Laparoscopic resection of synchronous intraductal papillary mucinous neoplasms: A case report

We describe herein a 68-year-old woman who was diagnosed with a quite rare entity of intraductal papillary mucinous neoplasms (IPMNs) occurring simultaneously in the left lateral lobe of liver and the tail of pancreas. Abdominal computed tomography and magnetic resonance cholangiopancreatography showed a cystic dilatation of the pancreatic duct in the pancreatic tail, which suggested an IPMN, and multiple intrahepatic duct stones in the left lateral lobe. The patient underwent a laparoscopic left lateral hepatolobectomy and spleen-preserving distal pancreatectomy. Intra-operative finding of massive mucin in the dilated bile duct implied an intraductal mucinous tumor in the liver. The diagnosis of synchronous IPMNs in the liver and pancreas was confirmed by pathological examination. The patient was followed up for 6 mo without signs of recurrence. Although several cases of IPMN of liver without any pancreatic association have been reported, the simultaneous occurrence of IPMNs in the liver and pancreas is very rare. To the best of our knowledge, it is the first reported case treated by laparoscopic resection.     

Management of intraductal papillary mucinous neoplasm of the pancreas

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a distinct entity characterized by papillary proliferations of mucin-producing epithelial cells with excessive mucus production and cystic dilatation of the pancreatic ducts. IPMNs have malignant potential and exhibit a broad histologic spectrum, ranging from adenoma to invasive carcinoma. IPMNs are classified into main duct and branch duct types, based on the site of tumor involvement. IPMN patients have a favorable prognosis if appropriately treated. The postoperative 5-year survival rate is nearly 100% for benign tumors and noninvasive carcinoma, and approximately 60% for invasive carcinoma. A main duct type IPMN should be resected. Surgical treatment is indicated for a branch duct IPMN with suspected malignancy (tumor diameter ≥ 30 mm, mural nodules, dilated main pancreatic duct, or positive cytology) or positive symptoms. Malignant IPMNs necessitate lymph node dissection (D1). IPMNs are associated with a high incidence of extrapancreatic malignancies and pancreatic ductal carcinoma.     

胰腺导管内乳头状黏液瘤

胰腺导管内乳头状黏液瘤（IPMN）是由胰腺导管内产生黏液的上皮细胞呈乳头状增殖形成的肿瘤。与经典的胰腺癌相比,IPMN具有低度恶性、生长缓慢、少有侵犯周围组织、淋巴结转移率和再发率低的特点。IPMN根据肿瘤累及的部位可分为主胰管型、分支胰管型和混合型,病理组织特征涵盖从单纯腺瘤到浸润癌等多个亚型,临床表现多样,多种影像学检查手段可显示弥漫性或节段性扩张的主胰管和囊状扩张的分支胰管,ERCP经扩大的乳头获取黏液和胰液,取胰腺导管内皮组织和壁结节供活检均有助于诊断。IPMN确诊后应积极手术,手术切除率高,术后5年生存率高于一般的胰腺癌。本文就其临床表现、分类、病理特征、影像学诊断和治疗等方面做一综述。     

Prevalence,Diagnosis and Management of Pancreatic Cystic Neoplasms: Current Status and Future Directions

Cystic neoplasms of the pancreas are found with increasing prevalence, especially in elderly asymptomatic individuals. Although the overall risk of malignancy is very low, the presence of these pancreatic cysts is associated with a large degree of anxiety and further medical investigation due to concerns about malignancy. This review discusses the different cystic neoplasms of the pancreas and reports diagnostic strategies based on clinical features and imaging data. Surgical and nonsurgical management of the most common cystic neoplasms, based on the recently revised Sendai guidelines, is also discussed, with special reference to intraductal papillary mucinous neoplasm (IPMN; particularly the branch duct variant), which is the lesion most frequently identified incidentally. IPMN pathology, its risk for development into pancreatic ductal adenocarcinoma, the pros and cons of current guidelines for management, and the potential role of endoscopic ultrasound in determining cancer risk are discussed. Finally, surgical treatment, strategies for surveillance of pancreatic cysts, and possible future directions are discussed.     

Intraductal papillary mucinous carcinoma with atypical manifestations： Report of two cases

Intraductal papillary mucinous neoplasms(IPMNs)are a well-characterized group of mucin-producing cystic neoplasms of the clear malignant potential type.We report here two cases of intraductal papillary mucinous carcinoma(IPMC)with atypical manifestations.In one case,we discussed a pseudomyxoma peritonei caused by a ruptured IPMC.In the other case we discussed the fistulization of IPMC into the stomach and duodenum.These two cases suggest that IPMN can either spontaneously rupture causing mucinous materials to spill into the free abdominal cavity or directly invade adjacent organs resulting in fistula development.     

胰腺癌前病变诊断和治疗

目的 通过对7例胰腺癌前病变患者的临床诊断和治疗分析,探讨此类疾病诊断方法的应用及治疗策略.方法 收集2006年1月-2007年12月92例手术治疗胰腺肿瘤中有癌前病变的7例患者,其中黏液囊腺瘤(MCN)1例、导管内乳头状黏液瘤(IPMN)2例、胰腺内分泌肿瘤1例、胰腺上皮内瘤变(PanIN)Ⅰ级、Ⅱ级及Ⅲ级各1例.采用免疫荧光分析法测定患者血清CA19-9.7例上腹部均行超声和螺旋CT检查;1例行超声内镜检查及穿刺;2例行逆行胰胆管造影检查.结果 胰腺癌前病变临床表现不典型,影像学检查常无实质性肿块,但PanIN可伴有胰管扩张、狭窄;IPMN在胰头处可表现为囊性扩张的胰管;囊腺瘤等在胰体尾处可表现为单个孤立的囊肿.肿瘤指标CA19-9在此类疾病中可轻度增高.但对诊断作用有限.手术切除可治愈,并可防止肿瘤的进一步癌变.结论 对疑为胰腺癌前病变患者需积极选用多种影像学方法进行诊断,并予以积极的手术探查及切除.     

Clinical aspects of intraductal papillary mucinous neoplasm of the pancreas

Intraductal papillary mucinous neoplasm (IPMN) is a spectrum of neoplasia in the pancreatic duct epithelium characterized by cystic dilation of the main and/or branch pancreatic duct. According to the site of involvement IPMNs are classified into three categories, i.e., main duct type, branch duct type, and combined type. Most branch duct IPMNs are benign, whereas the other two types are often malignant. A large size of branch duct IPMN and marked dilation of the main pancreatic duct indicate the presence of adenoma at least. The additional existence of large mural nodules increases the possibility of malignancy in all types. Of recent interest is the relatively high prevalence of synchronous and/or metachronous malignancy in various organs, including the pancreas. The prognosis is favorable after complete resection of benign and noninvasive malignant IPMNs. Malignant IPMNs acquiring aggressiveness after parenchymal invasion necessitate adequate lymph node dissection. On the other hand, asymptomatic branch duct IPMNs without mural nodules can be observed without resection for a considerably long time. This review addresses available data, current understanding, controversy, and future directions.     

Pancreatic incidentalomas: Investigation and management

The incidence of pancreatic incidentalomas (PIs) detected in otherwise asymptomatic patients is growing with the increasing quality and use of advanced imaging techniques. PI can present as isolated main pancreatic duct dilation or as a solid or cystic lesion. Although historically thought to be relatively rare, PIs are rather common, particularly cystic lesions of the pancreas, which can be detected in up to 49% of the general population. With the poor prognosis of pancreatic cancer, PIs are an opportunity for prevention and early diagnosis, but when managed poorly, they can also lead to overtreatment and unnecessary morbidity. The management of PI should begin with a dedicated pancreas protocol computed tomography (CT) scan or magnetic resonance imaging (MRI) to accurately characterize duct size, lesion characteristics and establish an accurate baseline for subsequent follow up. Diagnosis and subsequent management depends on the extent of main duct dilation and solid versus cystic appearance. Solid lesions are highly concerning for malignancy. Cystic lesions can be further categorized as intraductal papillary mucinous neoplasms of the pancreas (IPMNs) or mucinous cystic neoplasms (MCNs), both of which harbour malignant potential, or as serous cystic neoplasms (SCNs) that are benign. In this paper, we summarize the major challenges related to PI and present pragmatic suggestions for management.     

Imaging features of intraductal papillary mucinous neoplasms of the pancreas in multi-detector row computed tomography

AIM： To retrospectively evaluate the imaging features of pancreatic intraductal papillary mucinous neoplasms （IPMNs） in multi-detector row computed tomography （MDCT）.
METHODS： A total of 20 patients with pathologicallyconfirmed intraductal papillary mucinous neoplasms （IPMNs） were included in this study. Axial MDCT images combined with CT angiography （CTA） and multiplanar volume reformations （MPVR） or curved reformations （CR） were preoperatively acquired. Two radiologists （Tan L and Wang DB） reviewed all the images in consensus using an interactive picture archiving and communication system. The disputes in readings were resolved through consultation with a third experienced radiologist （Chen KM）. Finally, the findings and diagnoses were compared with the pathologic results.
RESULTS： The pathological study revealed 12 malignant IPMNs and eight benign IPMNs. The diameters of the cystic lesions and main pancreatic ducts （MPDs） were significantly larger in malignant IPMNs compared with those of the benign IPMNs （P 〈 0.05）. The combinedtype IPMNs had a higher rate of malignancy than the other two types of IPMNs （P 〈 0.05）. Tumors with mural nodules and thick septa had a significantly higher incidence of malignancy than tumors without these features （P 〈 0.05）. Communication of side-branch IPMNs with the MPD was present in nine cases at pathologic examination. Seven of them were identified from CTA and MPVR or CR images. From comparison with the pathological diagnosis, the sensitivity, specificity, and accuracy of MDCT in characterizing the malignancy of IPMN of the pancreas were determined to be 100%, 87.5% and 95%, respectively.
CONCLUSION： MDCT with CTA and MPVR or CR techniques can elucidate the imaging features of IPMNs and help predict the malignancy of these tumors.     

Resektion zystischer Pankreastumoren

Cystic tumors of the pancreas are diagnosed increasingly more due to increasing life expectancy and the moderate use of modern radiological diagnostics. Intraductal papillary mucinous neoplasms of the pancreas (IPMN), mucinous cystic neoplasms (MCN), solid pseudopapillary neoplasms (SPN) and serous cystic neoplasms (SCN) represent over 90?% of all cystic neoplasms of the pancreas. Although serous cystic lesions have a low or even no potential for malignant transformation, they are mostly resected when symptomatic. In contrast, mucinous lesions have an increased malignant potential and should therefore be resected in almost all cases. While this is true for all cases of MCNs and SPNs this is controversial for all IPMNs as they show a wide spectrum of morphological variants. The IPMNs may arise in the main pancreatic duct, major side branches or in both (mixed type). Although all IPMNs are considered to be precursor lesions to pancreatic adenocarcinomas it is not clear what the time course of such potential neoplastic transformation might be and whether all lesions progress to malignant tumors. As no currently used diagnostic test can reliably differentiate between benign and malignant tumors, the majority of newly diagnosed IPMNs should undergo surgical resection. According to current treatment guidelines (Sendai criteria), asymptomatic side branch IPMNs of less than 3 cm in diameter without suspicious radiological features, such as nodules, thickness of the cystic wall or size progression can be treated conservatively without the need for surgical resection. Recently, this approach has become controversial due to a relevant number of IPMNs reported as Sendai negative that showed malignant transformation on final histological examination.     

Small invasive ductal carcinoma of the pancreas distinct from branch duct intraductal papillary mucinous neoplasm

Endoscopic ultrasonography (EUS) is a highly sensitive diagnostic method for the detection of small pancreatic carcinomas.Recently, there have been some reports describing the utility of contrast-enhanced harmonic EUS (CEH-EUS) which uses sonographic contrast agent for differentiation of a pancreatic mass.This report describes a case of small adenocarcinoma of the pancreas distinct from branch duct intraductal papillary mucinous neoplasm (IPMN) in which investigation by EUS took place every 6 mo and diagnosis was made accurately by additional CEH-EUS during the followup of the branch duct IPMN.A 68-year-old female was admitted to our hospital because of a branch duct IPMN in the pancreatic body.She had been followedup by EUS every 6 mo.However, after 2 years EUS demonstrated a low echoic area distinct from the branch duct IPMN which was vaguely discernible by EUS, and accurate sizing and differential diagnosis were considered difficult on the EUS imaging.CHEUS with Sonazoid revealed a hypovascular tumor and we suspected small pancreatic carcinoma.The histopathological diagnosis was adenocarcinoma (10 mm) in the pancreatic tail, distinct from the branch duct IPMN of the pancreatic body.EUS and CEH-EUS may play an important role in the correct diagnosis of small pancreatic tumors, including synchronous and metachronous occurrence of IPMN and ductal adenocarcinoma of the pancreas.     

A case of intraductal papillary mucinous neoplasm of the pancreas arising from pancreas divisum without ventral pancreatic duct of Wirsung]

Most reported cases of intraductal papillary mucinous neoplasms (IPMNs) originate from Wirsung's duct or their branches. IPMNs arising from Santorini's duct and its branches have rarely been reported. Eight cases of IPMN arising from Santorini's duct have been published worldwide. However, these cases are associated with incomplete type of pancreas divisum. Recently, one report of IPMN with complete absence of Wirsung's duct has been reported. This patient was a 57-year-old woman who was admitted to the hospital due to progressive jaundice. On endoscopic retrograde cholangiopancreatography, there was a severely bulging ampulla of Vater and patulous minor papilla draining mucinous material and a cystic lesion communicating with the dilated Santorini's duct without any communication with Wirsung's duct. A pancreaticoduodenectomy was performed and the pathologic examination of resected specimen showed no evidence of Wirsung's duct, but an IPMN arising from Santorini's duct with peripancreatic lymph node metastasis. Herein, we report a case of invasive IPMN arising from pancreatic head without ventral pancreatic duct with a review of the relevant literatures.     

胰腺导管内乳头状黏液瘤的研究进展

胰腺导管内乳头状黏液瘤(intraductal papillary mucinous neoplasm,IPMNs)为来源于胰腺导管上皮的分化程度多样的胰腺肿瘤,位于主胰管或其分支内,可分泌黏液,为胰腺癌的癌前病变.区分IPMNs的良恶性对制定治疗方案,预估患者预后意义重大.随影像学和内镜的发展,IPMNs发现率逐年提...     

Synchronous pancreatic solid pseudopapillary neoplasm and intraductal papillary mucinous neoplasm

Solid pseudopapillary neoplasm (SPN) is a rare and low-grade malignant pancreatic neoplasm composed of poorly cohesive monomorphic neoplastic cells forming solid and pseudopapillary structures with frequent hemorrhagic-cystic degeneration. Intraductal papillary mucinous neoplasm (IPMN) is a pancreatic exocrine tumor composed of intraductal papillary growth of mucin containing neoplastic cells in the main pancreatic duct or its major branches. In the case presented here, a 53-year-old, Japanese man was found to have multiple cystic lesions and dilatation of the main pancreatic duct in the neck of the pancreas. Histological examination revealed a main-duct and branch-duct type IPMN, of the gastric-type, involving the neck of the pancreas, associated with a 0.5 cm SPN in the caudal side of the IPMN. We diagnosed this case as synchronous SPN and IPMN. As far as we know, only one other case of synchronous SPN and IPMN has been reported. Both the present case and the previously reported case showed abnormal nuclear expression of β-catenin in SPN, whereas IPMN showed no abnormal nuclear expression. These results suggest that β-catenin abnormality is not a common pathogenetic factor of synchronous SPN and IPMN.     

Endoscopic naso-pancreatic stent-guided single-branch resection of the pancreas for multiple intraductal papillary mucinous adenomas

INTRODUCTION Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is characterized by dilatation of the mainand/or branch pancreatic ducts, mucin-secretion, and intraductal papillary growth. IPMNs are classified into three types, a main pancreat…     

内镜诊治胰腺导管内乳头状粘液性肿瘤的应用

目的探讨胰腺导管内乳头状粘液性肿瘤(IPMN)的诊断、内镜下治疗,以提高对该疾病的认识及内镜对其诊断及治疗的意义。方法回顾性分析经内镜诊治的IPMN患者12例,总结并分析他们的一般情况、临床症状、影像学检查、实验室检查、内镜下治疗等方面资料。结果 12例IPMN患者以老年男性为主,无特异性临床表现,主要发生部位为胰头或钩突部,CT主要表现为囊实性低密度影,其内可有壁结节,胰管扩张伴或不伴胆管扩张,2例侵及胰腺实质。磁共振胰胆管造影(MRCP)主要表现为胰管扩张,胰腺萎缩,胰头部圆形高信号影。内镜下逆行胰胆管造影(ERCP)显示十二指肠乳头膨大,开口扩张,可见胶冻样粘液流出,胰管造影显示胰管全程扩张或胰头部囊状扩张,3例伴胆总管、肝内胆管扩张。9例行ERCP治疗,清除粘液栓并置入胰管支架,3例同时置入胆管支架。结论 IPMN是一种特殊类型的胰腺囊性疾病,影像学检查有其独特的表现,ERCP对其诊断及治疗有重要意义。     

Simultaneous liver mucinous cystic and intraductal papillary mucinous neoplasms of the bile duct:A case report

Cystic hepatic neoplasms are rare tumors,and are classified into two separate entities:mucinous cystic neoplasms(MCNs)and intraductal papillary mucinous neoplasms of the bile duct(IPMN-B).We report the case of a 56-year-old woman who presented with abdominal pain and jaundice due to the presence of a large hepatic multilocular cystic tumor associated with an intraductal tumor.Partial hepatectomy with resection of extrahepatic bile ducts demonstrated an intrahepatic MCN and an intraductal IPMN-B.This is the first report of the simultaneous occurrence of these two histologically distinct entities in the liver.     

Coexisting Type 1 Autoimmune Pancreatitis and Mixed-type Intraductal Papillary Mucinous Neoplasm

An 84-year-old man was referred to our hospital for a cystic lesion of the pancreatic head, swelling of the pancreatic tail and hilar biliary stricture, resulting in elevated liver enzyme levels. We suspected branch duct-type intraductal papillary mucinous neoplasm (IPMN) and type I autoimmune pancreatitis (AIP) associated with sclerosing cholangitis because of the high serum IgG4 levels. The main pancreatic duct on the tail side of the AIP lesion was moderately dilated. Although the biliary stricture and pancreatic swelling improved after prednisolone treatment, the pancreatic enzyme levels increased rapidly. The entire main pancreatic duct exhibited remarkable dilatation, which led to the diagnosis of mixed-type IPMN. The clinical characteristics of IPMN in the main pancreatic duct appear to have been initially masked by AIP.     

Experience of limited pancreatic head resection for management of branch duct intraductal papillary mucinous neoplasm in a single center

AIM： To share our surgical experience and the outcome of limited pancreatic head resection for the management of branch duct intraductal papillary mucinous neoplasm （IPMN）.
METHODS： Between May 2005 and February 2008, nine limited pancreatic head resections （LPHR） were performed for IPMN of the pancreatic head. We reviewed the nine patients, retrospectively.
RESULTS： Tumor was located in the uncinate process of the pancreas in all nine patients. Three patients had stents inserted in the main pancreatic duct due to inju- ry. The mean size of tumor was 28.4 mm. Postoperative complications were found in five patients： 3 pancreatic leakages, a pancreatitis, and a duodenal stricture. Pancreatic leakages were improved by external drainage. No perioperative mortality was observed and all patients are recorded alive during the mean follow-up period of 17.2 mo.
CONCLUSION： In selected patients after careful evaluation, LPHR can be used for the treatment of branch duct type IPMN. In order to avoid pancreatic ductal injury, pre- and intra-operative definite localization and careful operative techniques are required.     

Localized type 1 autoimmune pancreatitis superimposed upon preexisting intraductal papillary mucinous neoplasms

A 70-year-old woman was found to have 2 cystic lesions in the head of the pancreas on abdominal ultrasonography during a routine medical examination.Endoscopic ultrasonography(EUS)and magnetic resonance cholangiopancreatography showed multilocular cysts in the head of the pancreas without dilation of the main pancreatic duct.The patient was followed-up semiannually with imaging studies for suspected branch duct-type intraductal papillary mucinous neoplasm(IPMN).At 3 years after initial presentation,hypoechoic lesions were observed around each pancreatic cyst by EUS.Diffusion-weighted imaging showed high-intensity regions corresponding to these lesions.Therefore,a diagnosis of invasive carcinoma derived from IPMN could not be excluded,and subtotal stomach-preserving pancreaticoduodenectomy was performed.The macroscopic examination of the surgical specimen showed whitish solid masses in the head of the pancreas,with multilocular cysts within each mass.Microscopically,each solid mass consisted of inflammatory cells such as lymphocytes and plasma cells.Furthermore,immunochemical staining revealed immunoglobulin G4-positive cells,and many obliterating phlebitides were observed.The cysts consisted of mucus-producing epithelial cells and showed a papillary growth pattern.Based on these findings,we diagnosed multiple localized type 1 autoimmune pancreatitis occurring only in the vicinity of the branch ducttype IPMN.