Pancreatoscopy assisted by endoscopic sphincter dilation

To investigate tumor extension to the main pancreatic duct in patients with intraductal papillary mucinous tumors, we conducted pancreatoscopy assisted by endoscopic sphincter dilation (ESD). Two patients with intraductal papillary mucinous tumors were included in this study. The endoscopic sphincter dilation was performed using an Olbert balloon-tipped catheter. A mother-baby scope system was used for endoscopic examination. After dilation of the orifice, the babyscope was inserted smoothly. Tumorous lesions (especially the typical salmon-roe appearance of the tumors) were clearly observed in the main pancreatic duct in one patient, while no tumorous lesions were identified in the other patient. The ESD procedure may be applicable for assisting insertion of the endoscope through the papilla of Vater into the pancreatic duct. ESD is useful both for treating and for diagnosing pancreatic lesions. Received: June 19, 2001 / Accepted: February 22, 2002 Reprint requests to: N. Ueno, Present address: Third Department of Internal Medicine, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama 236-0004, Japan     

IPMN PENETRATION OF THE STOMACH

An 83‐year old Japanese man was transferred to our hospital due to a 1‐week history of melena and signs of disordered awareness. Esophagogastroduodenoscopy showed a villous tumor associated with massive white mucous discharge in the posterior wall of the gastric corpus, where pathologically identified mucin‐producing epithelium with nuclear atypia had developed into a papillary form. An abdominal enhanced computed tomography scan demonstrated communication between the dilated main pancreatic duct and the gastric lumen. Based on these findings, we reached a diagnosis of gastric penetration by an intraductal papillary mucinous neoplasm (IPMN) of the main pancreatic duct. IPMN is partly characterized by expansive mucinous growth that may result in penetration into adjacent organs.     

Intraductal papillary-mucinous adenoma developed in the ventral pancreas in a patient with pancreas divisum

A 34-year-old man was admitted to our hospital with the chief complaints of back pain and epigastralgia. The physical examinations on admission disclosed no abdominal tumor. The serum concentration of total bilirubin was 1.4?mg/dl. The serum elastase-1 level was elevated to 526?ng/dl. Computed tomography showed a cystic lesion, 1?cm in diameter, in the head of the pancreas, without dilatation of the main pancreatic duct. Endoscopic retrograde cholangiopancreatography via the papilla of Vater and the accessory papilla revealed an enlarged ventral pancreatic duct and pancreas divisum. The preoperative diagnosis was mucin-producing pancreatic tumor in the ventral pancreas of a patient with pancreas divisum. A pylorus-preserving pancreatoduodenectomy was performed. The gross findings of the cut surface of the resected specimen disclosed mural nodules in the dilated duct of the ventral pancreas. A histological examination of the mural nodules in the ventral pancreas revealed mucin and intraductal papillary adenoma. Benign tumors associated with pancreas divisum are rare; to the best of our knowledge, only three cases have been reported. Although in these three patients the tumor developed in the dorsal pancreas, the tumor developed in the ventral pancreas in our patient.     

Invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma without mucin hypersecretion but with changes in the pancreatic duct on pancreatography

We present a case of invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma without mucin hypersecretion in a 65‐year‐old man with a 45‐year history of alcohol abuse and a 2‐year follow‐up of chronic pancreatitis. Two years previously, in May 1998, he was admitted for investigation of abdominal pain. Computed tomography (CT) showed diffuse dilation of the main pancreatic duct with atrophy of the pancreatic parenchyma. Endoscopic retrograde pancreatography (ERP) showed a diffusely dilated main pancreatic duct with irregular side branches in the head of the pancreas. Chronic alcoholic pancreatitis was diagnosed on the basis of the pancreatography findings. The patient was readmitted for investigation of progressive weight loss in August 2000. Serum CA19‐9 levels were markedly elevated (750U/ml) and CT showed enlargement of the head and body of the pancreas. ERP showed irregularity of the main pancreatic duct in the head of the pancreas, and the distal main pancreatic duct (which was dilated on initial ERP examination) was interrupted in the body of the pancreas. Suspected pancreatic carcinoma was diagnosed, and pylorus‐preserving pancreatoduodenectomy was performed. Frozen section examination of the cut end of the pancreas revealed ductal carcinoma, and total pancreatoduodenectomy with portal vein resection was performed. Histologically, the resected tumor was diagnosed as an invasive carcinoma derived from intraductal papillary adenocarcinoma without mucin hypersecretion. We recommend observing changes in the pancreatic duct on pancreatography to diagnose invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma in a resectable state.     

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.     

CLINICAL SIGNIFICANCE OF INTRADUCTAL ULTRASONOGRAPHY FOR DIAGNOSIS OF INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM OF THE PANCREAS

Intraductal ultrasonography (IDUS) is useful for evaluating the horizontal spread along the main pancreatic duct in cases of intraductal papillary mucinous neoplasm (IPMN) of the pancreas, thus providing valuable information for the determination of the resection line at surgery. Differentiation between benign and malignant IPMN is also indispensable for management decisions. Measurement of the height of mural nodules by IDUS is expected to be useful for differential diagnosis of IPMN. Because IDUS cannot always demonstrate whole lesions, especially in branch duct IPMN, endoscopic ultrasonography plays a complementary role in such cases.     

Air in the main pancreatic duct associated with a pancreatic intraductal papillary mucinous neoplasm

A 62-year-old man was referred to our hospital after ultrasonographic mass screening detected a pancreatic cyst that proved to be an intraductal papillary mucinous neoplasm. Computed tomography additionally demonstrated air in the main pancreatic duct. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography delineated a filling defect in the main pancreatic duct in the body of the pancreas. The sphincter of Oddi was open. The main pancreatic duct was dilated by viscous mucin; air in the duct was attributed to consequent dysfunction of the sphincter. Laboratory findings included no significant abnormality. The patient has remained asymptomatic during follow-up. Of 25 previously reported cases with air in the duct, only 1 involved an intraductal papillary mucinous neoplasm.     

Intraductal papillary-mucinous tumor of the pancreas head with complete absence of the ventral pancreatic duct of Wirsung

A case of intraductal papillary mucinous tumor of the pancreas with complete absence of the ventral pancreatic duct of Wirsung is presented. A 74-year-old Japanese man was admitted to our hospital because of elevated serum amylase concentration. Abdominal computed tomography (CT) scanning revealed diffuse dilatation of the main pancreatic duct and a diffuse and uncircumscribed area with heterogeneous density in the pancreas head. Endoscopic retrograde cholangiopancreatography revealed that the main pancreatic duct was connected with an accessory papilla and was diffusely dilated, without any irregularity of the duct wall being observed in the entire length of the duct. The common bile duct was detected only by cannulation through Vater’s papilla, and no pancreatic duct or its communicating branch was found. Some branches, directed to the dorsal portion of the pancreas head, were found arising from the accessory pancreatic duct. Intraductal ultrasound examination performed through the accessory papilla and the common bile duct revealed a small tumor with a heterogeneous echo level in the pancreas head. From these findings, intraductal papillary-mucinous tumor (IPMT) occurring in the pancreas head was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. The resected specimen revealed IPMT in the pancreas head. A roentgenographic study of the resected specimen revealed a defect caused by the tumor located in the pancreatic duct connected with the accessory papilla and showed that there was complete absence of the pancreatic duct connected with Vater’s papilla. Surgical resection enabled us to completely analyze the duct system of pancreas divisum. Although it is not known whether there is a relationship between the pathogenesis of IPMT and embryological anomaly of the pancreatic duct system, this case may provide an insight into the pathogenesis of IPMT.     

Case of intraductal papillary mucinous tumor (noninvasive adenocarcinoma) of the pancreas resected 27 years after onset

Summary A case of intraductal papillary mucinous tumor (IPMT) of the pancreas resected 27 yr after onset is presented. In March of 1997, a 71-yr-old man was admitted to our hospital with a complaint of severe epigastric pain. He had initially undergone endoscopic retrograde pancreatography (ERP) in April 1971 in our hospital and the patient had been followed up for pancreatografic changes for 26 yr. Dilatation of the main pancreatic duct gradually progressed during follow-up, and the filing defect owing to the tumor became demonstrable. On admission, ERP revealed diffuse dilatation of the main pancreatic duct, which was 20 mm in diameter, and the filling defect of 35 mm in diameter. We diagnosed this patient as having an IPMT of the pancreas. Considering his general condition, pancreatic segmentectomy was carried out, and the postoperative course was favorable. Histological findings were compatible with those of noninvasive papillary adenocarcinoma. This is a precious case for studying the natural history of intraductal papillary tumor of the pancreas and to evaluate the application of surgery, because the biologic behavior of this tumor is much less aggressive than that of pancreatic ductal cell carcinoma.     

PERORAL PANCREATOSCOPY AND INTRADUCTAL ULTRASONOGRAPHY IN DETERMINING THE RESECTION LINES OF INTRADUCTAL PAPILLARY MUCINOUS TUMORS OF THE PANCREAS

In patients with malignant intraductal papillary–mucinous tumors (IPMT), the tumor spread along the main pancreatic duct is generally inspected as mural nodules within the ducts by peroral pancreatoscopy (POPS). Even the lower mural nodules including fish‐eggs‐like lesions and granular mucosa continued to the main taller mural nodules should be resected. Mural nodules along the main duct and cystic dilation of the branch ducts observed by intraductal ultrasonography (IDUS) are regarded as the tumor spread. Although POPS is superior to IDUS in identifying mural nodules along the main duct, POPS has limitations to detect mural nodules within the branch ducts. Therefore, the accuracy of the diagnosis by POPS is higher in main duct IPMT, whereas that by IDUS is higher in branch duct IPMT. These modalities should be performed as complementary tests in determining the resection lines preoperatively.     

Intraductal papillary adenocarcinoma with mucin hypersecretion and coexistent invasive ductal carcinoma of the pancreas with apparent topographic separation

We report a 66-year-old man who had a cystic intraductal papillary adenocarcinoma containing a papillary adenoma, in the head of the pancreas and a coexistent invasive, well differentiated solid tubular adenocarcinoma in the tail of the pancreas. He was hospitalized with acute epigastralgia. Computed tomography demonstrated a multilocular cystic mass in the head of the pancreas and a solid tumor in the tail. Endoscopic retrograde pancreatography showed mucin secretion from an enlarged papilla of Vater, marked dilatation of the main pancreatic duct in the head and body, cystic dilatation of the uncinate branch, and irregular narrowing of the main pancreatic duct in the tail. Total pancreatectomy was performed. Between the cystic tumor and the solid tumor there was a distance of 4.8 cm of normal pancreatic parenchyma and duct, recognized both grossly and microscopically. The patient died 35 months after the operation. At autopsy, peritonitis carcinomatosa was found in the abdominal cavity. Microscopically, disseminated nodules were also well differentiated tubular adenocarcinoma. The apparent anatomic separation of these two tumors within the pancreas is extremely unusual.     

European experts consensus statement on cystic tumours of the pancreas

Cystic lesions of the pancreas are increasingly recognized. While some lesions show benign behaviour (serous cystic neoplasm), others have an unequivocal malignant potential (mucinous cystic neoplasm, branch- and main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm). European expert pancreatologists provide updated recommendations: diagnostic computerized tomography and/or magnetic resonance imaging are indicated in all patients with cystic lesion of the pancreas. Endoscopic ultrasound with cyst fluid analysis may be used but there is no evidence to suggest this as a routine diagnostic method. The role of pancreatoscopy remains to be established. Resection should be considered in all symptomatic lesions, in mucinous cystic neoplasm, main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm as well as in branch duct intraductal papillary mucinous neoplasm with mural nodules, dilated main pancreatic duct >6 mm and possibly if rapidly increasing in size. An oncological partial resection should be performed in main duct intraductal papillary mucinous neoplasm and in lesions with a suspicion of malignancy, otherwise organ preserving procedures may be considered. Frozen section of the transection margin in intraductal papillary mucinous neoplasm is suggested. Follow up after resection is recommended for intraductal papillary mucinous neoplasm, solid pseudo-papillary neoplasm and invasive cancer.     

ENDOSCOPIC DIAGNOSIS OF INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM USING PERORAL PANCREATOSCOPY WITH NARROW BAND IMAGING

A 77‐year‐old man was diagnosed with a pancreas cyst at another hospital. Abdominal ultrasonography revealed a cyst in the head of the pancreas and a small protrusion. These findings suggested intraductal papillary mucinous neoplasm. Cytologic finding of the pancreatic juice revealed a Class III lesion, and intraductal ultrasonography and peroral pancreatoscopy (PPS) were performed. An abnormal course of main pancreatic duct (MPD) prevented the insertion of an ultrasonography probe into the MPD, and PPS was performed. The mucosal surface of the MPD near the papillary area was normal, and narrow band imaging (NBI) clearly showed the vascular structure. When the PPS was inserted more deeply, many small protrusions were observed and NBI delineated the protrusions more clearly. Papillary protrusions were observed in the cyst, but NBI did not reveal any tumor vessels. These findings led to a diagnosis of benign intraductal papillary mucinous neoplasm lesion. Since then, follow‐up examinations have been made. Changes in the cyst and protrusion have not been observed.     

Intraductal papillary mucinous neoplasm of the pancreas with a bifid pancreatic duct

A bifid pancreatic duct presenting a major bifurcation in the main pancreatic duct is one of the anatomical variations of the pancreatic ducts. We encountered a 71-year-old female with a 5-cm-diameter branch duct intraductal papillary mucinous neoplasm of the pancreas in whom preoperative endoscopic retrograde pancreatography demonstrated an anomalous bifurcation of the main pancreatic duct at the body of the pancreas. We performed a distal pancreatectomy, instead of a middle pancreatectomy, with a cutting line at the downstream pancreas to the duct bifurcation point. Intraoperative ultrasonography was useful to confirm the exact location of the pancreatic duct bifurcation as well as the tumor extension. The procedure resulted in a favorable outcome without any postoperative complications. Although a bifid pancreatic duct is an unusual anomalous condition, this case should alert surgeons to be aware of such anatomical variants when performing pancreatic resection, otherwise, incurable pancreatic complications may occur postoperatively.     

A case of intraductal papillary mucinous neoplasms after recurrent acute pancreatitis

We report a case of main pancreatic duct (MPD)-type intraductal papillary mucinous neoplasms of the pancreas (IPMNs), in whom diagnostic imaging modalities showed abnormal findings after 4 episodes of acute pancreatitis. The patient was 51 years old at his first admission for acute pancreatitis. He experienced two more episodes of acute pancreatitis, though repeated computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP) showed no abnormality to explain the cause of the pancreatitis. After 3? years from his first episode of pancreatitis, CT and endoscopic ultrasonography revealed pancreatic duct dilation of the pancreas head. Seven years after the first admission, a second ERCP and intraductal ultrasonography revealed a partially dilated MPD with papillary tumors. He underwent pancreaticoduodenectomy, and the pathological diagnosis was intraductal papillary mucinous adenoma with moderate atypia. This case suggests that acute pancreatitis can precede visualized IPMNs. Therefore, acute recurrent pancreatitis with unknown etiology should be followed up for the possibility of IPMNs, in order to detect neoplastic changes in the early stage to provide a better prognosis for the patient.     

IDUS IN THE DIAGNOSIS OF IPMT

Intraductal papillary mucinous tumor of the pancreas (IPMT) is considered as having low‐grade malignant potential, and malignancy is not infrequent. Therefore, accurate diagnosis is indispensable for appropriate patient care. IPMT is classified mainly into two categories based on the distribution of tumor, the main duct type and the branch duct type. In the diagnosis of IPMT, there are four important points: differentiation of IPMT from other pancreatic pathologies; differentiation of malignancy from benign lesions; evaluation of tumor extent along the MPD; and investigation of duct cell carcinoma coexistent with or derived from IPMT. IPMT should be distinguished from chronic pancreatitis, ductal adenocarcinoma, or cystic neoplasms. IPMT often requires pancreaticoduodenectomy for cure, but such invasive surgery should be avoided in patients with benign hyperplasia. Mural nodules in the MPD can be clearly visualized by intraductal ultrasonography (IDUS). Measurement of the height of the tumor mass by IDUS is useful in distinguishing benign from malignant IPMT. Intraductal spread of IPMT along the MPD is demonstrated by IDUS as irregular thickening of the MPD wall. To achieve a tumor‐free margin in surgery, evaluation by IDUS of the extent of the tumor along the MPD is important. The expected accuracy of IDUS in the diagnosis of invasive IPMT is reportedly over 90%.     

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.     

The role of pancreatoscopy in the preoperative evaluation of intraductal papillary mucinous tumor of the pancreas

BACKGROUND: Intraductal papillary mucinous tumor of the pancreas is a rare neoplasm managed by operative resection of the affected segment of the pancreas. GOALS: To evaluate the role of peroral pancreatoscopy in the diagnosis and preoperative localization of the affected region of the pancreatic duct and to undertake the appropriate operation for each patient. STUDY: Five patients with suspected intraductal papillary mucinous tumor of the pancreas were studied using endoscopic retrograde cholangiopancreatography, computed tomography of the abdomen, endoscopic ultrasonography, and peroral pancreatoscopy. The findings from these studies were compared, and operative resection was performed in each patient based on pancreatoscopic findings. RESULTS: Of the five patients with suspected intraductal papillary mucinous tumor, only four had histologically confirmed tumor, and the remaining one patient had a retention cyst of the pancreas. Pancreatoscopy correctly identified all four patients with the tumor while excluding the diagnosis of papillary tumor in one. CONCLUSION: Peroral pancreatoscopy is valuable in the preoperative evaluation of intraductal papillary mucinous tumor of the pancreas, especially in the localization of such tumor.     

Consensus Guidelines in the Management of Branch Duct Intraductal Papillary Mucinous Neoplasm: A Cost-Effectiveness Analysis

Background and Aims  

Based on consensus guidelines, surgical resection of branch duct intraductal papillary mucinous neoplasm (BD-IPMN) is indicated in patients with symptoms of cyst size ≥30 mm, intramural nodules, or dilated main pancreatic duct greater than 6 mm. The aim of this study was to determine the cost effectiveness of consensus guideline implementation in the management of BD-IPMN.     

Endoscopic diagnosis of intraductal papillary mucinous neoplasm of the bile duct

Background/Purpose

Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is considered an uncommon tumor. The purpose of this study was to evaluate the diagnostic utility of endoscopic cholangiography (ERC) with subsequent peroral cholangioscopy (POCS) and/or intraductal ultrasonography (IDUS) for this tumor.

Methods

From December 1991 to November 2006, a retrospective analysis was made of eight patients with IPMN-B. Their clinical features and the endoscopic diagnostic strategy for POCS and IDUS were reviewed.

Results

In all the patients, ERC failed to show papillary tumors, due to coexisting mucin or biliary sludge. POCS was carried out after ERC and it showed the presence and locations of papillary tumors in all patients, except for one with a tumor in the peripheral intrahepatic bile duct (B3). IDUS was performed in seven of the eight patients; in five of these patients, intraductal protruding tumors were clearly visualized, whereas flat tumors were not identified in the remaining two patients. In one of the eight patients, endoscopic nasobiliary drainage did not remove the huge amount of mucin. Hence, this patient required subsequent percutaneous biliary drainage. Six of the eight patients underwent surgical treatment; five patients underwent a hepatic resection with or without extrahepatic bile duct resection and one underwent a pancreaticoduodenectomy. Five of the six operated patients are still alive; one patient died of gastric cancer 90 months after the operation (mean follow-up period, 45.3 months). The two remaining patients, who were considered inoperable due to major medical comorbidities, died of liver failure and cholangitis 3 and 6 months, respectively, after stent placement.

Conclusion

ERC failed to delineate intraductal papillary tumors, due to coexisting mucin. The presence and location of papillary tumors were correctly diagnosed by both POCS and IDUS, but POCS may be better than IDUS to diagnose the extent of the tumor.