Carcinoid tumor

类癌肿瘤
  • 文章类型: Case Reports
    该病例报告描述了一名患者,该患者担心闭环小肠梗阻(SBO)。在剖腹探查术中,切除了由于闭环阻塞引起的缺血性肠区,以及偶然发现的发炎的Meckel憩室(MD)。切除标本为分化良好的良性类癌,最大直径为0.6cm,侵入粘膜下层(pT1b和pN0)。在过去的几年里,对于在腹部探查中偶然发现的无症状MD的情况下的适当手术治疗,一直存在争议,但没有达成共识。分享此病例的目的是强调决策过程在治疗手术时偶然发现的腹内病理状况的患者中的重要性。
    This case report describes a patient who presented with concern for a closed-loop small bowel obstruction (SBO). During exploratory laparotomy, an area of ischemic bowel due to closed loop obstruction was resected, along with an incidentally discovered inflamed-appearing Meckel\'s diverticulum (MD). The resected specimen contained a well-differentiated carcinoid tumor of benign behavior with a maximum diameter of 0.6 cm, which invaded the submucosal layer (pT1b and pN0). Over the last several years, there has been a debate with little consensus regarding the proper surgical management in the case of an asymptomatic MD that is discovered incidentally during abdominal exploration. The intention of sharing this case is to underline the importance of the decision-making process in treating patients with this intraabdominal pathologic condition found incidentally at the time of surgery.
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    文章类型: Case Reports
    一名64岁的男子抱怨下腹痛和呕吐。CT扫描显示粘连性肠梗阻;因此,进行小肠切除术。组织学发现显示印戒细胞癌和低分化腺癌。我们进行了回盲肠切除术作为额外的手术。手术发现腹膜结节。组织学发现提示杯状细胞类癌伴有腹膜播散。给予mFOLFOX+贝伐单抗,手术后30个月未观察到进展。阑尾杯状细胞类癌少见,预后差。这里,我们报告了一例阑尾GCC,尽管腹膜播散,但仍获得了相对长期的生存.
    A 64-year-old man complained of lower abdominal pain and vomiting. The CT scan showed adhesional ileus; therefore, small bowel resection procedure was performed. Histological findings showed signet-ring cell carcinoma and poorly differentiated adenocarcinoma. We performed ileocecal resection as an additional surgery. The operative findings revealed peritoneal nodules. The histological findings suggested goblet cell carcinoid with peritoneal dissemination. mFOLFOX+bevacizumab was administered, and no progression was observed for 30 months after the surgery. Appendiceal goblet cell carcinoid is rare and its prognosis is poor. Here, we report a case of appendiceal GCC that achieved a relatively long-term survival despite peritoneal dissemination.
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  • 文章类型: Journal Article
    附录,被认为是一个残留和一次性的器官,长期以来一直被视为腹部肿瘤的来源。在阑尾肿瘤中,杯状细胞腺癌(GCA)是一种罕见的原发性上皮性肿瘤,近年来经历了多次的名称变化和分类,增加了围绕这个独特的苯丙胺肿瘤的混乱。该实体以前被称为杯状细胞类癌和腺癌前杯状细胞类癌。这篇综述文章提供了关于病理学的最新信息,命名法,以及最近的分类系统,重点是2019年世界卫生组织肿瘤分类,3级分级系统1.
    Appendix, considered a vestigial and disposable organ, has been long neglected as a source of abdominal tumors. Among the appendiceal tumors, goblet cell adenocarcinoma (GCA) is a rare primary epithelial neoplasm which has undergone multiple name changes and classifications in recent years, adding to confusion surrounding this unique amphicrine tumor. This entity was previously known as goblet cell carcinoid and adenocarcinoma ex goblet cell carcinoid. This review article provides an update on pathology, nomenclature, and recent classification systems with emphasis on 2019 World Health Organization Classification of Tumors, 3-tiered grading system.1.
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  • 文章类型: Journal Article
    一名80多岁的男子,长期患有左侧喉咙不适,表现为张力减退和吞咽困难。灵活的鼻内窥镜检查显示左声门上有一个声门上肿块。在组织病理学检查中,紧急的显微喉镜检查和活检显示声门上喉部肿瘤与非典型类癌一致。头颈部多学科团队讨论导致选择消融性二氧化碳激光治疗作为主要治疗方法。两年后,局部复发,提示第二次激光切除和选择性颈清扫术。3年后进行了进一步的激光切除术,以再次复发。尽管当地控制部门最初做出了令人鼓舞的反应,初次治疗后4年,有实质性进展,有多个皮肤和皮下转移,并在不久后进一步局部复发.姑息性放疗对皮肤转移提供了部分益处,过渡到最好的支持性护理。他在最初出现症状10年后死亡,主要是由于癌症进展。
    A man in his 80s with long-standing left-sided throat discomfort presented with hypophonia and odynophagia. A flexible nasoendoscopy revealed a supraglottic mass in the left arytenoid. An urgent microlaryngoscopy and biopsy demonstrated a supraglottic laryngeal tumour consistent with atypical carcinoid on histopathological examination. Head and neck multidisciplinary team discussions led to the selection of ablative carbon dioxide laser therapy as primary treatment. Two years later, there was local recurrence, prompting a second laser resection and selective neck dissection. Further laser resection was performed 3 years later for another recurrence. Despite an initial encouraging response with local control, 4 years after the initial treatment, there was substantial progression with multiple cutaneous and subcutaneous metastases and further local recurrence soon after. Palliative radiotherapy to the cutaneous metastases provided partial benefit, transitioning to best supportive care. He died 10 years after the initial onset of symptoms, primarily due to cancer progression.
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  • 文章类型: Case Reports
    肠切除术后回肠穿孔是一种罕见且可能危及生命的并发症。我们介绍了一个60多岁的女性的独特病例,有阑尾类癌病史,做了右半结肠切除术.正电子发射断层扫描和监视CT显示正常的手术改变,没有复发的恶性肿瘤。术后三年,她出现了严重的腹痛。CT显示回肠结肠吻合术的回肠壁穿孔。她接受了紧急切除和重复回肠结肠吻合术。我们得出的结论是,患者患有吻合口的亚临床缺血,3年后最终进展为穿孔。我们讨论了有关小肠吻合口穿孔及其相关危险因素的文献综述。我们的病例和文献综述强调了在有肠癌病史的术后患者中考虑延迟吻合口漏的重要性。炎症性肠病,Roux-en-Y肠肠造口术或左右吻合术。
    Late perforation of the ileum is a rare and potentially life-threatening complication following intestinal resection. We present a unique case of a woman in her 60s with a history of appendiceal carcinoid tumour, who underwent a right hemicolectomy. Positron emission tomography and surveillance CTs showed normal surgical changes and no recurrent malignancy. Three years postoperatively, she presented with severe abdominal pain. CT revealed a perforation along the ileal wall of the ileocolonic anastomosis. She underwent emergent resection and repeat ileocolonic anastomosis. We conclude that the patient had subclinical ischaemia of the anastomosis, which eventually progressed to perforation 3 years later. We discuss a literature review on late small intestinal anastomotic perforations and their associated risk factors. Our case and literature review emphasise the importance of considering delayed anastomotic leak in postoperative patients with a history of intestinal cancer, inflammatory bowel disease, Roux-en-Y enteroenterostomy or side-to-side anastomosis.
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  • 文章类型: Case Reports
    异位促肾上腺皮质激素(EAS)通常与小细胞肺癌有关,但由多种肿瘤引起。在大约五分之一的案例中,原因仍未查明。过度促肾上腺皮质激素(ACTH)导致库欣综合征,这种表现可能是由于生化紊乱,如低钾血症和高血糖。或者,它可能表现为继发性症状,如体重增加,高血压,皮肤变薄,腹部纹,和/或精神病表现。在确认过量皮质醇和ACTH水平后,通过动态测试确定诊断。然后使用成像来识别激素活性病变。用类固醇生成抑制剂控制皮质醇增多是进行确定性治疗之前的第一步。理想情况下,肿瘤切除,如果可能,但是在不适合治愈性手术的情况下考虑双侧肾上腺切除术。
    Ectopic adrenocorticotropic secretion (EAS) is classically related to small-cell lung cancer but is caused by a wide variety of tumors. In approximately one-fifth of cases, the cause remains unidentified. Excess adrenocorticotropic hormone (ACTH) leads to Cushing\'s syndrome, and the presentation can be due to biochemical derangements such as hypokalemia and hyperglycemia. Alternatively, it may manifest with secondary symptoms such as weight gain, hypertension, skin thinning, abdominal striae, and/or psychotic manifestations. The diagnosis is established through dynamic testing after confirming excess cortisol and ACTH levels. Imaging is then used to identify the hormonally active lesion. Controlling hypercortisolism with steroidogenesis inhibitors is the initial step before proceeding to definitive treatment. Ideally, tumor resection, if possible, but bilateral adrenalectomies are considered in cases not amenable to curative surgery.
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  • 文章类型: Case Reports
    背景:类癌是罕见的神经内分泌恶性肿瘤,在我们中心有越来越多的表现。类癌肿瘤的发病率约为每100,000人中2.5至5例,其中约50%发展为类癌综合征。一旦类癌综合征发展起来,可发生类癌心肌病。类癌心脏病(CaHD)仍然是一种严重且罕见的并发症,其发病率和死亡率显着增加。虽然类癌肿瘤已经被认识和研究了几年,关于麻醉管理和围手术期的数据仍然很少。
    方法:我们描述了一例44岁的高加索女性,其异常表现为左侧CaHD,伴有回肠神经内分泌肿瘤和肝转移。我们的术前生长抑素给药方案,限制心脏损伤。维持稳定的血液动力学,使用平衡麻醉技术,同时对病理学有很好的理解,在麻醉的成功管理中发挥了重要作用。这个病例报告让我们介绍我们的决策算法在我们的三级医院的这种类型的病理的管理,圣吕克诊所大学。
    结论:尽管数据很少,通过有效的血流动力学监测和对病理生理学的充分了解,可以安全地对类癌患者进行麻醉管理。在转诊中心的奥曲肽管理和多学科咨询的明确机构算法的知识和应用对于这些患者的管理至关重要。
    BACKGROUND: Carcinoid tumors are rare neuroendocrine malignancies presenting in an increasing number in our center. The incidence of carcinoid tumors is approximatively between 2.5 and 5 cases per 100,000 people of whom about 50% develop carcinoid syndrome. Once the carcinoid syndrome has developed, a carcinoid cardiomyopathy can occur. Carcinoid heart disease (CaHD) remains a serious and rare complication associated with a significant increase in morbidity and mortality. Although carcinoid tumors have been known and studied for several years, there are still scarce data on the anesthetic management and the peri operative period.
    METHODS: We describe a case of a Caucasian 44-year-old woman with an unusual presentation of left CaHD with an ileal neuroendocrine tumor and liver metastases. Our preoperative somatostatin administration protocol, limit the cardiac damage. The maintenance of stable hemodynamics, the use of balanced anesthetic technique, all along with a good understanding of the pathology, played a major role in the successful management of anesthesia. This case report allows us to introduce our decision algorithm for the management of this type of pathology in our tertiary hospital, Cliniques Universitaires Saint-Luc.
    CONCLUSIONS: Despite the paucity of data, anesthetic management of patients with carcinoid tumor can be safely performed with effective hemodynamic monitoring and a good understanding of the pathophysiology. Knowledge and application of a clear institutional algorithm for octreotide administration and multidisciplinary consultation at a referral center are essential for the management of these patients.
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  • 文章类型: Journal Article
    目前,关于一期肺类癌(PC)肿瘤的手术范围尚无共识,其中包括典型类癌(TC)和非典型类癌(AC)肿瘤。亚叶切除包括节段性切除和楔形切除;前者被认为是一种更适合肿瘤治疗的解剖切除类型。因此,两种手术方式对患者生存时间的影响是否存在差异,有待进一步验证。使用倾向评分匹配(PSM)。主要终点是癌症特异性生存期(CSS)和总生存期(OS)时间。通过Kaplan-Meier方法和对数秩检验分析生存差异。在TC或AC肿瘤组中,PSM后肺叶下切除术和肺叶切除术组之间的生存率均无明显差异(均p>0.05)。共有1680例患者接受了肺楔形切除术(TC:n=1547,AC:n=133),398例患者接受了节段性切除术(TC:n=365,AC:n=33)。PSM之后,生存率没有统计学上的显著差异,无论OS或CSS是否被认为是主要终点(OS:p=0.337;CSS:p=0.470)。此外,在组织学基础上的不同亚组分析中,与楔形切除术相比,节段切除术没有延长患者的生存时间,年龄,和肿瘤大小(均p>0.05)。最后,通过多变量Cox分析获得了相同的结果(OS:p=0.153;HR=1.21;CSS:p=0.351,HR=1.32).对于早期典型或非典型的肺类癌患者,可考虑进行肺叶下切除术。前提是进行严格的淋巴结评估。如果肿瘤远离肺门,根据肿瘤的具体位置和患者的临床状况,可以进行段切除术或楔形切除术。
    Currently, there is no consensus regarding the extent of surgery for stage I pulmonary carcinoid (PC) tumors, which encompass typical carcinoid (TC) and atypical carcinoid (AC) tumors. Sublobar resection includes segmental resection and wedge resection; the former is regarded as a type of anatomical resection that is better suited for tumor treatment. Therefore, it needs to be further verified whether differences exist in the effects of the two surgical methods on the survival time of patients. Propensity score matching (PSM) was used. The primary endpoints were cancer-specific survival (CSS) and overall survival (OS) time. Survival differences were analyzed via the Kaplan-Meier method and the log-rank test. There was no significant difference in survival between the sublobar resection and lobectomy groups after PSM in either the TC or AC tumor groups (all p > 0.05). A total of 1680 patients underwent pulmonary wedge resection (TC: n = 1547, AC: n = 133), and 398 patients underwent segmental resection (TC: n = 365, AC: n = 33). After PSM, there were no statistically significant differences in survival, regardless of whether OS or CSS was considered the primary endpoint (OS: p = 0.337; CSS: p = 0.470). Furthermore, segmental resection did not prolong patient survival time compared with wedge resection in different subgroup analyses on the basis of histology, age, and tumor size (all p > 0.05). Finally, the same results were obtained via multivariate Cox analysis (OS: p = 0.153; HR = 1.21; CSS: p = 0.351, HR = 1.32). Sublobar resection could be considered for patients with early-stage typical or atypical pulmonary carcinoid, provided that a rigorous lymph node evaluation is conducted. If the tumor is distant from the pulmonary hilum, either segmentectomy or wedge resection may be performed depending on the specific location of the tumor and the clinical condition of the patient.
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  • 文章类型: Journal Article
    肺神经内分泌肿瘤(NENs)是一类以神经内分泌(NE)分化为特征的多样化肿瘤。在肺NENs中,肺类癌(LC)是一种罕见的肿瘤,具有独特的特征。最近的研究强调了转录因子(TFs)在建立肺NENs如小细胞肺癌基因表达程序中的重要性。然而,控制LC基因表达的TFs在很大程度上是未知的。在这项研究中,我们报告了称为Prospero同源盒蛋白1(PROX1)的TF在LC中的表达和潜在功能。公开可用的转录组数据表明PROX1在LC组织中高度表达,通过组织微阵列上的免疫组织化学分析证实了这一点。PROX1的敲低不影响LC衍生细胞系的细胞活力,NCI-H727。同时,转录组分析显示,PROX1敲低改变了NE分化相关基因的表达。ASCL1,CHGA,CALCA,和LINC00261被认为是PROX1的下游基因。这些发现表明,PROX1可能通过调节关键靶基因的表达在LC的NE身份中起重要作用。
    Lung neuroendocrine neoplasms (NENs) are a diverse group of tumors characterized by neuroendocrine (NE) differentiation. Among lung NENs, lung carcinoid (LC) is a rare tumor with unique characteristics. Recent research has highlighted the importance of transcription factors (TFs) in establishing gene expression programs in lung NENs such as small cell lung carcinoma. However, the TFs that control the gene expression of LC are largely unknown. In this study, we report the expression and potential function of a TF called Prospero homeobox protein1 (PROX1) in LC. Publicly available transcriptome data suggested that PROX1 was highly expressed in LC tissues, which was confirmed by immunohistochemical analysis on a tissue microarray. Knockdown of PROX1 did not impact the cellular viability of an LC-derived cell line, NCI-H727. Meanwhile, transcriptome analysis revealed that PROX1 knockdown altered the expression of genes involved in NE differentiation. ASCL1, CHGA, CALCA, and LINC00261 were suggested as downstream genes of PROX1. These findings indicate that PROX1 may play an important role in the NE identity of LC by regulating the expression of key target genes.
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  • 文章类型: Journal Article
    肺类癌(LCs)是一种起源于支气管肺系统的神经内分泌肿瘤(NET)。LC占所有NET的20-25%,约占肺癌的1-2%。鉴于NETs的高度血管化性质及其过度表达血管生长因子受体(VEGFR)的趋势,抑制血管生成似乎是减缓肿瘤生长和扩散的潜在治疗靶点。本研究评估了阿西替尼(AXI)的长期抗肿瘤活性和相关机制。一种VEGFR靶向药物,在LC细胞系中。
    将三种LC细胞系(NCI-H727、UMC-11和NCI-H835)与它们各自的EC50AXI浓度一起孵育6天。在孵化结束时,进行FACS实验和Western印迹分析以检查细胞周期的变化和凋亡的激活。添加了显微镜分析以描述存在时衰老和有丝分裂突变的机制。
    AXI对LC细胞系的主要作用是通过间接DNA损伤阻止肿瘤生长。值得注意的是,AXI在细胞系中以不同的方式触发这种反应,如诱导衰老或有丝分裂灾难。当DNA损伤减轻时,该药物似乎失去了功效,如在NCI-H835细胞中观察到的。
    AXI在LC肿瘤细胞中影响细胞活力和增殖的能力凸显了其作为治疗剂的潜力。DNA损伤的作用以及随之而来的衰老激活似乎是AXI发挥其功能的先决条件。
    UNASSIGNED: Lung carcinoids (LCs) are a type of neuroendocrine tumor (NET) that originate in the bronchopulmonary tract. LCs account for 20-25% of all NETs and approximately 1-2% of lung cancers. Given the highly vascularized nature of NETs and their tendency to overexpress vascular growth factor receptors (VEGFR), inhibiting angiogenesis appears as a potential therapeutic target in slowing down tumor growth and spread. This study evaluated the long-term antitumor activity and related mechanisms of axitinib (AXI), a VEGFR-targeting drug, in LC cell lines.
    UNASSIGNED: Three LC cell lines (NCI-H727, UMC-11 and NCI-H835) were incubated with their respective EC50 AXI concentrations for 6 days. At the end of the incubation, FACS experiments and Western blot analyses were performed to examine changes in the cell cycle and the activation of apoptosis. Microscopy analyses were added to describe the mechanisms of senescence and mitotic catastrophe when present.
    UNASSIGNED: The primary effect of AXI on LC cell lines is to arrest tumor growth through an indirect DNA damage. Notably, AXI triggers this response in diverse manners among the cell lines, such as inducing senescence or mitotic catastrophe. The drug seems to lose its efficacy when the DNA damage is mitigated, as observed in NCI-H835 cells.
    UNASSIGNED: The ability of AXI to affect cell viability and proliferation in LC tumor cells highlights its potential as a therapeutic agent. The role of DNA damage and the consequent activation of senescence seem to be a prerequisite for AXI to exert its function.
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