Pancreatic Intraductal Neoplasms

胰腺导管内肿瘤
  • DOI:
    文章类型: Journal Article
    目前的WHO消化系统肿瘤分类(2019年)提出了胰腺导管内和囊性肿瘤的诊断概念,主要基于综合分子数据和对其恶性潜力的评估。具有导管表型的胰腺导管内肿瘤包括胰腺导管腺癌的微观前体病变-胰腺上皮内瘤变和胰腺癌的宏观前体病变,其中导管内乳头状黏液性肿瘤是胰腺最常见的囊性肿瘤。现在,导管内嗜酸细胞乳头状肿瘤和导管内肾小管乳头状肿瘤都被归类为与胰腺癌侵袭性较低的亚型和更好的预后相关的独立实体。微小胰腺上皮内瘤变的临床意义有限,与其他导管内肿瘤相比,通过影像学方法将其呈现为囊性和/或实体瘤,对进一步治疗和手术治疗的指征具有重要影响(切除与“观察并等待”策略)。非导管源性肿瘤,如腺泡细胞癌和神经内分泌肿瘤,可以罕见地显示导管内生长,其正确分类具有很大的临床意义。此外,囊性胰腺病变的鉴别诊断不仅包括囊性和假性转化肿瘤,但也有大量的反应,炎症性和发育不良性囊性病变。
    The current WHO classification of digestive system tumours (2019) has presented the concept of diagnostics of intraductal and cystic neoplasms of the pancreas mostly based on integrated molecular data and evaluations of their malignant potential. Intraductal pancreatic neoplasms with ductal phenotype include microscopic precursor lesions of pancreatic ductal adenocarcinoma - the pancreatic intraepithelial neoplasia and macroscopic precursor lesions of pancreatic cancer, where intraductal papillary mucinous neoplasm represents the most common neoplasm of the pancreas with cystic appearance. Both intraductal oncocytic papillary neoplasm and intraductal tubulopapillary neoplasm are now classified as separate entities associated with less aggressive subtypes of pancreatic carcinoma and better prognosis. Clinical significance of microscopic pancreatic intraepithelial neoplasias is limited, in contrast to other intraductal neoplasms, which are presented as cystic and/or solid tumours by imaging methods with important consequences for further treatment and indication of surgical therapy (resection versus \"watch and wait\" strategies). Neoplasms of nonductal origin, such as acinar cell carcinomas and neuroendocrine neoplasms, can uncommonly display an intraductal growth and their correct classification has a great clinical importance. Moreover, differential diagnostics of cystic pancreatic lesions include not only cystic and pseudocystically transformed neoplasms, but also a large spectrum of reactive, inflammatory and dysontogenetic cystic lesions.
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  • 文章类型: Case Reports
    背景技术微创胰腺切除术已成为治疗良性和恶性胰腺肿瘤的标准做法。已知诸如机器人和腹腔镜方法的技术通过提供诸如减少失血的益处来降低发病率。减轻疼痛,缩短住院时间,和更快的恢复时间。初次胰腺手术后复发性或从头胰腺肿瘤的重复微创胰腺切除术的适应症仍存在争议。病例报告一名50岁的妇女因胰头导管内乳头状粘液性肿瘤的诊断而入院。2010年,她在胰腺钩突过程中接受了腹腔镜单支切除术。在5年的随访中,检测到新的导管内乳头状黏液性肿瘤,在接下来的7年中显示出逐渐的生长和壁结节的存在。患者的CEA水平升高至7.0ng/mL。考虑到肿瘤的进展和壁结节的出现,我们推荐了一个机器人辅助的Whipple手术.手术开始于腹腔镜粘连松解术。在使用Kocher手法分离粘连并重新固定十二指肠后,达芬奇手术系统继续手术。术后期间平安无事,患者在术后第20天出院。病理检查示导管内乳头状黏液原位癌,切缘阴性。结论本案例验证了在先前接受过微创胰腺手术的患者中对新诊断的胰腺肿瘤进行机器人Whipple手术的安全性和可行性。
    BACKGROUND Minimally invasive pancreatectomy has become the standard practice for the management of benign and malignant pancreatic tumors. Techniques such as robotic and laparoscopic approaches are known to reduce morbidity by offering benefits such as less blood loss, reduced pain, shorter hospital stays, and quicker recovery times. The indication for repeated minimally invasive pancreatectomy for recurrent or de novo pancreatic neoplasm after primary pancreatic surgery remains debated. CASE REPORT A 50-year-old woman was admitted to our hospital with a diagnosis of an intraductal papillary mucinous neoplasm in the pancreatic head. In 2010, she underwent laparoscopic single-branch resection for a branch-type tumor in the pancreatic uncinate process. During a 5-year follow-up, a de novo intraductal papillary mucinous neoplasm was detected, showing gradual growth and the presence of a mural nodule over the next 7 years. The patient\'s CEA level was elevated to 7.0 ng/mL. Considering the tumor\'s progression and the appearance of a mural nodule, we recommended a robot-assisted Whipple procedure. The operation began with laparoscopic adhesiolysis. After detachment of the adhesions and remobilization of the duodenum using the Kocher maneuver, the operation continued with the Da Vinci surgical system. The postoperative period was uneventful, and the patient was discharged on postoperative day 20. Pathological examination revealed intraductal papillary mucinous carcinoma in situ with negative resection margins. CONCLUSIONS This case verifies the safety and feasibility of performing a robotic Whipple procedure for a newly diagnosed pancreatic neoplasm in patients who have previously undergone minimally invasive pancreatic surgery.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    这项研究的主要目的是利用从对比增强腹部计算机断层扫描(CT)扫描得出的影像组学特征来设计预测模型,以辨别导管内乳头状粘液性肿瘤(IPMNs)的良性和恶性。通过从指定的训练队列(n=84)中的对比增强CT图像中提取相关特征,精心制作了放射学特征,以从恶性IPMNs中描绘良性。随后使用来自独立测试队列(n=37)的数据进行验证。通过受试者工作特性(ROC)曲线分析,定量评价了模型的判别能力,结合精心挑选的临床特点进行比较分析。动脉期图像用于构建包含8个特征的模型,以区分良性和恶性病例。该模型的准确度为0.891[95%置信区间(95%CI),交叉验证集中为0.816-0.996],测试集中为0.553(95%CI0.360-0.745)。相反,采用静脉期中的9项特征,模型的交叉验证准确度为0.862(95CI0.777-0.946),测试集准确度为0.801(95%CI0.653-0.950).将识别的临床特征与成像特征相结合,得到的模型具有0.934(95%CI0.879-0.990)的交叉验证准确性和0.904(95%CI0.808-0.999)的测试集准确性。从而进一步提高其辨别能力。我们的发现清楚地表明,就IPMNs性质的预测准确性而言,静脉期影像组学的特征掩盖了动脉期影像组学的特征。此外,用影像组学数据对临床特征进行综合和细致的筛选,显着提高了我们模型的诊断效能,强调在IPMN管理中采用全面和综合的方法进行准确的风险分层至关重要。
    The primary aim of this investigation was to leverage radiomics features derived from contrast-enhanced abdominal computed tomography (CT) scans to devise a predictive model to discern the benign and malignant nature of intraductal papillary mucinous neoplasms (IPMNs). Radiomic signatures were meticulously crafted to delineate benign from malignant IPMNs by extracting pertinent features from contrast-enhanced CT images within a designated training cohort (n = 84). Subsequent validation was conducted with data from an independent test cohort (n = 37). The discriminative ability of the model was quantitatively evaluated through receiver operating characteristic (ROC) curve analysis, with the integration of carefully selected clinical features to improve the comparative analysis. Arterial-phase images were utilized to construct a model comprising 8 features for distinguishing between benign and malignant cases. The model achieved an accuracy of 0.891 [95% confidence interval (95% CI), 0.816-0.996] in the cross-validation set and 0.553 (95% CI 0.360-0.745) in the test set. Conversely, employing 9 features from the venous-phase resulted in a model with a cross-validation accuracy of 0.862 (95%CI 0.777-0.946) and a test set accuracy of 0.801 (95% CI 0.653-0.950).Integrating the identified clinical features with imaging features yielded a model with a cross-validation accuracy of 0.934 (95% CI 0.879-0.990) and a test set accuracy of 0.904 (95% CI 0.808-0.999), thereby further improving its discriminatory ability. Our findings distinctly illustrate that venous-phase radiomics features eclipse arterial-phase radiomic features in terms of predictive accuracy regarding the nature of IPMNs. Furthermore, the synthesis and meticulous screening of clinical features with radiomic data significantly increased the diagnostic efficacy of our model, underscoring the pivotal importance of a comprehensive and integrated approach for accurate risk stratification in IPMN management.
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  • DOI:
    文章类型: Case Reports
    一名87岁的妇女因左大腿疼痛被送往急诊科,并诊断为坐骨神经疼痛。胸部CT扫描显示支气管扩张和树芽,胃液抗酸染色试验阳性;进一步,痰的M.avium-PCR和培养结果均为阳性,从而诊断为肺部非结核分枝杆菌感染(NTM)。腹部CT显示主胰管扩张和胰尾多灶性囊性肿瘤,发现并发导管内乳头状粘液性肿瘤(IPMN)。
    An 87-year-old woman presented to the emergency department with left thigh pain, and sciatic nerve pain was diagnosed. A chest CT scan showed bronchiectasis and tree-in buds and an acid-fast stain test of gastric juice was positive; further, M. avium-PCR of sputum and culture results were positive leading to a diagnosis of pulmonary nontuberculous mycobacterial infection(NTM). Abdominal CT showed dilatation of the main pancreatic duct and a multifocal cystic tumor in the pancreatic tail, which was found to be complicated with an intraductal papillary mucinous tumor(IPMN).
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    背景:导管内乳头状黏液性肿瘤引起的腺癌切除后长期生存的预测因素尚不清楚。这项研究确定了由导管内乳头状黏液性肿瘤引起的腺癌的长期(>5年)无病生存和复发的预测因素,并得出了无病生存的预后模型。
    方法:在2010年至2017年期间,在欧洲和亚洲的18个学术胰腺中心,连续接受胰腺切除术的患者来自导管内乳头状粘液性肿瘤,至少5年随访。使用Cox比例风险模型确定与无病生存相关的因素。进行了内部验证,并对判别和校准指标进行了评估。
    结果:在研究中,288例患者(中位年龄,70岁;52%男性);140(48%)患者在中位随访98个月后复发(四分位距,78.4-123),57例患者(19.8%)出现局部复发,109例(37.8%)全身复发。切除后5年,总生存率和无病生存率分别为46.5%(134/288)和35.0%(101/288),分别。关于Cox比例风险模型分析,多内脏切除术(危险比,2.20;95%置信区间,1.06-4.60),胰尾位置(危险比,2.34;95%置信区间,1.22-4.50),肿瘤分化差(风险比,2.48;95%置信区间,1.10-5.30),淋巴管浸润(危险比,1.74;95%置信区间,1.06-2.88),和神经周浸润(危险比,1.83;95%置信区间,1.09-3.10)与长期无病生存率呈负相关。最终的预测模型纳入了8个预测因子,并证明了对无病生存的良好预测能力(C指数,0.74;校准,坡度1.00)。
    结论:由于导管内乳头状黏液性肿瘤引起的腺癌,1/3的患者在胰腺切除术后获得长期无病生存(>5年)。当前研究中开发的预测模型可用于估计长期无病生存的概率。
    BACKGROUND: Predictors of long-term survival after resection of adenocarcinoma arising from intraductal papillary mucinous neoplasms are unknown. This study determines predictors of long-term (>5 years) disease-free survival and recurrence in adenocarcinoma arising from intraductal papillary mucinous neoplasms and derives a prognostic model for disease-free survival.
    METHODS: Consecutive patients who underwent pancreatic resection for adenocarcinoma arising from intraductal papillary mucinous neoplasms in 18 academic pancreatic centers in Europe and Asia between 2010 to 2017 with at least 5-year follow-up were identified. Factors associated with disease-free survival were determined using Cox proportional hazards model. Internal validation was performed, and discrimination and calibration indices were assessed.
    RESULTS: In the study, 288 patients (median age, 70 years; 52% male) were identified; 140 (48%) patients developed recurrence after a median follow-up of 98 months (interquartile range, 78.4-123), 57 patients (19.8%) developed locoregional recurrence, and 109 patients (37.8%) systemic recurrence. At 5 years after resection, the overall and disease-free survival was 46.5% (134/288) and 35.0% (101/288), respectively. On Cox proportional hazards model analysis, multivisceral resection (hazard ratio, 2.20; 95% confidence interval, 1.06-4.60), pancreatic tail location (hazard ratio, 2.34; 95% confidence interval, 1.22-4.50), poor tumor differentiation (hazard ratio, 2.48; 95% confidence interval, 1.10-5.30), lymphovascular invasion (hazard ratio, 1.74; 95% confidence interval, 1.06-2.88), and perineural invasion (hazard ratio, 1.83; 95% confidence interval, 1.09-3.10) were negatively associated with long-term disease-free survival. The final predictive model incorporated 8 predictors and demonstrated good predictive ability for disease-free survival (C-index, 0.74; calibration, slope 1.00).
    CONCLUSIONS: A third of patients achieve long-term disease-free survival (>5 years) after pancreatic resection for adenocarcinoma arising from intraductal papillary mucinous neoplasms. The predictive model developed in the current study can be used to estimate the probability of long-term disease-free survival.
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  • 文章类型: Journal Article
    导管内肾小管乳头状肿瘤(ITPNs)是罕见的胰腺肿瘤,具有独特的组织学和分子特征。考虑到已知的良好预后以及ITPN中潜在可靶向融合的高频率和多样性,将ITPN与其他胰腺肿瘤区分开来至关重要。虽然ITPN的组织学特征有据可查,关于细胞学特征的报道很少,和ITPN的分子表征。作者报告了2016年至2021年间在他们的实验室诊断出的三例病例。临床数据,细胞形态学和组织学特征,描述了这些病例的免疫表型和分子特征,并与文献报道的病例进行了比较。根据显微镜下存在的导管内结节,所有3例均被诊断为ITPN,该结节由紧密堆积的小管状腺体组成,并由缺乏明显粘蛋白的立方体细胞衬砌。在分子谱分析中,在病例1中发现KRAS和TP53变体,在病例2中发现FGFR2-INA融合,并且在病例3中检测到STARD3NL-BRAF融合。免疫组织化学(IHC)显示病例1的肿瘤细胞MUC2阳性,MUC6阴性,但在病例2和3中,MUC2阴性,MUC6阳性。这些结果证明了组织学相似的胰腺肿瘤的免疫表型和分子变异性。缺乏更常见的胰腺肿瘤特征性改变,应提示在形态学相关病例中考虑融合研究。形态学的结合,IHC,考虑到ITPN潜在的临床管理意义,分子分析对于可靠鉴定ITPN非常重要.
    Intraductal tubulopapillary neoplasms (ITPNs) are rare pancreatic tumors with distinct histological and molecular features. Distinction of ITPN from other pancreatic neoplasms is crucial given the known favorable prognosis and the high frequency and diversity of potentially targetable fusions in ITPN. While the histological features of ITPN are well documented, there are few reports on the cytological features, and molecular characterization of ITPN. The authors reported three cases diagnosed in their laboratory between 2016 and 2021. Clinical data, cytomorphological and histological features, with immunophenotypic and molecular characterizations of these cases are described and compared with those reported in the literature. All 3 cases were diagnosed as ITPN based on the microscopic presence of intraductal nodules composed of tightly packed small tubular glands lined by cuboidal cells lacking apparent mucin. On molecular profiling KRAS and TP53 variants were found in Case 1, FGFR2-INA fusion in Case 2, and STARD3NL-BRAF fusion was detected in Case 3. Immunohistochemistry (IHC) revealed that the neoplastic cells in Case 1 were MUC2 positive and MUC6 negative, but in Cases 2 and 3, were negative for MUC2 and positive for MUC6. These results demonstrate the immunophenotypic and molecular variabilities of histologically similar pancreatic neoplasms. The absence of alterations characteristic of more common pancreatic neoplasms should prompt the consideration of fusion studies in morphologically relevant cases. The combination of morphological, IHC, and molecular analyses is important for reliable identification of ITPN given its potential clinical management implications.
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  • 文章类型: Journal Article
    关于导管内乳头状粘液性肿瘤(IPMNs)的治疗指南对高危病变的手术指征都略有不同。我们的目的是回顾性比较四个指南在推荐高危IPMN手术的准确性。并评估CA-19-9水平升高的准确性和被认为是高风险的IPMNs的影像学特征在预测恶性肿瘤或高级别异型增生(HGD)方面的准确性。
    将2013-2020年期间手术切除的高风险IPMNs的最终组织病理学诊断与术前手术适应症进行比较,正如四项指南所列举的:2015年美国胃肠病学协会(AGA),2017年国际共识2018欧洲研究小组,和2018年美国胃肠病学学院(ACG)。如果手术标本的组织病理学显示HGD/恶性肿瘤,则认为手术是“合理的”。或术后症状改善。
    26/65例(40.0%)患者术后合理手术。所有患有HGD/恶性肿瘤的IPMN均由2018年ACG和2018年欧洲指南联合(绝对和相对标准)检测。综合(“高风险污名”和“令人担忧的特征”)2017年国际指南错过了1/19(5.3%)患有HGD/恶性肿瘤的IPMNs。2015年AGA指南错过了大多数HGD/恶性肿瘤IPMNs(11/19,57.9%)。我们发现与HGD/恶性肿瘤最相关的特征是胰腺导管扩张,和升高的CA-19-9水平。
    遵循2015年AGA指南,HGD/恶性肿瘤的漏诊率最高,但在没有这些功能的IPMN上运行的速率最低;同时,2018年ACG和2018年欧洲综合指南(绝对和相对标准)导致IPMNs更多无HGD/恶性肿瘤的手术,但IPMNs中HGD/恶性肿瘤的漏诊率最低。
    UNASSIGNED: The guidelines regarding the management of intraductal papillary mucinous neoplasms (IPMNs) all have slightly different surgical indications for high-risk lesions. We aim to retrospectively compare the accuracy of four guidelines in recommending surgery for high-risk IPMNs, and assess the accuracy of elevated CA-19-9 levels and imaging characteristics of IPMNs considered high-risk in predicting malignancy or high-grade dysplasia (HGD).
    UNASSIGNED: The final histopathological diagnosis of surgically resected high-risk IPMNs during 2013-2020 were compared to preoperative surgical indications, as enumerated in four guidelines: the 2015 American Gastroenterological Association (AGA), 2017 International Consensus, 2018 European Study Group, and 2018 American College of Gastroenterology (ACG). Surgery was considered \"justified\" if histopathology of the surgical specimen showed HGD/malignancy, or there was postoperative symptomatic improvement.
    UNASSIGNED: Surgery was postoperatively justified in 26/65 (40.0%) cases. All IPMNs with HGD/malignancy were detected by the 2018 ACG and the combined (absolute and relative criteria) 2018 European guidelines. The combined (\"high-risk stigmata\" and \"worrisome features\") 2017 International guideline missed 1/19 (5.3%) IPMNs with HGD/malignancy. The 2015 AGA guideline missed the most cases (11/19, 57.9%) of IPMNs with HGD/malignancy. We found the features most-associated with HGD/malignancy were pancreatic ductal dilation, and elevated CA-19-9 levels.
    UNASSIGNED: Following the 2015 AGA guideline results in the highest rate of missed HGD/malignancy, but the lowest rate of operating on IPMNs without these features; meanwhile, the 2018 ACG and the combined (absolute and relative criteria) 2018 European guidelines result in more operations for IPMNs without HGD/malignancy, but the lowest rates of missed HGD/malignancy in IPMNs.
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  • 文章类型: Journal Article
    目的:内窥镜超声(EUS)是一种出色的诊断工具,可提供胰腺囊性病变的高分辨率图像。然而,其在恶性导管内乳头状黏液性肿瘤(IPMN)的诊断中的作用仍然有限且不清楚。我们旨在确定这种诊断方式的有用性。
    方法:总的来说,从2018年4月至2021年6月,在计算机断层扫描(CT)/磁共振成像(MRI)后接受了IPMNEUS的246例患者进行了随访,直至2022年3月。我们评估了CT或MRI后进行EUS诊断恶性IPMN的附加值,用接收器工作特性曲线分析。在本研究中,如果厚度为2mm的墙壁高度不均匀,则认为它们是增厚的。
    结果:EUS明显提高了识别增强结节和增厚壁的准确性。CT/MRI检查时,受试者工作特征曲线下面积和相应的95%置信区间分别为0.655(0.549-0.760)和0.566(0.478-0.654),但使用EUS观察时分别为0.853(0.763-0.942)和0.725(0.634-0.817)。结节大小的组合,加厚的墙壁,并且主管道尺寸产生了接收器工作特性曲线下的最高面积(0.944[0.915-0.973])。
    结论:EUS更准确地检测恶性IPMN,由于CT/MRI无法识别不均匀的壁增厚和某些结节。
    OBJECTIVE: Endoscopic ultrasound (EUS) is an excellent diagnostic tool that provides high-resolution images of pancreatic cystic lesions. However, its role in the diagnosis of malignant intraductal papillary mucinous neoplasm (IPMN) remains limited and unclear. We aimed to determine the usefulness of this modality for such diagnosis.
    METHODS: Overall, 246 patients who underwent EUS for IPMN after computed tomography (CT)/magnetic resonance imaging (MRI) from April 2018 to June 2021 were followed up until March 2022. We assessed the added value of performing EUS after CT or MRI for diagnosing malignant IPMN, using receiver operating characteristic curve analysis. Walls as thick as 2 mm were considered thickened in this study if they were highly uneven.
    RESULTS: EUS clearly enhanced accuracy in identifying enhancing nodules and thickened walls. The areas under the receiver operating characteristic curve and corresponding 95% confidence intervals were 0.655 (0.549-0.760) and 0.566 (0.478-0.654) upon CT/MRI but 0.853 (0.763-0.942) and 0.725 (0.634-0.817) when observed using EUS. The combination of nodule size, thickened wall, and main duct size yielded the highest area under the receiver operating characteristic curve (0.944 [0.915-0.973]).
    CONCLUSIONS: EUS more accurately detects malignant IPMN, as uneven wall thickening and certain nodules cannot be identified with CT/MRI.
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