neuroendocrine tumor

神经内分泌肿瘤
  • 文章类型: Case Reports
    Although neuroendocrine tumors (NETs) can occur in any organ, the majority of them occur in the gastrointestinal (GI) tract. We present the case of a 27-year-old female who presented with ascites. She underwent an ascitic fluid analysis, an esophagogastroduodenoscopy (EGDscopy) with biopsies, and a positron emission tomography (PET) scan, all of which culminated in a diagnosis of a poorly differentiated gastric NET (small cell type) with peritoneal metastasis. She was treated with cisplatin and etoposide. Depending on the differentiation and grade, NETs can manifest in a variety of ways. Definitive diagnosis requires histopathological examination and immunostaining. For smaller well-differentiated NETs, management is either endoscopic or surgical resection. For neuroendocrine carcinomas with metastasis, chemotherapy and symptomatic management are advised. This case report highlights the rare presentation of a neuroendocrine carcinoma as well as discusses its diagnostic approach and possible treatment options.
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  • 文章类型: Journal Article
    甲状腺起源的副神经节瘤非常罕见,并且缺乏有关其管理的既定准则。
    提出了一个案例研究,并对文献进行了回顾。
    诊断副神经节瘤需要多种诊断方法,包括24小时测量间肾上腺素或儿茶酚胺,使用MR或CT扫描的解剖成像,和使用MIBG或18F-DOPAPET/CT扫描的功能成像。此外,副神经节瘤上存在生长抑素受体,奥曲肽扫描如68GaDOTATATEPET/CT扫描的使用预计将很快增加.喉副神经节瘤的主要治疗方法是手术干预,旨在实现最佳的肿瘤切除,同时保留尽可能高的喉功能水平。鉴于转移和复发的风险较低,不应进行选择性颈淋巴结清扫术。尽管副神经节瘤的复发率和转移率很低,通过诊所访问继续监测,生化检测,成像仍然是必要的。此外,后续工作还应考虑对与副神经节瘤相关的关键基因进行基因检测.
    尽管关于甲状腺副神经节瘤的存在仍存在争议,尽管如此,它仍可以归类为喉神经节旁瘤的亚型。所有高血管甲状腺结节都需要考虑甲状腺相关的副神经节瘤。
    UNASSIGNED: Thyroid-originated paragangliomas are very uncommon, and there is a lack of established guidelines regarding their management.
    UNASSIGNED: A case study was presented, and a review of the literature was conducted.
    UNASSIGNED: Diagnosing a paraganglioma requires multiple diagnostic methods, including a 24-h measurement of metanephrines or catecholamines, anatomical imaging using magnetic resonance or computed tomography (CT) scans, and functional imaging using metaiodobenzylguanidine or 18F-DOPA PET/CT scans. Additionally, with the presence of somatostatin receptors on paragangliomas, the use of octreotide scans such as a 68Ga DOTATATE PET/CT scan is expected to increase soon. The primary treatment for laryngeal paragangliomas is surgical intervention aimed at achieving optimal tumor removal while retaining the highest possible level of laryngeal function. One should not do elective neck dissection given the low risk of metastasis and recurrence. Although the rate of recurrence and metastasis for paragangliomas is low, continued monitoring through clinic visits, biochemical testing, and imaging is still necessary. Furthermore, follow-up efforts should also consider genetic testing of the critical genes associated with paragangliomas.
    UNASSIGNED: Although there is still debate regarding the existence of thyroid paraganglioma, it can nonetheless be classified as a subtype of laryngeal paragangliomas. All hypervascular thyroid nodules require the consideration of thyroid-associated paragangliomas.
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  • 文章类型: Case Reports
    小肠神经内分泌肿瘤(NETs)是罕见的,但由于其具有挑战性的诊断途径和潜在的潜在进展,具有临床意义。在这些情况下,早期识别对于有效治疗和改善预后至关重要。
    方法:这里,我们介绍了一例没有明显病史的75岁患者,其急性表现为弥漫性腹痛,呕吐,和肠梗阻的迹象.诊断检查,包括CT成像,显示回肠远端神经内分泌肿块伴肠系膜淋巴结肿大,需要紧急手术干预。
    结论:该病例强调了与小肠NET相关的诊断复杂性和治疗挑战。手术切除和细致的淋巴结清扫仍然是治疗的基石,旨在实现完整的肿瘤切除和最佳的疾病控制。考虑到患者的临床过程,讨论了成像方式和生化标志物在指导临床决策和术后管理策略中的作用。
    结论:及时识别和干预在小肠NET的管理中至关重要,考虑到他们可能出现迟发症状和非特异性症状。尽管在这种情况下强调了诊断和程序上的挑战,对于小肠NETs患者,早期手术干预和全面随访是取得良好结局和将复发风险降至最低的关键.
    UNASSIGNED: Neuroendocrine tumors (NETs) of the small bowel are rare but clinically significant due to their challenging diagnostic pathways and potential for insidious progression. Early identification is critical for effective management and improved prognosis in these cases.
    METHODS: Here, we present a case of a 75-year-old patient with no significant medical history who presented acutely with diffuse abdominal pain, vomiting, and signs of bowel obstruction. Diagnostic workup, including CT imaging, revealed a distal ileal neuroendocrine mass with mesenteric lymphadenopathy, necessitating urgent surgical intervention.
    CONCLUSIONS: This case underscores the diagnostic complexities and therapeutic challenges associated with small bowel NETs. Surgical resection with meticulous lymph node dissection remains the cornerstone of treatment, aimed at achieving complete tumor excision and optimal disease control. The role of imaging modalities and biochemical markers in guiding clinical decisions and postoperative management strategies is discussed considering the patient\'s clinical course.
    CONCLUSIONS: Timely recognition and intervention are crucial in the management of small bowel NETs, given their potential for late presentation and nonspecific symptoms. Despite diagnostic and procedural challenges highlighted in this case, early surgical intervention and comprehensive follow-up are essential for achieving favorable outcomes and minimizing recurrence risks in patients with small bowel NETs.
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  • 文章类型: Journal Article
    胰腺囊性病变(PCL)由于其发病率增加以及横截面成像和内窥镜超声引导的细针抽吸(EUS-FNA)的局限性而提出了诊断挑战。EUS引导通过穿刺活检(EUS-TTNB)已成为提高囊肿类型确定和肿瘤危险分层准确性的有希望的工具。EUS-TTNB表现出优于EUS-FNA的诊断性能,提供重要的术前信息,这些信息可以显着影响患者管理并减少不必要的手术。然而,该程序有风险,总体不良事件发生率约为9%。预防措施和进一步的前瞻性研究对于优化其安全性和有效性至关重要。这篇综述强调了EUS-TTNB在增强PCL患者的诊断和管理方法方面的潜力。它检查了EUS-TTNB的当前状态,包括可用的设备,适应症,程序技术,标本处理,诊断产量,临床影响,和相关的不良事件。
    Pancreatic cystic lesions (PCLs) pose a diagnostic challenge due to their increasing incidence and the limitations of cross-sectional imaging and endoscopic-ultrasound-guided fine-needle aspiration (EUS-FNA). EUS-guided through the needle biopsy (EUS-TTNB) has emerged as a promising tool for improving the accuracy of cyst type determination and neoplastic risk stratification. EUS-TTNB demonstrates superior diagnostic performance over EUS-FNA, providing critical preoperative information that can significantly influence patient management and reduce unnecessary surgeries. However, the procedure has risks, with an overall adverse event rate of approximately 9%. Preventive measures and further prospective studies are essential to optimize its safety and efficacy. This review highlights the potential of EUS-TTNB to enhance the diagnostic and management approaches for patients with PCLs. It examines the current state of EUS-TTNB, including available devices, indications, procedural techniques, specimen handling, diagnostic yield, clinical impact, and associated adverse events.
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  • 文章类型: Journal Article
    基本原理:评估肽受体放射性核素治疗(PRRT)在转移性神经内分泌肿瘤(mNETs)患者中的长期安全性和有效性。方法:回顾性分析在2018年4月4日至2022年2月2日之间接受177Lu-DOTATATEPRRT且有或没有先前TAE的mNET患者。最近的临床,成像,和实验室发现,包括肝脏常见不良事件术语标准v5.0,与PRRT前进行了比较。结果:171例患者(95M,76F,中位年龄=66),不同主要部位的mNET(9个前肠,中肠100,9后肠,44胰腺,9未知)接受至少1个周期的PRRT,至少6个月的随访,其中110名患者未栓塞,61名患者既往有TAE。中位随访时间为22个月(范围:6-43)。有TAE的患者平均肝肿瘤负荷高于没有TAE的患者;然而,差异无统计学意义(p=0.06)。在先前接受TAE和未接受TAE的患者中,G3或G4肝毒性的发生率没有显着差异(分别为p=0.548和p=0.999)。TAE初治患者的无肝进展生存期为22.9个月,1、2和3例TAE治疗患者的无肝进展生存期为25.7、20.2和12.8个月。分别。结论:肽受体放射性核素治疗mNET经动脉缓慢栓塞是安全有效的。
    Rationale: Evaluating the long-term safety and efficacy of peptide receptor radionuclide therapy (PRRT) in patients with metastatic neuroendocrine tumors (mNETs) who have undergone prior bland hepatic transarterial embolization (TAE). Methods: Retrospective review of mNET patients who received PRRT with 177Lu-DOTATATE between 4/2018 and 02/2022 with and without prior TAE. The most recent clinical, imaging, and laboratory findings, including hepatic Common Terminology Criteria for Adverse Events v5.0, were compared to pre-PRRT. Results: 171 patients (95 M, 76 F, median age = 66) with mNET of different primary sites (9 foregut, 100 midgut, 9 hindgut, 44 pancreas, 9 unknown) received at least 1 cycle of PRRT with at least 6 months of follow-up, 110 of whom were embolization-naïve and 61 who had prior TAE. The median follow up was 22 months (range: 6-43). Patients with prior TAE had higher liver tumor burden on average than patients without prior TAE; however, the difference was not statistically significant (p = 0.06). There was no significant difference in the rates of G3 or G4 hepatotoxicity (p = 0.548 and p = 0.999, respectively) in patients who underwent prior TAE and those who were TAE-naïve. The hepatic progression-free survival was 22.9 months in TAE-naïve patients and 25.7, 20.2, and 12.8 months in patients with 1, 2, and 3 prior TAE treatments, respectively. Conclusion: Peptide receptor radionuclide therapy following transarterial bland embolization for mNET is safe and effective.
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  • 文章类型: Case Reports
    偶然发现阑尾的神经内分泌肿瘤(NETs)很少见,经常在手术中发现其他条件。该报告强调了在结直肠手术中进行彻底病理评估的重要性。
    方法:一名48岁男性出现间歇性腹痛,减肥,改变了排便习惯.结肠镜和活检证实右结肠腺癌。患者接受了右半结肠切除术。组织病理学检查显示升结肠中分化腺癌(pT3N2b)和阑尾尖端偶然的0.8cm高分化NET。NET为低级别(G1),无淋巴管浸润或转移。患者接受奥沙利铂和卡培他滨辅助化疗治疗结肠腺癌。由于其低风险特征,附带NET没有改变化疗方案。
    结论:虽然,NET的存在并没有改变化疗方案,鉴于其体积小、档次低的性质。然而,这种偶然肿瘤的发现凸显了对勤奋随访的必要性,因为患有同步肿瘤的患者可能会增加复发或发展为新的恶性肿瘤的风险。
    结论:该病例强调了在结直肠手术中进行细致的病理检查的重要性,因为偶然发现会显著影响患者管理和随访.尽管小型阑尾NET具有典型的惰性,它们的检测对于准确分期和适当的患者护理至关重要。
    UNASSIGNED: Incidental detection of neuroendocrine tumors (NETs) of the appendix is rare, often discovered during surgeries for other conditions. This report emphasizes the importance of thorough pathological evaluations in colorectal surgeries.
    METHODS: A 48-year-old male presented with intermittent abdominal pain, weight loss, and altered bowel habits. Colonoscopy and biopsy confirmed adenocarcinoma of the right colon. The patient underwent a right hemicolectomy. Histopathological examination revealed a moderately differentiated adenocarcinoma of the ascending colon (pT3N2b) and an incidental 0.8 cm well-differentiated NET at the tip of the appendix. The NET was low-grade (G1) with no lymphovascular invasion or metastasis. The patient received adjuvant chemotherapy with oxaliplatin and capecitabine for the colonic adenocarcinoma. The incidental NET did not alter the chemotherapy regimen due to its low-risk features.
    CONCLUSIONS: Although, the presence of the NET did not alter the chemotherapy regimen, given its small size and low-grade nature. However, the discovery of this incidental tumor highlights the need for diligent follow-up, as patients with synchronous tumors may have an increased risk of recurrence or development of new malignancies.
    CONCLUSIONS: This case highlights the significance of meticulous pathological examination in colorectal surgeries, as incidental findings can significantly impact patient management and follow-up. Despite the typically indolent nature of small appendiceal NETs, their detection is crucial for accurate staging and appropriate patient care.
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  • 文章类型: Journal Article
    胰腺神经内分泌肿瘤(NETs)是罕见的肿瘤,由于其缓慢的病程,对诊断和治疗提出了复杂的挑战。在过去的二十年中,胰腺神经内分泌肿瘤的发病率显着增加。目前有限数量的胰腺神经内分泌细胞系可用于该研究。这里,我们展示了3D-iNETORION,一种新的三维(球体)细胞系,分离人胰腺神经内分泌肿瘤肝转移。在过去的几年中,三维生长(3D)癌细胞系已经引起了人们的兴趣,因为3D癌细胞系更好地概括了肿瘤的体内结构,更适用于体外和体内实验。当嵌入基质胶基质并表达突触素和EpCAM时,3D-iNETORION癌细胞系显示出形成肿瘤球的高潜力。癌细胞系的电子显微镜分析证明存在致密的神经分泌颗粒。当异种移植到无胸腺小鼠中时,3D-iNETORION细胞产生缓慢生长的肿瘤,嗜铬粒蛋白和突触素呈阳性。人类核心外显子组分析显示3DiNETORION细胞系保留了在原始肿瘤中检测到的遗传畸变谱。总之,我们新开发的神经内分泌癌细胞系可以被认为是体外和体内实验的新研究工具。
    Neuroendocrine tumors (NETs) of the pancreas are rare neoplasms that present complex challenges to diagnosis and treatment due to their indolent course. The incidence of pancreatic neuroendocrine tumors has increased significantly over the past two decades. A limited number of pancreatic neuroendocrine cell lines are currently available for the research. Here, we present 3D-iNET ORION, a novel 3-dimensional (spheroid) cell line, isolated from human pancreatic neuroendocrine tumor liver metastasis. Three-dimensionally grown (3D) cancer cell lines have gained interest over the past years as 3D cancer cell lines better recapitulate the in vivo structure of tumors, and are more suitable for in vitro and in vivo experiments. 3D-iNET ORION cancer cell line showed high potential to form tumorspheres when embedded in Matrigel matrix and expresses synaptophysin and EpCAM. Electron microscopy analysis of cancer cell line proved the presence of dense neurosecretory granules. When xenografted into athymic mice, 3D-iNET ORION cells produce slow-growing tumors, positive for chromogranin and synaptophysin. Human Core Exome Panel Analysis has shown that 3DiNET ORION cell line retains the genetic aberration profile detected in the original tumor. In conclusion, our newly developed neuroendocrine cancer cell line can be considered as a new research tool for in vitro and in vivo experiments.
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  • 文章类型: Journal Article
    背景:颈动脉体瘤是颈动脉分叉附近罕见的神经内分泌生长。虽然有些人提倡术前栓塞以减少出血,其他人避免它,由于并发症。这项研究分享了单个中心在不进行术前栓塞的情况下管理颈动脉体瘤患者的经验。
    方法:这是一项针对2020年至2024年之间颈动脉体瘤患者的横断面研究。数据收集自医院登记处。必要时,血常规检查,颈部超声检查,并进行计算机断层扫描。根据Shamblin分类对肿瘤进行分类。平均随访时间为20个月。
    结果:该研究涉及25名患者,22(88%)女性和3(12%)男性。他们的年龄从27岁到85岁不等。20例(80%)颈部肿胀,6人(24%)有阳性病史。肿瘤主要在右侧(52%),20(80%)显示不明确的颈部肿块。肿瘤大小从1.5到7厘米,在大多数病例中发现了ShamblinII型肿瘤(72%)。肿瘤类型与肿瘤大小显著相关(p值<0.05)。5例(20%)需要输血,三个来自III型,两个来自II型,I型无(p值=0.001)。3例(12%)出现暂时性神经功能缺损。无功能障碍或死亡记录。
    结论:颈动脉体瘤是一种病因不明的罕见肿瘤。不进行术前栓塞的手术可能是可行的,结果可接受。
    BACKGROUND: Carotid body tumors are uncommon neuroendocrine growths near the carotid bifurcation. While some advocate preoperative embolization to minimize bleeding, others avoid it due to complications. This study shares the experience of a single center in managing patients with carotid body tumors without practicing preoperative embolization.
    METHODS: This was a cross-sectional study of patients with carotid body tumors managed between 2020 and 2024. Data were collected from the hospital\'s registry. When necessary, routine blood tests, neck ultrasonography, and computed tomography scans were conducted. The tumors were categorized according to Shamblin\'s classification. The average duration of follow-up was 20 months.
    RESULTS: The study involved 25 patients, 22 (88%) females and 3 (12%) males. Their ages ranged from 27 to 85 years old. Twenty (80%) cases presented with neck swelling, and six (24%) had a positive medical history. Tumors were mainly on the right side (52%), with 20 (80%) showing ill-defined neck masses. Tumor sizes ranged from 1.5 to 7 cm, with Shamblin type II tumors being discovered in the majority of cases (72%). Types of tumors were significantly associated with the tumor size (p-value < 0.05). Blood transfusion was required in five cases (20%), three from type III and two from type II, with none from type I (p-value = 0.001). Temporary neurological deficits occurred in 3 cases (12%). No functional impairment or mortality was recorded.
    CONCLUSIONS: Carotid body tumors are rare tumors with an unknown etiology. Operation without practicing preoperative embolization may be feasible with an acceptable outcome.
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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: Case Reports
    散发性双侧颈动脉体瘤是一种罕见的头颈部副神经节瘤,通常无症状,在其他病理检查中偶然发现。这些肿瘤来自位于颈动脉分叉处的化学感受器器官,可以是局部侵入性的,导致邻近的神经血管结构受累。大多数双侧颈动脉体瘤与家族综合征有关,例如MEN2或VonHippelLindau,散发性双侧疾病的发病率极为罕见。在这个案例报告中,我们报告了1例患有焦虑和心动过速的54岁女性患者的双侧颈动脉体瘤,该患者接受了MRI和CTA评估,显示左侧颈动脉体瘤大于右侧颈动脉体瘤的证据.以分阶段的方式对患者进行手术治疗,解决了她的症状,没有术后发病率或死亡率。双侧肿块的病理检查证实副神经节瘤的证据,免疫组织化学染色显示肿瘤细胞突触素阳性,嗜铬粒蛋白,和S100无淋巴结累及。
    Sporadic bilateral carotid body tumors are a rare paraganglioma of the head and neck that are often asymptomatic and incidentally found during workup for other pathologies. These tumors arise from the chemoreceptor organ located at the carotid bifurcation and can be locally invasive, resulting in the involvement of adjacent neurovascular structures. With the majority of bilateral carotid body tumors having an association with familial syndromes, such as MEN2 or Von Hippel Lindau, the incidence of sporadic bilateral disease is exceedingly rare. In this case report, we report a case of bilateral carotid body tumors in a 54-year-old female with anxiety and tachycardia who underwent MRI and CTA evaluation showing evidence of left greater than right carotid body tumors. The patient was managed operatively in a staged fashion with a resolution of her presenting symptoms and no post-operative morbidity or mortality. Pathologic examination of the bilateral masses confirmed evidence of paraganglioma with immunohistochemical stains showing neoplastic cells positive for synaptophysin, chromogranin, and S100 without lymph node involvement.
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