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。     

Multifocal intraductal papillary mucinous neoplasm of the pancreas-A case report

Cystic neoplasms of the pancreas are relatively rare, comprising 10 percent of pancreatic cysts and only 1 percent of pancreatic cancers. Cystic neoplasms include mucinous cystic neoplasms, serous cystadenomas, papillary cystic tumors, cystic islet cell tumors and intraductal papillary mucinous neoplasms of the pancreas （IPMNs）. IPMN was first described in 1982. It has been most commonly described in 60 to 70 years old males, and represents a relatively ＂new＂ but increasingly recognized disease. The improvement and widespread use of modern imaging equipments and heightened awareness of physicians contribute to the increasing incidence of IPMN. The majority of IPMNs are located in the pancreatic head （75%） while the rest involves the body/tail regions. Multifocal IPMNs have been hypothesized, but the true presence of multifocality is unknown. Here we present a 72-year- old male diagnosed with IPMN （carcinoma in situ） in the pancreatic head and a branch duct type IPMN （duct atypia） in the pancreatic body and tail. The patient underwent a Whipple intervention and a distal pancreatectomy. A three-year disease-free survival has been observed so far.     

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.     

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.     

Malignancies associated with intraductal papillary mucinous neoplasm of the pancreas

AIM: As intraductal papillary mucinous neoplasm (IPMN) has a favorable prognosis, associated malignancies have potential significance in these patients. We examined the incidence and characteristics of pre-existing, coexisting and subsequent malignancies in patients with IPMN. METHODS: Seventy-nine cases of IPMN were diagnosed by detection of mucous in the pancreatic duct during endoscopic retrograde pancreatography. Histological diagnosis was confirmed in 30 cases (adenoma (n=19) and adenocarcinoma (n =11). Other primary malignancies associated with IPMN, occurring in the prediagnostic or postdiagnostic period, were investigated. Postdiagnostic follow-up period was 3.3±0.5 years (range, 0.2-20 years). RESULTS: Other 40 malignancies occurred in 28 patients (35%). They were found before(n=15), at (n=19) and after (n=6) the diagnosis of IPMT. Major associated malignancies were gastric cancer (n=12), colonic cancer (n=7), esophageal cancer (n=4), pulmonary cancer (n=4), and independent pancreatic cancer (n=3). Pancreatic cancer was synchronous with IPMN in two patients and metachronous in one (3 years after diagnosis of IPMN). Thirty-one lesions were treated surgically or endoscopically. Fourteen patients died of associated cancers. Development of other malignancies was related to age (71.9±8.2 vs66.8±9.3, P<0.05), but not to gender or site of the tumor. CONCLUSION: IPMN is associated with a high incidence of other malignancies, particularly gastric and colonic cancers. Common genetic mechanisms between IPMN and other associated malignancies might be present. Clinicians should pay attention to the possibility of associated malignancies in preoperative screening and follow-up of patients with IPMN.     

Dilated papilla with mucin extrusion is a potential predictor of acute pancreatitis associated with intraductal papillary mucinous neoplasms of pancreas

Background/objectivesAs intraductal papillary mucinous neoplasm (IPMN) of the pancreas is associated with acute pancreatitis (AP) in some cases, predicting the risk of pancreatitis is as important as predicting the risk of malignancy in IPMN cases. In this study, we attempted to clarify the characteristics of IPMN associated with AP, compared to those of IPMN not associated with AP.MethodsFrom January 2006 to March 2013, data from 88 patients who underwent surgery for IPMN were retrospectively investigated and analyzed. We evaluated clinical and pathological variables of each patient and compared patients with IPMN with AP to those without AP. Furthermore, we presented representative cases of mild and severe pancreatitis caused by IPMN.ResultsOverall, 12 of 88 patients with IPMN (13.6%) had AP. Seven of the 12 patients had a single episode of AP, whereas the remaining 5 patients were diagnosed with IPMN with repeated AP. Ten of 12 patients with AP were diagnosed with mild AP and the remaining 2 with severe AP. Regarding clinical findings, the proportion of dilated papilla with mucin extrusion was significantly higher in patients with IPMN with AP than in those without AP (p = 0.035). Histological findings indicated that the proportion of intestinal-subtype IPMN was significantly higher in patients with IPMN with AP (p = 0.013).ConclusionsAP caused by IPMN derives mostly from intestinal IPMN. Dilated papilla with mucin extrusion can be a potential predictor of AP.     

不同病理类型的胰腺导管内乳头状黏液性肿瘤的临床特征

目的 总结不同病理类型胰腺导管内黏液性乳头状瘤(IPMT)的临床表现,提高对IPMT的诊治水平.方法 回顾分析1996年3月至2008年9月间经手术切除且病理证实的49例IPMT患者的临床表现、影像学特点及病理学特征,比较分析良性、交界性、恶性IPMT的临床表现.结果 49例IPMT患者中男27例,女22例,平均年龄(58±11)岁.良性19例,交界性9例,恶性21例.3类IPMT患者在性别、发病年龄、烟酒史、胰腺炎发作史、糖尿病病史,是否存在腹痛、腰背部放射痛、腹胀、腹泻、消瘦等症状,血CEA、AST、ALT水平,肿瘤位置及肿瘤分型上的差异均无明显统计学意义.但黄疸、术前CA19-9和碱性磷酸酶水平、肿瘤直径、主胰管直径、囊性肿瘤内是否存在隔膜、附壁结节的大小等方面的差异有统计学意义.恶性IPMT术后5年有1例病死,1例复发;交界性和良性IPMT有1例复发,1例因其他疾病病死.结论 不同病理类型的IPMT 临床特征存在一些差异,需综合判断进行鉴别.     

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...     

Endoscopic ultrasonography findings of pancreatic parenchyma for predicting subtypes of intraductal papillary mucinous neoplasms

Background and aimsThe subtypes of intraductal papillary mucinous neoplasms (IPMNs) are closely associated with the clinicopathological behavior and recurrence after surgical resection. However, there are no established non-invasive methods to confirm the subtypes of IPMNs without surgery. The aim of this study is to predict the subtypes of IPMNs using the findings of endoscopic ultrasonography (EUS).MethodsSixty-two consecutive patients with IPMNs who underwent EUS before surgery were retrospectively reviewed. The following EUS findings were analyzed and their relationship with the subtypes was evaluated: diameter of the main pancreatic duct, cyst size, number of cysts, height of mural nodule, early chronic pancreatitis (CP) finding, fatty parenchyma and atrophic parenchyma.ResultsThe subtypes of IPMNs were as follows: gastric (G)-type 38 (61%), intestinal (I) -type 14 (23%) and pancreatobiliary (PB) -type 10 (16%). Fatty parenchyma was significantly associated with G-type (P < 0.0001). Early CP findings ≥2 and atrophic parenchyma were significantly correlated with I-type (P < 0.0001). PB-type was significantly associated with pancreatic parenchyma without early CP findings or fatty degeneration in comparison to the other subtypes (P < 0.0001). Using the above characteristic EUS findings, the sensitivity, specificity, and accuracy were as follows: 63%, 92% and 74%, respectively, in G-type, 57%, 96% and 87% in I-type, and 90%, 94% and 94% in PB-type.ConclusionsThe evaluation of EUS findings, especially focused on the pancreatic parenchyma, has the potential to predict the subtypes of IPMN.     

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.     

Helicobacter pylori is undetectable in intraductal papillary mucinous neoplasm

BackgroundAbout half of the world population is infected with Helicobacter pylori (H. pylori), a bacterium associated with gastric cancer and considered to be a risk factor for pancreatic ductal adenocarcinoma. Whether the bacterium is associated with intraductal papillary mucinous neoplasm, believed to be a precursor of pancreatic ductal adenocarcinoma, is unknown. The aim of this study was to investigate the presence of H. pylori DNA in tissue sections of intraductal papillary mucinous neoplasm.MethodsThe presence of H. pylori DNA was tested in a retrospective controlled study of formalin-fixed, paraffin-embedded pancreatic tissues from 24 patients who underwent surgery for intraductal papillary mucinous neoplasm. Histologically normal tissues surrounding neoplasms were used as control. H. pylori DNA was evaluated after deparaffinization, DNA extraction, and purification, and results were evaluated statistically.ResultsSamples were collected from 13 males and 11 females with mean age 59 years (range 44–77), and consisted of 19 cases of main-duct and three cases of branched-duct intraductal papillary mucinous neoplasm. Two patients were diagnosed with pancreatic cancer and main-duct intraductal papillary mucinous neoplasm. H. pylori DNA was not detected either in intraductal papillary mucinous neoplasm tissue, or in surrounding normal tissue.ConclusionsAlthough H. pylori has been implicated in pancreatic ductal adenocarcinoma, it may not play a key role in the development of intraductal papillary mucinous neoplasm.     

胰管内乳头状黏液性肿瘤的术前良、恶性与浸润性预测因子

目的 分析术前预测胰管内乳头状黏液性肿瘤(IPMN)良、恶性与浸润性的因子,及不同病理类型IPMN的手术后生存率.方法 回顾性分析长海医院1993年1月至2009年9月间手术切除的78例IPMN病例资料,采用单因素与多因素分析的方法分析病史、临床表现、肝功能、CEA、CA19-9、影像学征象等在术前判断IPMN良、恶性与浸润性的价值,分析患者术后生存率.结果 单因素分析结果显示,黄疸、急性胰腺炎、血CA19-9>37 U/ml、AKP、肿块边界不清为恶性及浸润性预测因子;主胰管扩张、分支胰管直径>30 mm、出现壁节结等为恶性预测因子;CEA>6 ng/ml为浸润性预测因子.多因素分析显示,肿块边界不清为恶性及浸润性预测因子;急性胰腺炎为浸润性预测因子.良性IPMN患者的5年生存率为100%;恶性IPMN的2年生存率为78.9%、5年生存率为68.5%,其中浸润性IPMN的2年生存率为64.6%、5年生存率为43.1%.结论 肿块边界不清为IPMN恶性预测因子,急性胰腺炎、肿块边界不清是IPMN的浸润性预测因子.     

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…     

Clinicopathological and prognostic significance of MUC13 and AGR2 expression in intraductal papillary mucinous neoplasms of the pancreas

Background

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a primary pancreatic ductal epithelial neoplasm with the potential to develop into an invasive adenocarcinoma. This study aimed to investigate the clinicopathologic and prognostic significance of four potential biomarkers for the preoperative evaluation of patients with IPMN.

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.     

Clinicopathological features and medical management of intraductal papillary mucinous neoplasms

BACKGROUND: Intraductal papillary mucinous neoplasms (IPMN) are a clinicopathological entity that is being diagnosed with increasing frequency. However, the best approach to medical management of IPMN needs to be clarified. The aim of the present study was to identify preoperative features that may be predictors of malignant IPMN, and to define the medical management of IPMN of the pancreas. METHODS: A total of 23 patients who underwent surgical resection for IPMN of the pancreas at Kochi Medical School between 1982 and 2004 were examined. Multivariate Cox regression analysis was used to identify factors independently associated with IPM carcinoma. RESULTS: Among the 23 patients, 12 had IPMN adenoma, three had borderline IPMN, four had IPMN with carcinoma in situ, and four had IPMN with invasive carcinoma. In multivariate analysis, elevated serum carcinoembryonic antigen (CEA) and carbohydrate antigen (CA) 19-9 levels were found to be preoperative predictors of malignant IPMN. These results suggest that the following IPMN of the pancreas should be resected: (i) IPMN of the pancreas situated in the main duct; (ii) IPMN located in the branch duct if the size of the cystic lesion is >30 mm and the mural nodules are >5 mm in height by endoscopic ultrasound (EUS); and (iii) the diameter of the main pancreatic duct is >10 mm by endoscopic retrograde pancreatography (ERP). Careful observation of patients with branch-type IPMN with small cysts and/or without mural nodules is recommended as a management strategy. CONCLUSION: The present study reinforces the need for immediate surgical resection of malignant IPMN and suggests indicators for IPMN that should assist physicians in making decisions on treatment options.