于各种恶性和非恶性气道疾病,与预后不良有关。CAO的管理依赖于提供商的培训和当地资源,这可能使临床方法和结果高度可变。我们回顾了当前的文献,并为CAO的管理提供了基于证据的建议。
    方法:一个多学科专家小组使用PICO(患者,干预,比较器,和结果)格式,并使用MEDLINE(PubMed)和Cochrane图书馆进行了系统的文献检索。小组筛选了纳入的参考文献,并使用经过审查的评估工具来评估纳入研究的质量并提取数据,并对支持每个建议的证据水平进行分级。使用改进的Delphi技术就建议达成共识。
    结果:九千,审查了68份摘要,评估了150篇全文,31项研究纳入分析.编写了一份良好做法说明和十项分级建议。证据的总体确定性很低。
    结论:支气管镜治疗可以改善症状,生活质量,恶性和非恶性CAO患者的生存率。多模式治疗选择,包括全身麻醉的硬支气管镜检查,肿瘤/组织清创术,消融,膨胀,和支架放置应在适当的时候使用。治疗选择和结果取决于CAO的潜在病因。强烈鼓励多学科方法和与患者共同决策。
    BACKGROUND: Central airway obstruction (CAO), seen in a variety of malignant and non-malignant airway disorders, is associated with a poor prognosis. The management of CAO is dependent on provider training and local resources, which may make the clinical approach and outcomes highly variable. We reviewed the current literature and provided evidence-based recommendations for the management of CAO.
    METHODS: A multidisciplinary expert panel developed key questions using the PICO (Patient, Intervention, Comparator, and Outcomes) format and conducted a systematic literature search using MEDLINE (PubMed) and the Cochrane Library. The panel screened references for inclusion and used vetted evaluation tools to assess the quality of included studies and extract data, and graded the level of evidence supporting each recommendation. A modified Delphi technique was used to reach consensus on recommendations.
    RESULTS: A total of 9,688 abstracts were reviewed, 150 full-text articles were assessed, and 31 studies were included in the analysis. One good practice statement and 10 graded recommendations were developed. The overall certainty of evidence was very low.
    CONCLUSIONS: Therapeutic bronchoscopy can improve the symptoms, quality of life, and survival of patients with malignant and non-malignant CAO. Multi-modality therapeutic options, including rigid bronchoscopy with general anesthesia, tumor/tissue debridement, ablation, dilation, and stent placement, should be utilized when appropriate. Therapeutic options and outcomes are dependent on the underlying etiology of CAO. A multidisciplinary approach and shared decision-making with the patient are strongly encouraged.
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  • 文章类型: Case Reports
    类癌综合征是由类癌肿瘤产生的不同激素引起的一系列体征和症状。很少,这些肿瘤可以转移到心脏并引起肿瘤的心脏受累。这项研究提出了一个非常罕见的继发性心脏肿瘤影响左心室的病例,该病例来自起源于小肠的转移性类癌,而没有类癌瓣膜性心脏病。
    Carcinoid syndrome is a constellation of signs and symptoms caused by different hormones produced by carcinoid tumors. Very rarely, those tumors can metastasize to the heart and cause cardiac involvement of the tumor. This study presents a very rare case of secondary cardiac tumor affecting the left ventricle from a metastatic carcinoid tumor originating from the small intestine without carcinoid valvular heart disease.
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  • 文章类型: Letter
    暂无摘要。
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  • 文章类型: Journal Article
    库欣综合征,以皮质醇血症为特征的临床状况,在某些患者中表现出明显的临床体征,并与循环皮质醇分泌有关。环状库欣综合征的临床表现可能是模糊的,其诊断通常具有挑战性。我们经历了一名72岁的女性,患有由肺部类癌肿瘤引起的环状ACTH依赖性库欣综合征。由于低谷期延长,诊断具有挑战性,而负责的病变最初身份不明。随后的随访计算机断层扫描显示肺部病变,通过肺动脉采样证实了该病变的异位ACTH分泌。尽管ACTH的分泌高峰期较短(约一周),手术切除肿瘤的免疫染色证实ACTH阳性。有趣的是,在高峰和低谷期间,储存的血浆嗜铬粒蛋白A水平均升高。评估该患者的经验促使我们研究血浆嗜铬粒蛋白A作为ACTH依赖性库欣综合征的诊断标志物的潜在用途。进行了一项回顾性研究,以确定血浆嗜铬粒蛋白A在3例异位ACTH综合征(EAS)患者中的疗效。包括本案,以及2018年至2021年期间到我院就诊的6名库欣病(CD)患者。值得注意的是,EAS患者的血浆嗜铬粒蛋白A水平高于CD患者。此外,在这种情况下,在低谷阶段的嗜铬粒蛋白A水平低于峰值阶段的嗜铬粒蛋白A水平,与CD患者相似。血浆嗜铬粒蛋白A水平的测量可以帮助区分EAS和CD。
    Cushing\'s syndrome, a clinical condition characterized by hypercortisolemia, exhibits distinct clinical signs and is associated with cyclic cortisol secretion in some patients. The clinical presentation of cyclic Cushing\'s syndrome can be ambiguous and its diagnosis is often challenging. We experienced a 72-year-old woman with cyclic ACTH-dependent Cushing\'s syndrome caused by a pulmonary carcinoid tumor. Diagnosis was challenging because of the extended trough periods, and the responsible lesion was initially unidentified. A subsequent follow-up computed tomography revealed a pulmonary lesion, and ectopic ACTH secretion from this lesion was confirmed by pulmonary artery sampling. Despite the short peak secretion period of ACTH (approximately one week), immunostaining of the surgically removed tumor confirmed ACTH positivity. Interestingly, stored plasma chromogranin A levels were elevated during both peak and trough periods. The experience in evaluating this patient prompted us to investigate the potential use of plasma chromogranin A as a diagnostic marker of ACTH-dependent Cushing\'s syndrome. A retrospective study was conducted to determine the efficacy of plasma chromogranin A in three patients with ectopic ACTH syndrome (EAS), including the present case, and six patients with Cushing\'s disease (CD) who visited our hospital between 2018 and 2021. Notably, plasma chromogranin A levels were higher in patients with EAS than in those with CD. Additionally, a chromogranin A level in the present case during the trough phase was lower than that in the peak phase, and was similar to those in CD patients. The measurement of plasma chromogranin A levels could aid in differentiating EAS from CD.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    中耳神经内分泌肿瘤(MeNET)是一种少见复发或转移的低度恶性肿瘤。这里,我们描述了一个29岁的男性,他患有MeNET,在10年内复发了3次,最终转移到大脑。病人接受了手术切除治疗,放射治疗,和化疗。然而,由于脑转移瘤紧密粘附在脑干上,肿瘤未完全切除.由于多发脑肿瘤切除后肿瘤破裂出血,严重的昏迷发展。最后,患者在最后一次手术后10个月死亡。据我们所知,这是一例MeNET多发性脑转移病例的首次报道。本案的特点表明,CK,SYN,增加了Ki67指数,ATRX可能是侵袭性MeNET的潜在生物标志物。脑转移性MeNET患者的生存期可以通过手术切除来延长,放射治疗,和化疗。还建议密切随访与复发相关的独特转移和生物标志物。
    Middle ear neuroendocrine tumor (MeNET) is a low-grade tumor with rare recurrence or metastasis. Here, we describe the case of a 29-year-old man who suffered from MeNET that recurred 3 times over 10 years and eventually metastasized to the brain. The patient was treated with surgical resection, radiotherapy, and chemotherapy. However, the tumor was not entirely removed as the brain metastatic tumor adhered tightly to the brainstem. Due to tumor rupture and bleeding after multiple brain tumor removal, profound coma developed. Finally, the patient died 10 months after the last surgery. To our knowledge, this is the first report of a MeNET case with multiple brain metastases. Characteristics of the present case indicate that CK, SYN, increased Ki67 index, and ATRX may be potential biomarkers of invasive MeNET. The survival of patients with brain metastatic MeNET may be extended by surgical resection, radiotherapy, and chemotherapy. Close follow-up of distinctive metastases and biomarkers related to recurrence is also suggested.
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