Increased risk of second malignancy in pancreatic intraductal papillary mucinous tumors: Review of the literature

AIM: To analyze the available evidence about the risk of extrapancreatic malignancies and pancreatic ductal adenocarcinoma associated to pancreatic intraductal papillary mucinous tumors(IPMNs).METHODS: A systematic search of literature was undertaken using MEDLINE, EMBASE, Cochrane and Web-of-Science libraries. No limitations for year of publication were considered; preference was given to English papers. All references in selected articles were further screened for additional publications. Both clinical series and Literature reviews were selected. For all eligible studies, a standard data extraction form was filled in and the following data were extracted: study design, number of patients, prevalence of pancreatic cancer and extrapancreatic malignancies in IPMN patients and control groups, if available.RESULTS: A total of 805 abstracts were selected and read; 25 articles were considered pertinent and 17 were chosen for the present systematic review. Eleven monocentric series, 1 multicentric series, 1 casecontrol study, 1 population-based study and 3 case report were included. A total of 2881 patients were globally analyzed as study group, and the incidence of pancreatic cancer and/or extrapancreatic malignancies ranged from 5% to 52%, with a mean of 28.71%.When a control group was analyzed(6 papers), the same incidence was as low as 9.4%.CONCLUSION: The available Literature is unanimous in claiming IPMNs to be strongly associated with pancreatic and extrapancreatic malignancies. The consequences in IPMNs management are herein discussed.

Role of frozen section assessment for intraductal papillary and mucinous tumor of the pancreas

Intraductal papillary mucinous neoplasms(IPMN) of the pancreas include a spectrum of dysplasia ranging from minimal mucinous hyperplasia to invasive carcinoma and are extensive tumors that often spread along the ductal tree.Several studies have demonstrated that preoperative imaging is not accurate enough to adapt the extent of pancreatectomy and have suggested routinely using frozen sectioning(FS) to evaluate the completeness of resection and also to check if ductal dilatation is active or passive,in order to avoid an excessive pancreatic resection.Separate main duct and branch duct analysis is needed due to the difference in the natural history of the disease.FS accuracy averages 95%.Eroded epithelium on the main duct,severe ductal inflammation mimicking dysplasia and reactive epithelial changes secondary to obstruction can lead to inappropriate FS results.FS results change the planned extent of resection in up to 30% of cases.The optimal cut-off leading to extend pancreatectomy is not consensual and our standard option is to extend pancreatec-tomy if FS reveals:(1) at least IPMN adenoma on the main duct;or(2) at least borderline IPMN on branch ducts;or(3) invasive carcinoma.However,the decision to extend resection must be taken after a multidisciplinary discussion since it does not exclusively depend on the FS result but also on age,general condition and expected prognosis after resection.The main limitation of using FS is the existence of discontinuous("skip") lesions which account for approximately 10% of IPMN in surgical series and can lead to reoperation in up to 8% of cases.

MSCT and MRCP features of intraductal papillary mucinous tumor of the pancreas

Objective:To study the multi-slice CT(MSCT) and magnetic resonance cholangio-pancreatography(MRCP) features of intraductal papillary mucinous tumor(IPMT) of the pancreas.Methods:The clinical information,laboratory val-ues,imaging features and pathological findings of 8 cases were reviewed.Results:There were 6 males and 2 females with average 71.3 years old in this series.These lesions involved pancreatic branch ducts or main duct.Among them,2 cases were involved in the pancreatic uncinate process,1 case in the pancreatic head,1 in the pancreatic body,2 in the pancreatic tail and 2 cases involved multiple pancreatic duct.The common complaint was chronic upper abdominal pain.The imaging features included cystic lesion and it's association with the dilated pancreatic main duct,and bulging of the duodenal papilla.Mucin plug,septa or mural nodule were found in these cystic lesions.5 cases were confirmed with endoscopic retrograde cholangio-pancreatography(ERCP).3 cases had undergone surgery.Pathological findings of the 3 cases were adenoma,adenocarcinoma and borderline tumor,respectively.Conclusion:The MSCT and MRCP features of IPMT are somewhat characteristic,and the correct diagnosis can be made with the combination of clinical features.Diameter of branch duct ≥ 3 cm with multiple or large mural nodules,or severe dilated main duct indicates the probability of malignant IPMT.

molecular pathology of intraductal papillary mucinous neoplasms of the pancreas

Since the first description of intraductal papillary mucinous neoplasms(IPMNs)of the pancreas in the eighties,their identification has dramatically increased in the last decades,hand to hand with the improvements in diagnostic imaging and sampling techniques for the study of pancreatic diseases.However,the heterogeneity of IPMNs and their malignant potential make difficult the management of these lesions.The objective of this review is to identify the molecular characteristics of IPMNs in order to recognize potential markers for the discrimination of more aggressive IPMNs requiring surgical resection from benign IPMNs that could be observed.We briefly summarize recent research findings on the genetics and epigenetics of intraductal papillary mucinous neoplasms,identifying some genes,molecular mechanisms and cellular signaling pathways correlated to the pathogenesis of IPMNs and their progression to malignancy.The knowledge of molecular biology of IPMNs has impressively developed over the last few years.A great amount of genes functioning as oncogenes or tumor suppressor genes have been identified,in pancreatic juice or in blood or in the samples from the pancreatic resections,but further researches are required to use these informations for clinical intent,in order to better define the natural history of these diseases and to improve their management.

Intraductal papillary mucinous neoplasm originating from a jejunal heterotopic pancreas:A case report

BACKGROUND Intraductal papillary mucinous neoplasm(IPMN)is a rare pancreatic tumor and has the potential to become malignant.Surgery is the most effective treatment at present,but there is no consensus on the site of resection.Heterotopic pancreas occurs in the gastrointestinal tract,especially the stomach and duodenum but is asymptomatic and rare.We report a case of ectopic pancreas with IPMN located in the jejunum.CASE SUMMARY A 56-year-old male patient suffered from severe pain,nausea and vomiting due to a traffic accident and sought emergency treatment at our hospital.Contrast-enhanced computed tomography of the whole abdomen suggested splenic congestion,which was considered to be splenic rupture.Emergency laparotomy was performed,and the ruptured spleen was removed during the operation.Unexpectedly,a cauliflower-like mass of about 2.5 cm×2.5 cm in size was incidentally found about 80 cm from the ligament of Treitz during the operation.A partial small bowel resection was performed,and postoperative pathology confirmed the small bowel mass as heterotopic pancreas with low-grade IPMN.CONCLUSION Ectopic pancreas occurs in the jejunum and is pathologically confirmed as IPMN after surgical resection.

Reoperation for heterochronic intraductal papillary mucinous neoplasm of the pancreas after bile duct neoplasm resection:A case report

BACKGROUND Intraductal papillary neoplasm of the bile duct(IPNB)and intraductal papillary mucinous neoplasm(IPMN)of the pancreas have similar pathological manifestations.However,they often develop separately and it is rare for both to occur together.Patients presenting with heterochronic IPMN after IPNB are prone to be misdiagnosed with tumor recurrence.CASE SUMMARY A 67-year-old male patient was admitted 8.5 years after IPNB carcinoma and 4 years after the discovery of a pancreatic tumor.A left hepatic bile duct tumor with distal bile duct dilatation was found 8.5 years ago by the computed tomography;therefore,a left hepatectomy was performed.The postoperative pathological diagnosis was malignant IPNB with negative cutting edge and pathological stage T1N0M0.Magnetic resonance imaging 4 years ago showed cystic lesions in the pancreatic head with pancreatic duct dilatation,and carcinoembryonic antigen continued to increase.Positron emission tomography showed a maximum standard uptake value of 11.8 in the soft tissue mass in the pancreatic head,and a malignant tumor was considered.Radical pancreatoduodenectomy was performed.Postoperative pathological diagnosis was pancreatic head IPMN with negative cutting edge,pancreaticobiliary type,stage T3N0M0.He was discharged 15 d after the operation.Follow-up for 6 mo showed no tumor recurrence,and quality of life was good.CONCLUSION IPNB and IPMN are precancerous lesions with similar pathological characteristics and require active surgery and long-term follow-up.

Validation of serum tumor biomarkers in predicting advanced cystic mucinous neoplasm of the pancreas

BACKGROUND Early detection of advanced cystic mucinous neoplasms[(A-cMNs),defined as high-grade dysplasia or malignancy]of the pancreas is of great significance.As a simple and feasible detection method,serum tumor markers(STMs)may be used to predict advanced intraductal papillary mucinous neoplasms(IPMNs)and mucinous cystic neoplasms(MCNs).However,there are few studies on the usefulness of STMs other than carbohydrate antigen(CA)19-9 for early detection of A-cMNs.AIM To study the ability of five STMs-CA19-9,carcinoembryonic antigen(CEA),CA125,CA724,and CA242 to predict A-cMNs and distinguish IPMNs and MCNs.METHODS We mainly measured the levels of each STM in patients pathologically diagnosed with cMNs.The mean levels of STMs and the number of A-cMN subjects with a higher STM level than the cutoff were compared respectively to identify the ability of STMs to predict A-cMNs and distinguish MCNs from IPMNs.A receiver operating characteristic curve with the area under curve(AUC)was also created to identify the performance of the five STMs.RESULTS A total of 187 patients with cMNs were identified and 72 of them showed AcMNs.We found that CA19-9 exhibited the highest sensitivity(SE)(54.2%)and accuracy(76.5%)and a moderate ability(AUC=0.766)to predict A-cMNs.In predicting high-grade dysplasia IPMNs,the SE of CA19-9 decreased to 38.5%.The ability of CEA,CA125,and CA724 to predict A-cMNs was low(AUC=0.651,0.583,and 0.618,respectively).The predictive ability of CA242 was not identified.The combination of STMs improved the SE to 62.5%.CA125 may be specific to the diagnosis of advanced MCNs.CONCLUSION CA19-9 has a moderate ability,and CEA,CA125,and CA724 have a low ability to predict A-cMNs.The combination of STM testing could improve SE in predicting A-cMNs.

Molecular alterations in pancreatic tumors

Genetic alterations in pancreatic tumors can usually be classified in:(1)Mutational activation of oncogenes;(2)Inactivation of tumor suppressor genes;and(3)Inactivation of genome maintenance genes controlling the repair of DNA damage.Endoscopic ultrasound-guided fine-needle aspiration has improved preoperative diagnosis,but the management of patients with a pancreatic lesion is still challenging.Molecular testing could help mainly in solving these“inconclusive”specimens.The introduction of multi-gene analysis approaches,such as next-generation sequencing,has provided a lot of useful information on the molecular characterization of pancreatic tumors.Different types of pancreatic tumors(e.g.,pancreatic ductal adenocarcinomas,intraductal papillary mucinous neoplasms,solid pseudopapillary tumors)are characterized by specific molecular alterations.The aim of this review is to summarize the main molecular alterations found in pancreatic tumors.

Tubulopapillary adenoma of the gallbladder accompanied by bile duct tumor thrombus

Intraductal papillary mucinous neoplasm of the bile duct(IPNB)is recognized as a precancerous lesion;however,both its pathogenesis and progression remain unclear.We present here a case of IPNB arising from the gallbladder accompanied by bile duct tumor thrombus in a 79-year-old female.The resected specimen revealed a tubulopapillary adenoma with no malignant cells.This case suggests that even in the absence of malignant cells,these tumors can behave as malignant tumors requiring aggressive treatment.Even if no malignant cells are present,intraepithelial neoplasms occurring in the ampullopancreatobiliary tract can behave as malignant tumors.

胰腺浆液性囊腺瘤误诊病例的MSCT影像特征与病理分析

目的探讨胰腺浆液性囊腺瘤(PSC)的影像特征及病理基础,分析误诊原因,总结防范措施。方法回顾性分析2017—2023年经手术病理证实为PSC且术前多层螺旋CT误诊为其他病变22例的临床资料及CT影像特征,并与病理HE染色对照分析。结果本组22例中因腹痛就诊7例,恶心呕吐就诊1例,其余14例均为体检发现,其中微囊型10例,大囊型8例,混合型3例,实质型1例;8例位于胰腺头颈部,14例位于体尾部;分叶状13例,中央瘢痕2例,钙化5例,上游胰管扩张5例;增强后明显强化3例,中度强化5例,轻度强化6例,无强化8例。13例PSC呈分叶状轮廓、边界清晰、呈蜂窝或多囊状结构、与胰管不相通,2例PSC可见内部纤维瘢痕,5例可见钙化等特征性影像表现。误诊为黏液性囊腺瘤和神经内分泌瘤各5例,实性假乳头状瘤4例,未定性3例,假性囊肿2例,潴留囊肿、胰腺导管内乳头状瘤、胰腺癌各1例。误诊时间7~180 d。均手术治疗并经术后病理学检查确诊。随访均无复发。结论不同类型PSC影像表现存在一定差异,但同时存在一些共同影像特征,对于非典型病例,CT诊断困难,误诊率高,需提高对于本病的认知,同时密切结合临床病史,可提高其诊断准确率。

胰腺导管内乳头状肿瘤病理诊断及鉴别诊断

目的探讨胰腺导管内乳头状肿瘤3种不同预后的组织学亚型的临床及病理特点,旨在提高病理医师诊断水平,更好的为临床服务。方法收集本院2019—2023年16例胰腺导管内乳头状肿瘤患者资料,并结合相关文献梳理诊断重点。结果3种组织学亚型为导管内乳头状黏液性肿瘤(IPMN)、导管内嗜酸细胞乳头状肿瘤(IOPN)和导管内管状乳头状肿瘤(ITPN)。三者临床特征相似,但预后差异较大,伴浸润时IPMN预后较差,ITPN其次,IOPN最好,组织学形态是其主要鉴别要点,免疫表型对其鉴别起辅助作用。结论病理组织学形态是区分胰腺导管内乳头状肿瘤组织学亚型的关键,免疫表型具有辅助作用。

良恶性胰腺导管内乳头状瘤的临床及影像特征

目的:探讨术前根据患者的临床表现特点以及影像学特征区分胰腺导管内乳头状黏液性肿瘤(IPMT)良、恶性的可能性。方法:回顾分析1996年7月至2007年3月间40例IPMT患者的临床及影像学资料。所有患者的病变均经术后病理明确诊断,其中良性病例17例,恶性病例23例。结果:从临床表现特点来看,恶性IPMT患者出现腹痛以及黄疸较良性患者更为常见(P<0.05),但两者在性别、发病率、发病年龄以及出现体质量减轻上无显著性差异;从影像学表现特征来看,恶性IPMT肿瘤大小及病灶内的附壁结节较良性IPMT为大(P<0.05),前者主胰管扩张常较后者明显(P<0.05),且更常出现附壁结节以及囊性病灶内不规则的隔膜(P<0.05),但两者在好发部位上无显著性差异。结论:恶性与良性IPMT患者在临床表现特点以及影像学表现特征的差异对术前诊断具有重要参考价值,从而为决定手术时机和(或)手术方式提供依据。

胰腺囊性肿瘤的诊治要点

胰腺囊性肿瘤主要分为3类:浆液性囊性肿瘤(serous cystic neoplasm,SCN)、黏液性囊性肿瘤(mucinous cysticneoplasm,MCN)和导管内乳头状黏液性囊腺瘤(intraductal papillary mucinous neoplasm,IPMN)。SCN多为良性,恶性罕见,多是微囊腺瘤,典型表现呈蜂巢样结构。MCN有明显的恶性倾向,诊断多为体积较大且有分隔的囊肿。IPMN的特点是囊肿与胰管相通,伴有胰管扩张。囊肿的影像学特征是囊性肿瘤鉴别的主要依据,还应注意与实性假乳头状瘤(solid pseudopapillary tumors,SPT)和假性囊肿相鉴别。除了已经获得的明确诊断、肿瘤较小且无明显症状的SCN或较小的分支型IPMN之外,胰腺囊性肿瘤都应积极手术治疗。肿瘤完整切除的患者多数可以获得长期存活以上肿瘤的。具体手术方式应根据肿瘤所在部位、病理类型、与主胰管的关系、医师的经验以及患者全身情况综合考虑。

不同病理类型胰腺导管内乳头状黏液性肿瘤的临床分析

目的:总结胰腺导管内乳头状黏液性肿瘤(IPMT)的诊治经验。方法:回顾性分析2003年5月至2005年12月经手术切除且病理证实的30例IPMT患者的临床资料,对IPMT的不同病理类型进行比较分析。将胰腺导管内乳头状黏液性囊腺瘤(IPMA)定为良性组,胰腺导管内乳头状黏液性囊腺癌(IPMC)和胰腺导管内乳头状黏液性交界性囊腺瘤(IPMB)归入恶性组。结果:恶性组男性易患率高于女性(76.5%vs30.8%,P<0.05);30例患者中IPMA 13例,IPMB 4例,IPMC 13例;主胰管型14例,分支胰管型15例,混合型1例主胰管型恶性率显著高于分支胰管型(P=0.030)。30例患者中21例有腹痛、腰背痛或消瘦等临床表现;影像学表现上,恶性组病灶囊内附壁结节的发生率高于良性组(P=0.025);主胰管型IPMT的主胰管直径(MPD)≥10 mm与<10 mm者、分支胰管型IPMT的肿瘤最大径>40mm与≤40mm者恶性率差异的比较有统计学意义(P<0.05)。结论:恶性IPMT好发于60岁左右老年男性;对IPMT患者可将病灶囊内是否存在结节以及主胰管直径、肿瘤大小等方面作为术前对IPMT进行良恶性鉴别的参考。

胆胰管内超声内镜对胰腺疾病的诊断价值

目的评估胆胰管内超声内镜检查(IDUS)在胰腺疾病中的诊断价值。方法回顾分析2005年2月至2010年2月间行IDUS的63例胰腺疾病患者的影像学资料,IDUS均在经内镜逆行胰胆管造影(ERCP)时用经导丝的UMG20-29R腔内超声探头扫查胰腺。比较IDUS与CT、MRI、超声内镜(EUS)及腹部B超等5种方法在诊断胰腺疾病中的区别,以进一步评估IDUS在胰腺疾病的临床诊断中的应用价值。结果 IDUS对慢性胰腺炎(23例)、胰腺癌(10例)、导管内乳头状黏液瘤(IPMT,25例)、胰腺囊腺瘤(4例)的诊断准确率和Kappa检测值分别为93.7%(59/63),0.854;100.0%(63/63),1.00;90.5%(57/63),0.898以及96.8%(61/63),0.848。IDUS诊断准确率和Kappa检测值均优于其他4种方法 ,并且对1例胰腺囊腺癌也做出正确的术前诊断。结论 IDUS对胰腺疾病的诊断有较高的应用价值。

胰腺囊性肿瘤的诊断与治疗

目的探讨胰腺囊性肿瘤的诊断和治疗。方法回顾性分析2008年1月-2012年7月间56例本院胰腺囊性肿瘤患者的临床资料,男性15例,女性41例,男女比例为1:2.73,其中浆液性囊腺瘤12例,黏液性囊腺瘤22例,黏液性囊腺瘤癌6例,导管内乳头状囊腺瘤3例和乳头状囊腺瘤癌1例,实性假乳头状瘤12例。结果所有患者均经手术治疗,胰十二指肠切除术13例,胰腺肿瘤节段切除术4例,1例因肿瘤侵及周围脏器及大血管行胃空肠及胆肠吻合术,1例行胰腺钩突部肿瘤切除、部分十二指肠空肠切除、十二指肠空肠吻合术,胰腺肿瘤局部切除术3例,胰体尾切除术18例,保留脾脏的腹腔镜胰体尾切除术8例,腹腔镜胰体尾加脾切除2例,腹腔镜胰腺肿瘤局部切除术6例。本组无围手术期死亡,腹腔感染2例,胃排空障碍3例,胰瘘4例,胆漏2例,均经保守治疗痊愈。结论 B超、CT和MRI是胰腺囊性肿瘤的主要诊断方法,具体手术方式应根据肿瘤所在部位、病理类型、与主胰管的关系以及患者全身情况综合考虑。

胰腺导管内乳头状黏液性肿瘤的临床病理学分析(附29例报告)

目的:回顾性分析胰腺导管内乳头状黏液性肿瘤(IPMT)的诊治经验及预后,以期提高对该病的治疗水平。方法:1993年5月至2006年8月,共29例IPMT病人在我院接受手术治疗,男17例,女12例。现回顾性分析其临床表现、病理学分类以及术后随访情况。结果:术后病理证实,本组共有11例是类腺瘤,2例交界性肿瘤,16例腺癌。其中,主胰管型11例,分支胰管型16例,混合型2例。2例病人由于病变累及全胰而施行全胰切除术,其余病人分别施行胰十二指肠切除术(16例)、胰体尾切除术(7例)及局部切除术(4例)。在腺癌病例中,有37.5%(6/16)发生淋巴结转移。结论:IPMT有独特的临床病理学特征。对主胰管直径≥10mm、肿瘤直径>40mm以及主胰管型病例,应高度怀疑恶性病变的可能性。如术前考虑IPMT有恶性可能,则应施行合并淋巴结清扫的根治性切除术。

30例胰腺囊性肿瘤的诊断和治疗

目的探讨胰腺囊性肿瘤的诊治方法。方法对1995年1月至2006年1月诊治的30例胰腺囊性肿瘤进行回顾性分析。结果CT对胰腺囊性肿瘤的确诊率达90%(27/29),但不能确定其组织类型。所有病例均行手术治疗并随访,手术切除率86.7%,胰腺囊腺瘤18例均完整切除,术后无复发。胰腺囊腺癌12例,8例完整切除后5年生存率66%,不能行切除者预后差。结论加强对本病的认识是减少误诊率的关键;早期诊断、早期手术治疗是目前唯一可治愈的方法。

胰腺囊性病变的影像表现与临床特点(下)

胰腺囊性病变(PCL)是胰腺上皮和间质组织发生囊腔病变的一大类疾病,以胰腺内囊性包块为主要特征,具有不同的病因、临床和组织病理学特点。本文的前两部分介绍了胰腺炎症相关囊性病变(包括胰腺假性囊肿与胰腺包裹性坏死)与胰腺真性囊肿(包括孤立性胰腺上皮囊肿、von Hippel-Lindau病、多囊肾和囊性纤维化)以及常见的胰腺浆液性囊腺瘤、胰腺黏液性囊腺瘤和胰腺实性假乳头状瘤的影像表现与临床特点。本篇为最后一部分,就常见的胰腺导管内乳头状黏液瘤与胰腺少见囊性肿瘤予以介绍和分析,以期为临床诊断与治疗提供重要依据。

磁共振成像技术对胰腺导管内乳头状黏液瘤的诊断价值

目的探讨磁共振成像技术对胰腺导管内乳头状黏液瘤(ZPMN)的诊断价值。方法研究选择的对象共24例,均为我院2005年1月～2013年1月间收治的IPMN患者,均采用磁共振成像技术诊断,回顾分析其临床资料。结果检查显示主胰管型共6例,其中弥漫型4例,位于胰头部的节段型2例,MRI检查以主胰管弥漫性扩张或节段性扩张为主要表现,呈长T1低信号,同时呈长T2高信号;主胰管在MRCP图像上显示扩张呈中-重度,管径>9 mm。弥漫型病例均有胰腺实质萎缩伴发。分支胰管型18例,共中钩突部和胰头14例,胰体部4例;多房型16例,单房型2例。MRI检查显示为多个或单个囊性病变簇状组成,为长T1低信号、长T2高信号,其间有短T2低信号分隔影存在,为线条状,MRCP检查显示主胰管同上述囊性肿瘤相通,其中主胰管伴轻、重度扩张8例。分支胰管型所占比例显著高于主胰管型,差异有统计学意义(P<0.05)。结论胰腺导管内乳头状黏液瘤诊断中,MRI具有较高有效性,并具无创特点,使诊断准确率明显提高,具有非常积极的临床意义。