carcinoid tumour

类癌肿瘤
  • 文章类型: Case Reports
    Meckel的憩室,真正的憩室起源于胚胎发育过程中卵黄管的不完全闭合,很少出现类癌肿瘤。腹腔镜乙状结肠切除术治疗憩室炎后,Meckel憩室和类癌的共存是一种罕见的现象,文献中记录的案例有限。我们介绍了一例有高血压和憩室炎病史的74岁男性,他接受了腹腔镜乙状结肠切除术,原因是在结肠镜检查中发现息肉的增生异常和癌变。最初,患者的术后旅程顺利,恢复了规律的排便和良好的饮食进展。然而,他后来因腹痛和腹胀恶化而向急诊科就诊。由于穿孔和梗阻,影像学提示手术干预,从而鉴定出穿孔的Meckel憩室内的类癌肿瘤。这个案例突出了术后并发症的复杂挑战,尤其是在结肠切除术后出现了意外的Meckel憩室病理。非典型的介绍,在Meckel的憩室穿孔内有一个类癌肿瘤,强调术后评估腹部症状的重要性。
    Meckel\'s diverticulum, a true diverticulum originating from the incomplete closure of the vitelline duct during embryologic development, rarely presents with carcinoid tumors. The coexistence of a Meckel\'s diverticulum and carcinoid tumor following laparoscopic sigmoid colectomy for diverticulitis is an uncommon phenomenon, with limited documented cases in the literature. We present a case of a 74-year-old male with a past medical history of hypertension and diverticulitis who underwent a laparoscopic sigmoid colectomy for dysplastic and cancerous changes of a polyp revealed during a screening colonoscopy. Initially, the patient\'s postoperative journey was uneventful with the resumption of regular bowel movements and favorable diet progression. However, he later presented to the emergency department for worsening abdominal pain and distension. Imaging prompted surgical intervention due to perforation and obstruction, resulting in the identification of a carcinoid tumor within a perforated Meckel\'s diverticulum. This case highlights the intricate challenges of postoperative complications, particularly the unexpected emergence of Meckel\'s diverticulum pathology following a colectomy. The atypical presentation, featuring a carcinoid tumor within a perforated Meckel\'s diverticulum, underscores the importance of evaluating abdominal symptoms postoperatively.
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  • 文章类型: Case Reports
    弥漫性特发性肺神经内分泌细胞增生(DIPNECH),一种以前被忽视的罕见疾病,逐渐被认为是肺神经内分泌肿瘤的重要前兆状态。症状表现的非常阴险的发作使得DIPNECH的早期诊断在临床上几乎是不可能的。在这份报告中,我们介绍了一例持续且恶化的咳嗽超过5年的病例,伴有不同大小的打蜡和脱落的肺结节,最终在活检中被诊断为DIPNECH.然而,由于这些结节的位置和多样性,在这种情况下,手术切除不是一种选择.诊断检查包括成像和活检,管理选项,并详细讨论了DIPNECH的可能预后。本报告强调了DIPNECH作为一种临床实体的认识,在为表现为慢性持续咳嗽和相关肺结节的患者制定鉴别诊断的过程中,应注意。
    Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH), a rare disease previously overlooked, is gradually being recognized as an important precursor state to pulmonary neuroendocrine tumors. The very insidious onset of symptom presentation makes early diagnosis of DIPNECH almost impossible in clinical settings. In this report, we present a case of persistent and worsening cough for over five years with waxing and waning lung nodules of varying sizes which were eventually diagnosed as DIPNECH on biopsy. However, due to the location and the multiplicity of these nodules, surgical resection was not an option in this case. The diagnostic workup including imaging and biopsy, management options, and possible prognosis of DIPNECH are discussed in detail. This report highlights the growing recognition of DIPNECH as a clinical entity to be aware of during the formulation of a differential diagnosis for patients presenting with chronic unrelenting cough and associated lung nodules.
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  • 文章类型: Systematic Review
    小肠神经内分泌肿瘤(SI-NET)是最常见的小肠肿瘤。这些肿瘤的一个特别具有挑战性的子集是那些涉及肠系膜上动脉或静脉的肿瘤,手术的作用和可行性经常受到质疑。本系统综述旨在确定和评估用于这些复杂SI-NET的管理策略。确定的研究显示手术和多模式治疗的积极结果。
    Small intestinal neuroendocrine tumours (SI-NETs) are the most common small intestinal tumours. A particularly challenging subset of these tumours is those that involve the superior mesenteric artery or vein for which the role and feasibility of surgery are often questioned. This systematic review aimed to identify and evaluate the management strategies used for these complex SI-NETs. The identified studies showed positive outcomes with surgery and multimodality therapy.
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  • 文章类型: Journal Article
    背景:由神经内分泌肿瘤(NET)引起的类癌性心脏病(CHD)与由于瓣膜功能障碍和右侧心力衰竭引起的发病率和死亡率增加相关。本研究旨在评估NET患者CHD的患病率和一年发病率。肿瘤特征,实验室测量,和超声心动图检查结果进行评估以确定CHD表现的预测因子。
    方法:该研究由研究者发起,单心,前瞻性审判。纳入先前未诊断为CHD的NET患者,并进行了全面的胃肠病学和肿瘤学诊断。在基线和一年后进行超声心动图检查。
    结果:47名NET患者被纳入研究,其中64%表现出类癌综合征(CS)的临床特征。3例患者在基线时出现CHD,3例患者在随访期间出现心脏受累,基线时患病率为6%,一年内发病率为6.8%。羟基吲哚乙酸(5-HIAA)被鉴定为预测冠心病的发生(OR,1.004;95%CI,5-HIAA增加1.001-1.006),而嗜铬粒蛋白A(CGA),Kiel抗原67(Ki67%)无预测价值。与NET患者相比,在12个月的随访中,六名CHD患者显示出基线时右心直径更大,心肌性能指数(MPEI)值增加的趋势。
    结论:冠心病的基线患病率和一年发病率为6-7%。5-HIAA被鉴定为预测CHD发展的唯一标记。
    BACKGROUND: Carcinoid heart disease (CHD) caused by neuroendocrine tumours (NET) is associated with an increased morbidity and mortality due to valvular dysfunction and right sided heart failure. The present study aimed to assess the prevalence and one-year-incidence of CHD in NET patients. Tumour characteristics, laboratory measurements, and echocardiographic findings were evaluated to identify predictors of CHD manifestation.
    METHODS: The study was an investigator-initiated, monocentric, prospective trial. Patients with NET without previously diagnosed CHD were included and underwent comprehensive gastroenterological and oncological diagnostics. Echocardiographic examinations were performed at baseline and after one year.
    RESULTS: Forty-seven NET patients were enrolled into the study, 64% of them showed clinical features of a carcinoid syndrome (CS). Three patients presented with CHD at baseline and three patients developed cardiac involvement during the follow-up period corresponding to a prevalence of 6% at baseline and an incidence of 6.8% within one year. Hydroxyindoleacetic acid (5-HIAA) was identified to predict the occurrence of CHD (OR, 1.004; 95% CI, 1.001-1.006 for increase of 5-HIAA), while chromogranin A (CgA), and Kiel antigen 67 (Ki 67%) had no predictive value. Six patients with CHD at twelve-month follow-up revealed a tendency for larger right heart diameters and increased values of myocardial performance index (MPEI) at baseline compared to NET patients.
    CONCLUSIONS: The prevalence at baseline and one-year-incidence of CHD was 6-7%. 5-HIAA was identified as the only marker which predict the development of CHD.
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  • 文章类型: Case Reports
    血管球瘤是一种罕见的间叶性肿瘤,通常位于血管球状体周围,例如甲下区域(例如,手指甲和脚趾甲)。其他部位包括前臂,手腕,或树干。甚至罕见的是当这些肿瘤被发现在粘膜下层。在胃里,常见于胃窦。胃血管球瘤(GTT)通常是在诊断出其他胃肿瘤后偶然发现的,如胃肠道间质瘤(GIST)或类癌瘤。GGT的可变临床表现以及组织学是确认诊断的唯一方法的事实使GGT成为如此难以捉摸的肿瘤。我们的病例是一名出现体重减轻和反流的患者。进行食管胃十二指肠镜(EGD)和结肠镜检查,并推测诊断为类癌。初步病理提示类癌的诊断。病人最终做了胃大部切除术,并接受了活检标本的免疫组织化学染色,最终确认GGT的诊断。
    Glomus tumor is a rare mesenchymal tumor commonly located in the periphery of glomus bodies, such as the subungual regions (e.g., fingernails and toenails). Other locations include the forearm, wrist, or trunk. Even rare is when these tumors are found in the submucosa. In the stomach, it is commonly found at the gastric antrum. Gastric glomus tumors (GGTs) are often found incidentally after a presumption of other gastric tumors is diagnosed, such as gastrointestinal stromal tumors (GISTs) or carcinoid tumors. The variable clinical presentation of GGT and the fact that histology is the only way to confirm the diagnosis is what makes GGT such an elusive tumor. Our case is a patient that presented with weight loss and reflux. Esophagogastroduodenoscopy (EGD) and colonoscopy were done, and the diagnosis of carcinoid tumor was presumed. Preliminary pathology was suggestive of a diagnosis of carcinoid tumor. The patient eventually had a subtotal gastrectomy, and a biopsy with immunohistochemical staining of the specimen was received, finally confirming the diagnosis of a GGT.
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  • 文章类型: Journal Article
    神经内分泌肿瘤(NENs)是一组异质的罕见肿瘤,源自肽能神经元和能够分泌各种肽或胺的专门神经内分泌细胞。这些细胞可存在于内分泌组织中或扩散于消化或呼吸系统的组织中。本文回顾了NENs的特征,特别强调皮肤表现,如坏死性游走性红斑(NME),舌头发炎,角状唇炎,胰高血糖素瘤中的静脉血栓形成和脱发;“潮红”,“狮子脸”,糙皮病皮肤症状,类癌肿瘤中的“硬皮病样特征,无雷诺现象”。本文还介绍了皮肤神经内分泌肿瘤-默克尔细胞癌的临床表现。这项研究的目的是提请注意需要对患者进行精确和全面的诊断,特别强调皮肤病变作为神经内分泌肿瘤的启示者。这种管理允许早期实施适当的治疗。
    Neuroendocrine neoplasms (NENs) are a heterogeneous group of rare tumours derived from peptidergic neurons and specialized neuroendocrine cells capable of secreting various peptides or amines. These cells may be present in endocrine tissue or diffused in the tissues of the digestive or respiratory system. The article reviews the characteristic features of NENs, with particular emphasis on skin manifestations, such as necrolytic migratory erythema (NME), tongue inflammation, angular cheilitis, venous thrombosis and alopecia in glucagonoma; \"flushing\", \"lion face\", pellagra skin symptoms, \"scleroderma-like features without Raynaud\'s phenomenon\" in carcinoid tumours. The paper also presents the clinical picture of the neuroendocrine tumour of the skin - Merkel cell carcinoma. The aim of this study was to draw attention to the need for precise and comprehensive diagnosis of the patients, with particular emphasis on skin lesions as a revelator of neuroendocrine tumours. This management allows for the early implementation of appropriate treatment.
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  • 文章类型: Case Reports
    梅克尔憩室(MD),最常见的先天性小肠疾病,常表现为无痛性直肠出血和肠梗阻。有症状的MD的治疗包括切除病变,而与患者年龄无关;然而,在成人中切除无症状和偶然发现的MD仍然存在争议.一方面,由MD引起的并发症随着年龄的增长而减少,与预防性切除相比,获益率较低。另一方面,恶性肿瘤,如神经内分泌肿瘤,随着时间的推移,可能会从未处理的MD中产生。这可能导致预后不良的并发症,如肝脏或淋巴结转移。在这个案例报告中,我们描述了一名成年男性急性乙状结肠憩室炎剖腹探查术中偶然发现的Meckel憩室。后来的活检发现病变包含1级神经内分泌肿瘤。根据我们的文献综述结果,考虑到手术的低并发症风险以及恶性转化和进展的可能性,切除意外Meckel憩室是一种合理的方法.
    Meckel\'s diverticulum (MD), the most common congenital disease of the small bowel, commonly presents with symptoms of painless rectal bleeding and intestinal obstruction. The treatment of symptomatic MD involves resection of the lesion regardless of patient age; however, the excision of asymptomatic and incidentally identified MDs in adults remain controversial. On one hand, the complications arising from MDs decrease with age, leading to a lower benefit than risk ratio with prophylactic resection. On the other hand, malignancies, such as neuroendocrine tumors, may arise over time from untreated MDs. This can lead to poor prognostic complications, such as liver or lymph node metastases. In this case report, we describe an incidental Meckel\'s diverticulum discovered during an exploratory laparotomy for acute sigmoid diverticulitis in an adult male. Later biopsy findings discovered the lesion to contain a grade 1 neuroendocrine tumor. Based on our literature review findings, resection of the incidental Meckel\'s diverticulum was a reasonable approach given the low complication risks of the procedure and the possibility of malignant transformation and progression.
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  • 文章类型: Journal Article
    卵巢神经内分泌肿瘤是一种罕见且高度异质性的肿瘤。本研究旨在描述其人口统计学和临床病理特征,并确定其预后因素。
    2004年至2016年间诊断为卵巢神经内分泌肿瘤的399例患者的临床数据,流行病学,和最终结果(SEER)数据库进行回顾性分析。使用Kaplan-Meier方法绘制生存曲线,使用对数秩检验评估不同亚组之间的比较,和多变量Cox回归分析确定了独立的预后因素。
    不同组织学类型(类癌,神经内分泌癌和特殊类型类癌)占25.5%,96.1%和75.0%,分别(P<0.001)。多变量Cox分析显示,在类癌肿瘤中,高级FIGO阶段是唯一的预测因子。此外,在第二阶段之间没有观察到显著差异,III和IV使用对数秩检验。在神经内分泌癌中,高级FIGO阶段和高级分化是危险因素,而化疗是一个保护因素。在所有已知组织学分化状态的卵巢神经内分泌肿瘤中,不同组织学类型间无显著差异;只有高度分化是独立的危险因素,化疗是一个保护因素。
    FIGO晚期神经内分泌癌和类癌患者预后不良。神经内分泌癌的分化差表明生存期短,辅助化疗似乎是有效的。与其他已知因素相比,卵巢神经内分泌肿瘤的组织学分化是最有效的预后因素。一起来看,卵巢神经内分泌肿瘤未来可能比目前使用的基于组织学类型的分类更好地分为低级别或高级别。
    Ovarian neuroendocrine neoplasm is a rare and highly heterogeneous neoplasm. This study is aimed to describe its demographic and clinicopathological features and identify its prognostic factors.
    Clinical data of 399 patients diagnosed with ovarian neuroendocrine neoplasms between 2004 and 2016 in the Surveillance, Epidemiology, and End Results (SEER) database were analysed retrospectively. Survival curves were drawn using the Kaplan-Meier method, comparisons among different subgroups were evaluated using log-rank tests, and multivariate Cox regression analyses identified independent prognostic factors.
    The five-year survival rates of patients with different histological types (carcinoid tumour, neuroendocrine carcinoma and special type of carcinoid tumour) were 25.5%, 96.1% and 75.0%, respectively (P < 0.001). Multivariate Cox analysis revealed that in carcinoid tumours, advanced FIGO stage was the only predictor. Additionally, no significant difference was observed among stages II, III and IV using the log-rank test. In neuroendocrine carcinoma, an advanced FIGO stage and high-grade differentiation were risk factors, while chemotherapy was a protective factor. Among all ovarian neuroendocrine neoplasms with a known histological differentiation status, no significant difference was observed among different histological types; only high-grade differentiation was an independent risk factor, and chemotherapy was a protective factor.
    Patients with neuroendocrine carcinomas and carcinoid tumours of an advanced FIGO stage have a poor prognosis. Poor differentiation of neuroendocrine carcinomas indicates a short survival time, and adjuvant chemotherapy appears to be effective. Histological differentiation of ovarian neuroendocrine neoplasms is the most potent prognostic factor comparing to other known factors. Taken together, ovarian neuroendocrine neoplasms might be better classified as low- or high-grade ones rather than the currently used classification based on histological types in the future.
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  • 文章类型: Case Reports
    产生5-羟色胺的肿瘤是神经内分泌肿瘤的一个子集,当活跃时,可导致由血管活性物质如5-羟色胺的分泌引起的类癌综合征,组胺,和缓激肽.除了常见的潮红症状,腹泻,腹痛,和类癌心脏病,类癌综合征也已知会引起抑郁症和焦虑的精神症状,很少,精神病症状.该疾病的神经精神表现的治疗也因同时使用生长抑素类似物而复杂化,这也会影响总循环血清素水平,与上述精神疾病有关的神经递质。在这个案例报告中,我们将讨论一名64岁女性转移性类癌患者的精神病性抑郁症的治疗,以及我们使用沃替西汀治疗的理由。
    Serotonin-producing tumours are a subset of neuroendocrine tumours which, when active, can lead to carcinoid syndrome caused by the secretion of vasoactive substances such as serotonin, histamine, and bradykinins. In addition to the common symptoms of flushing, diarrhoea, abdominal pain, and carcinoid heart disease, carcinoid syndrome is also known to cause psychiatric symptoms of depression as well as anxiety and, very rarely, psychotic symptoms. The treatment of the neuropsychiatric manifestations of the disease is also complicated by the concurrent use of somatostatin analogues, which also affect total circulating serotonin levels, a neurotransmitter implicated in the aforementioned psychiatric disorders. In this case report, we will discuss the management of psychotic depression in a 64-year-old female patient with a metastatic carcinoid tumour and our rationale for the use of vortioxetine in her treatment.
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  • 文章类型: Case Reports
    类癌心脏病(CHD)患者卵圆孔未闭(PFO)可能会导致缺氧,这是由于存在大的右(R)到左(L)心内分流导致缺氧和临床病情恶化。经皮封堵PFO可使氧饱和度正常化,缓解症状,减少左侧心脏瓣膜受累。我们描述了一例70岁的女性患者,该患者每月进行奥曲肽输注,有小肠神经内分泌肿瘤病史,通过PFO表现为R至L心内分流继发的劳力性呼吸困难和缺氧恶化。决定用Amplatzer(普利茅斯,MN:Abbott)PFO封堵器装置,可使氧饱和度立即恢复正常并缓解呼吸困难。
    Patent foramen ovale (PFO) in patients with carcinoid heart disease (CHD) may result in hypoxia due to the presence of large right (R) to left (L) intracardiac shunts leading to hypoxia and worsening clinical condition. Percutaneous closure of the PFO can normalize oxygen saturation, relieve symptoms, and lessens left-sided heart valves involvement. We describe a case of a 70-year-old female patient with a history of small bowel neuroendocrine tumor on monthly octreotide infusion presented with worsening exertional dyspnea and hypoxia secondary to R to L intracardiac shunt through the PFO. The decision was made to close the PFO percutaneously with Amplatzer (Plymouth, MN: Abbott) PFO occluder device which resulted in immediate normalization of oxygen saturation and relief of her dyspnea.
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