Insulinoma

胰岛素瘤
  • 文章类型: Case Reports
    胰岛素瘤是罕见的内分泌胰腺肿瘤。胰岛素瘤的微创治疗方案越来越突出,替代手术切除,由于其相关的发病率和死亡率。射频消融(RFA)已成为相关的治疗选择。我们介绍了一例女性患者的神经糖减少症状和严重的低血糖危机。腹部磁共振成像(MRI)显示胰腺体内有一个小的结节性病变。进行了剖腹手术,然后使用15毫米主动针头进行RFA。没有出现并发症,随访12个月期间未观察到低血糖发作.
    Insulinomas are rare neoplasms of the endocrine pancreas. Minimally invasive treatment options for insulinomas have gained prominence, replacing surgical resection due to its associated morbidity and mortality. Radiofrequency ablation (RFA) has emerged as a relevant treatment option. We present a case of a female patient with neuroglycopenic symptoms and severe hypoglycemic crises. The abdominal magnetic resonance imaging (MRI) showed a small nodular lesion in the pancreatic body. Laparotomy was performed, followed by RFA using a 15-mm active-tipped needle. No complications transpired, and no hypoglycemic episodes were observed during 12 months of follow-up.
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  • 文章类型: Case Reports
    胰岛素瘤占胰腺肿瘤的<10%。它是一种功能性神经内分泌肿瘤,可因低血糖引起反复发作和严重的意识丧失。手术切除是唯一的治疗方法。必须针对每位患者选择最佳手术技术。目前,在降低发病率的侵入性较小的技术中,有一些新兴的创新。我们介绍了一名23岁妇女的情况,该妇女在腹腔镜手术后接受了位于胰尾尖端的胰岛素瘤的摘除术,专注于血管和脾保存。在手术过程中安全地鉴定了肿瘤,并摘除了脾脏和邻近血管结构的损伤或术后并发症。
    Insulinomas represent <10% of pancreatic tumors. It is a functional neuroendocrine tumor that can cause recurrent and severe episodes of loss of consciousness due to hypoglycemia. Surgical removal is the only curative treatment. The selection of the optimal surgical technique must be individualized for each patient. Currently, there are emerging innovations in less invasive techniques that reduce morbidity. We present the case of a 23-year-old woman who underwent enucleation of an insulinoma localized at the tip of the pancreatic tail after laparoscopic surgery, with a focus on vascular and splenic preservation. The tumor was safely identified during surgery and enucleated without injury to the spleen and adjacent vascular structures or postoperative complications.
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  • 文章类型: Journal Article
    手术是胰岛素瘤的主要治疗方法,精确的术前定位对于确定切除范围和排除多发病变很重要。选择性动脉钙注射(SACI)测试有助于胰岛素瘤的定位。在这里,我们报告了一名患者,其中胰腺胰岛素瘤的确切位置无法通过常规SACI测试确定,因此手术改为口服二氮嗪。超选择性SACI测试随后准确定位病变,允许手术切除胰体和胰尾,同时保留胰头。当常规SACI测试无法准确确定胰腺内胰岛素瘤病变的位置时,我们建议使用高选择性SACI测试。
    Surgery is the main treatment for insulinoma, and precise preoperative localization is important to determine the extent of resection and to rule out multiple lesions. The selective arterial calcium injection (SACI) test is instrumental in the localization of insulinoma. Here we report a patient in whom the exact location of pancreatic insulinoma could not be determined by the conventional SACI test, and thus surgery was replaced with oral diazoxide. The hyperselective SACI test subsequently localized the lesion accurately, allowing surgical resection of the pancreatic body and tail while preserving the pancreatic head. We recommend the use of the hyperselective SACI test when the conventional SACI test fails to accurately determine the location of insulinoma lesions within the pancreas.
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  • 文章类型: Journal Article
    功能性神经内分泌肿瘤(NENs)是与NENs激素分泌相关的特定症状相关的肿瘤。虽然只有不到25%的NENs在诊断时具有功能性,1相关综合征显著增加患者的疾病负担。激素NEN症状的管理可能涉及肿瘤切除或其他减少策略(例如,化疗,栓塞治疗,etc),但也有针对减少激素合成的特定疗法,分泌,或最终器官效应。在这次审查中,我们专注于许多NEN综合征的具体症状管理,除了主要针对肿瘤体积和生长的管理之外,还可以继续进行。继续关注与NEN激素分泌相关的症状管理,在其他减少肿瘤体积和生长的努力中,可以显着改善患者的健康。
    Functional neuroendocrine neoplasms (NENs) are those associated with specific symptoms related to the hormonal secretion of the NENs. Although less than 25 % of NENs are functional at diagnosis,1 the associated syndromes significantly increase the patient burden of disease. Management of hormonal NEN symptoms may involve tumor resection or other reduction strategies (e.g., chemotherapy, embolotherapy, etc), but also specific therapies directed at decreasing hormonal synthesis, secretion, or end-organ effects. In this review, we focus on specific symptomatic management of many of the NEN syndromes, which may be pursued in addition to management primarily directed at tumor bulk and growth. A continued focus on symptom management related to the hormonal secretions of NENs, in the context of other efforts to reduce tumor bulk and growth, could significantly improve patient wellbeing.
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  • 文章类型: Case Reports
    在儿童中期,由于局灶性或弥漫性胰腺病变引起的低血糖反复发作而引起的高胰岛素血症很少见。对于诊断婴儿期以外的局灶性胰岛素产生病变的金标准成像方法尚无共识。一个有复杂病史和难治性癫痫的14岁男孩,血糖(BG)为52mg/dL(2.9mmol/L)(正常参考范围:70-100mg/dL[3.9-5.6mmol/L]),癫痫发作频率增加。他在4小时内禁食失败了,血糖为48mg/dL(2.7mmol/L),胰岛素水平为4.6µIU/mL(24.6pmol/L)(低血糖时诊断为>1.25µU/mL[8.7pmol/L])。常规影像学检查未显示胰腺病变。用卡比多巴预先给药的氟-18-L-二羟基苯丙氨酸正电子发射断层扫描/磁共振成像(18F-DOPA-PET/MRI)扫描显示,远端胰尾有强烈的局灶性18F-DOPA摄取。他接受了远端胰腺切除术。组织病理学显示局灶性胰岛细胞增生,超过90%的神经内分泌胰岛细胞对嗜铬粒蛋白和突触素呈阳性,p57染色无丢失。遗传研究对ABCC8,KCNJ11,GCK,或GLUD1基因,多发性内分泌瘤(MEN)1型和Beckwith-Wiedemann综合征。手术后BG恢复正常。癫痫发作频率提高。此病例突出了18F-DOPAPET/MRI成像在诊断婴儿期以外的局灶性高胰岛素血症中的实用性。
    Hyperinsulinism due to focal or diffuse pancreatic lesions causing recurrent episodes of hypoglycemia is rare in mid-childhood. There is no consensus on the gold-standard imaging method to diagnose focal insulin-producing lesions beyond infancy. A 14-year-old boy with a complex medical history and refractory epilepsy, presented with blood glucose (BG) of 52 mg/dL (2.9 mmol/L) (normal reference range: 70-100 mg/dL [3.9-5.6 mmol/L]) and increased seizure frequency. He failed a fast within 4 hours, with BG of 48 mg/dL (2.7 mmol/L) and insulin level of 4.6 µIU/mL (24.6 pmol/L) (diagnostic at the time of hypoglycemia >1.25 μU/mL [8.7 pmol/L]). Conventional imaging studies showed no pancreatic lesion. Fluorine-18-L-dihydroxyphenylalanine positron emission tomography/magnetic resonance imaging (18F-DOPA-PET/MRI) scan premedicated with carbidopa demonstrated intense focal 18F-DOPA uptake in the distal pancreatic tail. He underwent distal pancreatectomy. Histopathology showed focal pancreatic islet cell hyperplasia, with more than 90% of the neuroendocrine islet cells being positive for chromogranin and synaptophysin, with no loss of p57 staining. Genetic studies were negative for mutations in ABCC8, KCNJ11, GCK, or GLUD1 genes, multiple endocrine neoplasia (MEN) type 1, and Beckwith-Wiedemann syndrome. BG normalized after surgery. Seizure frequency improved. This case highlights the utility of 18F-DOPA PET/MRI imaging in diagnosing focal hyperinsulinism beyond infancy.
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  • 文章类型: Case Reports
    在文献中,与糖尿病患者有关的低血糖意识不足(HU)的描述更为频繁。我们注意到,胰岛素瘤病例中HU的报告也在增加。我们报告了一家医院的报告,偶然发现了一位五十多岁的绅士的低血糖意识不足,他最终在生化研究后被诊断出患有胰岛素瘤,胰腺和脾脏之间切除病变的放射学评估和组织学评估。我们已经回顾了有关此事件的可能病因和管理选择的现有文献证据。需要进行更多的研究以确定这种关联的流行病学,并确定增加对表现出HU的胰岛素瘤患者的检测方案。
    Hypoglycaemic unawareness (HU) is more frequently described in relation to diabetics in the literature. We have noted that there is also an increasing reporting of HU in insulinoma cases. We report a hospital presentation for an incidental finding of hypoglycaemic unawareness in a gentleman in his fifties who was eventually diagnosed with insulinoma following biochemical studies, radiologic evaluation and histologic evaluation of an excised lesion between the pancreas and the spleen. We have reviewed existing literature evidence regarding the possible aetiologies and management options for this occurrence. More research studies to identify the epidemiology of this association and the determination of a protocol for increased detection of patients with insulinoma who display HU will need to be done.
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  • 文章类型: Case Reports
    转移性胰岛素瘤的胃肠道出血(GIB)是一种罕见的现象。似乎引起GIB的转移性胰岛素瘤病例很少见,通常受肿瘤位置的影响。我们的病例涉及一名82岁男性,患有痴呆症,有反复低血糖史,表现出精神状态改变的情节。患者表现出低血糖以及黑便发作和贫血。诊断标准,包括惠普尔的三合会,确认内源性胰岛素生产。计算机断层扫描(CT)显示左侧主动脉旁/腹膜后肿块。食管胃十二指肠镜检查(EGD)显示了胃体的外部肿块,这导致了溃疡表面,用夹闭和止血治疗。患者的反复低血糖发作采用葡萄糖治疗,而他的GIB通过止血和剪裁进行管理。然而,病人不是手术候选人,家庭停止了进一步的医疗。
    A gastrointestinal bleed (GIB) in the setting of metastatic insulinoma is a rare phenomenon. It appears that cases of metastatic insulinoma causing GIB are rare, often influenced by the tumor\'s location. Our case involves an 82-year-old male with dementia and a history of recurrent hypoglycemia, presenting with an episode of altered mental status. The patient exhibited hypoglycemia alongside a melena episode and anemia. Diagnostic criteria, including Whipple\'s triad, confirmed endogenous insulin production. Computed tomography (CT) showed a left paraaortic/retroperitoneal mass. Esophagogastroduodenoscopy (EGD) visualized an extrinsic mass at the gastric body, which caused an ulcerated surface that was treated with clipping and hemostasis. The patient\'s recurrent hypoglycemic episodes were treated with glucose, while his GIB was managed with hemostasis and clipping. However, the patient was not a surgical candidate, and further medical treatment was ceased by the family.
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  • 文章类型: Journal Article
    胰腺肿块在儿科患者中极为罕见,可用数据有限。这种数据的缺乏使得儿童中这些肿瘤的诊断和管理极具挑战性。因此,我们的目的是描述演讲,临床课程,以及我们中心胰腺肿瘤患儿的结局。回顾性分析了2003年至2022年在一家学术独立儿童医院诊断为胰腺肿块的所有儿科患者。数据包括人口统计,临床表现,workup,管理,收集和汇总随后的发病率和死亡率。此外,我们在国家外科质量改善计划-儿科(NSQIP-P)数据库中回顾了胰腺肿瘤切除病例,以确定常见的不良结局和质量改善措施.总的来说,在我们的机构确定了17名患者。诊断包括实性假乳头(n=9),胃泌素瘤(n=1),横纹肌肉瘤(n=2),胰母细胞瘤(n=2),和胰岛素瘤(n=1)。两名患者没有组织病理学诊断,被排除在随后的分析之外。总的来说,12例患者接受了手术干预,最常见的手术是胰十二指肠切除术和远端胰腺切除术,最后一次接触时,所有12人都还活着。有3人死亡,都是由于与转移性疾病相关的并发症。此外,在NSQIP-P数据集中,儿科患者胰腺手术的术后30天结果非常好,发病率可忽略不计,索引手术后无死亡率。
    结论:接受手术切除的胰腺肿瘤患儿似乎具有足够的长期生存率。诊断时的短期结果是极好的,并且主要似乎受到初始表现时转移性疾病的影响。
    背景:•胰腺肿块是儿童中的罕见实体,其表现数据有限,管理和手术结果。•实性假乳头状瘤是手术干预后预后良好的儿童中最常见的胰腺肿瘤之一。
    背景:•对于没有侵袭性肿瘤类型或转移性疾病的患者,小儿胰腺肿瘤的外科治疗是安全有效的。•我们的病例系列提供了这些胰腺肿瘤的一个值得注意的队列,其中五种肿瘤类型的治疗和结果。
    Pancreatic masses are extremely rare in pediatric patients, with limited data available. This lack of data makes the diagnosis and management of these tumors in children extremely challenging. Therefore, we aimed to describe the presentations, clinical course, and outcomes of children with pancreatic tumors at our center. A retrospective analysis was performed of all pediatric patients diagnosed with pancreatic masses between 2003 and 2022 in an academic freestanding children\'s hospital. Data including demographics, clinical presentation, workup, management, and subsequent morbidity and mortality were collected and aggregated. Furthermore, we reviewed cases of pancreatic tumor resections in the National Surgical Quality Improvement Program - Pediatric (NSQIP-P) database to identify common adverse outcomes and measures for quality improvement. In total, 17 patients were identified at our institution. Diagnoses included solid pseudopapillary (n = 9), gastrinoma (n = 1), rhabdomyosarcoma (n = 2), pancreatoblastoma (n = 2), and insulinoma (n = 1). Two patients did not have a histopathologic diagnosis and were excluded from subsequent analysis. Overall, 12 patients underwent surgical intervention, with the most common procedures being pancreaticoduodenectomy and distal pancreatectomy, and all 12 were known to be alive at last contact. There were 3 deaths, all due to complications related to metastatic disease. Furthermore, 30-day postoperative outcomes in the NSQIP-P dataset for pancreatic surgeries in pediatric patients are excellent, with negligible morbidity and no mortalities after the index surgery.
    CONCLUSIONS: Children with pancreatic tumors amenable to surgical resection appear to have adequate long-term survival. Short-term outcomes at diagnosis are excellent and mainly appear to be influenced by the presence of metastatic disease at initial presentation.
    BACKGROUND: • Pancreatic masses are a rare entity in children with limited data on their presentation, management and surgical outcomes. • Solid Pseudopapillary tumors are one of the most common pancreatic tumors in children with a fair prognosis after surgical intervention.
    BACKGROUND: • Surgical management of pediatric patients with pancreatic tumors is safe and effective in patients who do not have aggressive tumor types or metastatic disease. • Our case series provides a notable cohort of these pancreatic tumors with insight into the presentation, management and outcomes of five of these tumor types.
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  • 文章类型: Journal Article
    We demonstrate robot-assisted treatment of a patient with benign pancreatic insulinoma. A 31-year-old patient suffered from attacks of weakness, numbness of the fingertips and «turbidity of consciousness» for 2 years. These symptoms occurred on an empty stomach and regressed after eating. We found pancreatic insulinoma. The patient underwent robotic enucleation of pancreatic tumor. Surgery time was 145 min. Postoperative period proceeded without complications. Hyperglycemia up to 10.5 mmol/l on the first postoperative day was followed by normalization after 4 days. The patient was discharged in 6 days after surgery. Minimally invasive robotic enucleation of insulinoma minimizes surgical trauma and provides precise resection of tumor. The key aspect of safe enucleation is localization of tumor at a distance of at least 2 mm from the pancreatic duct.
    Продемонстрирован опыт лечения пациента с инсулиномой поджелудочной железы методом робот-ассистированной энуклеации опухоли. Пациент, 31 год, в течение 2 лет отмечал приступы слабости, онемение кончиков пальцев на руках, «помутнения сознания», которые возникали натощак и купировались приемом пищи. При клинико-инструментальном обследовании обнаружена опухоль в теле-хвосте поджелудочной железы, выставлен диагноз инсулиномы. Пациенту выполнена робот-ассистированная энуклеация опухоли поджелудочной железы, длительность вмешательства составила 145 мин. Послеоперационный период протекал без осложнений. В первые послеоперационные сутки наблюдалась гипергликемия до 10,5 ммоль/л с последующей полной компенсацией гликемии до нормальных значений к 4 сут послеоперационного периода. Пациент выписан на 6-е сутки после операции. Применение робот-ассистированного мини-инвазивного доступа при выполнении энуклеации инсулином поджелудочной железы позволяет прецизионно удалить опухоль и минимизировать операционную травму. Ключевым моментом возможности безопасного выполнения энуклеации является расположение опухоли на расстоянии не менее 2 мм от протока поджелудочной железы.
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  • 文章类型: Journal Article
    目的:这项回顾性研究评估了68Ga-DOTATATEPET/CT在胰岛素瘤诊断和定位中的价值,无论是零星的,恶性或MEN-1相关胰岛素瘤。
    方法:该研究包括43例患者,具有临床(症状性低血糖)和/或实验室怀疑患有胰岛素瘤(72小时空腹试验,血清胰岛素≥18pmol/L),与可用的术前68Ga-DOTATATEPET/CT和CE-CT,并经术后组织病理学证实诊断为胰岛素瘤。术前影像学由两名放射科医师进行回顾性分析,他们对最终诊断和其他影像学检查的结果一无所知。标本的组织病理学被认为是参考标准,术前CE-CT和PET影像学检查结果的头对头比较。结果被归类为真阳性(TP),正负(TN),假阳性(FP),和每个模态的假阴性(FN)。基于这些结果,灵敏度,特异性,CE-CT的阳性预测值(PPV)和阴性预测值(NPV),计算了68Ga-DOTATATEPET/CT用于检测胰岛素瘤。
    结果:43名患者(N=43名患者,L=56个病灶),从这些中,良性散发性胰岛素瘤37例(N=37,L=42),只有3例患者患有恶性散发性胰岛素瘤(N=2,L=9),3例患者患有MEN-1综合征相关胰岛素瘤(N=3,L=5)。在使用68Ga-DOTATATEPET/CT的整个队列中,胰岛素瘤定位的敏感性(P=0.3058)和PPV(P=0.5533)没有显着统计学差异(87.5%,90.74%)与CE-CT(80.36%,93.75%)。
    结论:68Ga-DOTATATEPET/CT是一种非侵入性成像模式,可以识别大多数胰岛素瘤。尽管如此,当肿瘤被其他解剖成像研究定位时,它提供了有限的额外信息,因此,当影像学研究无法定位胰岛素瘤患者的肿瘤时,应用作辅助手段,特别是当微创手术的目的。
    OBJECTIVE: This retrospective study evaluates the value of 68Ga-DOTATATE PET/CT in the diagnosis and localization of insulinomas, whether sporadic, malignant or MEN-1 associated insulinoma.
    METHODS: The study included 43 patients, having clinical (symptomatic hypoglycemia) and/or laboratory suspicion of having insulinoma (72 h fasting test with serum insulin ≥18 pmol/L), with available pre-operative 68Ga-DOTATATE PET/CT and CE-CT, and diagnosed with insulinoma confirmed by post-operative histopathology. Preoperative imaging was retrospectively analyzed by two radiologists who were blinded to the final diagnosis and to the results of other imaging modalities. Histopathology of specimen was considered the reference standard, and head-to-head comparison of preoperative CE-CT and PET imaging findings. Findings were classified as true positive (TP), true negative (TN), false positive (FP), and false negative (FN) for each modality. Based on these results, sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of CE-CT, and 68Ga-DOTATATE PET/CT for the detection of insulinoma were calculated.
    RESULTS: 43 patients (N = 43 patients, L = 56 lesions), out of these, 37 patients had benign sporadic insulinoma (N = 37, L = 42), only 3 patients had malignant sporadic insulinoma (N = 2, L = 9), and 3 patients had MEN-1 syndrome associated insulinoma (N = 3, L = 5). There was no significant statistical difference in sensitivity (P = 0.3058) and PPV (P = 0.5533) for insulinoma localization in the overall cohort with 68Ga-DOTATATE PET/CT (87.5 %, 90.74 %) compared to CE-CT (80.36 %, 93.75 %).
    CONCLUSIONS: 68Ga-DOTATATE PET/CT is a non-invasive imaging modality that can identify most insulinomas. Still, it offers limited additional information when the tumor is localized by other anatomic imaging studies, so should be used as an adjunct when imaging studies fail to localize the tumor in insulinoma patients, especially when minimally invasive surgical is intended.
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