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A pancreaticogastric fistula related to intraductal papillary mucinous neoplasm of the pancreas developed during follow‐up: A case report and literature review
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Intraductal papillary mucinous neoplasms (IPMN) are mucin producing tumors which arise from epithelial cells of the main pancreatic duct, pancreatic branch ducts, or both. They are characterized by mucin-producing columnar cells, papillary ductal proliferation, cyst formation, and varying degrees of dysplasia. IPMNs are classified as main duct or branch duct based upon the pancreatic duct anatomy which the IPMN is arising from. Additionally, they can be classified based on their histologic subtypes, which carry varying associations with dysplasia and/or malignancy. Many patients have incidentally identified IPMNs, which are asymptomatic. However, patients may also present with pancreatitis, elevation of liver enzymes, dilation of the pancreatic duct or bile duct as well as distention of the ampullary pancreatic orifice(s), due to impaction and obstruction with mucus. This is known as an endoscopically visualized "fish eye" sign. Patients may also develop exocrine and endocrine pancreatic insufficiency and maldigestion. Some studies also suggest that patients with IPMNs may also be at increased risk for gastric, colorectal, biliary, renal cell, and thyroid malignancies. Rarely, IPMNs can be complicated by fistulation between the main pancreatic duct and neighboring organs. Herein, we present an unusual case of simultaneous fistulation to both the gastric body and the duodenum.
We herein report a rare case of intraductal papillary mucinous neoplasm with a pancreatogastric fistula in an elderly Japanese man admitted to our hospital. The pancreatogastric fistula was confirmed using endoscopic retrograde pancreatography via a cannulated guidewire placed in the stomach. Six months after admission, the patient was diagnosed with intraductal papillary mucinous carcinoma. A pancreatogastric fistula is generally a rare complication of intraductal papillary mucinous neoplasm. It was caused by mechanical penetration in this case. Interestingly, we also observed endoscopic and histochemical mucosal changes in the fistula.
Background
Intraductal papillary mucinous neoplasms (IPMN) of the pancreas complicated by fistula formation to adjacent organs is an uncommon phenomenon. We present an IPMN of the pancreas with malignant transformation and multiple fistulae to stomach and duodenum.
Case Presentation
A 50-year-old female was referred for investigation of recent epigastric pain and a past history of recurrent pancreatitis. Imaging with computed tomography showed gross dilatation of the entire pancreatic duct with heterogeneous enhancement of the periductal parenchyma. Passage of oral contrast was noted from greater curvature and pylorus of the stomach into the dilated duct suggestive of fistulae formation. Gastro-duodenoscopy demonstrated these fistulae in the stomach and the proximal duodenum, and an exophytic growth at the ampulla obliterating the view of ampullary opening. Endosonography (EUS) guided fine needle aspiration cytology (FNAC) showed cells with high grade atypia. A total pancreatectomy, distal gastrectomy and splenectomy was performed and recovery was uneventful. Histology revealed a ductal adenocarcinoma arising from an intestinal type intraductal papillary mucinous neoplasm with high grade dysplasia. A year and a half after surgery, she is healthy with good glycaemic control and nutritional status.
Conclusion
This case highlights the importance investigating patients for the aetiology in recurrent acute pancreatitis and their follow-up. Awareness of cystic pancreatic neoplasms including IPMN is important to avoid misdiagnosis or delayed diagnosis. Referral of these patients to centres with facilities for multidisciplinary input and specialised management is strongly recommended.
Intraductal papillary mucinous neoplasms (IPMN) of the pancreas are characterized by proliferation of mucin-secreting cells in the main pancreatic duct (PD) or its branches. The secreted thick mucin usually leads to PD obstruction and dilation. A common complication of IPMN is recurrent acute pancreatitis secondary to poor pancreatic fluid drainage, and rarely, pancreatobiliary and pancreatointestinal fistulae. We describe a unique case of IPMN in a 57-year-old male who was referred to our institution for evaluation of recurrent acute pancreatitis. After extensive evaluation, he was diagnosed with main duct IPMN. Intraductal PD biopsy revealed intestinal type IPMN with intermediate grade dysplasia. Patient was managed clinically by large caliber (10 French) PD stenting which eliminated his recurrent acute pancreatitis. The patient was initially referred for pancreatic resection; however, surgery was aborted and evaluated to be high risk with high morbidity secondary to the extensive adhesions between the pancreas and surrounding structures. Patient remained clinically stable for a few years except for an episode of acute pancreatitis that happened after a trial of stent removal. Subsequently, the patient did well after the PD stent was replaced. Recently, repeat abdominal imaging revealed a large pancreatoduodenal fistula which was confirmed on repeat endoscopic retrograde cholangiopancreatography. We were able to perform pancreatoscopy by advancing a regular upper scope through the fistula and into the PD. Interestingly, the fistula relieved the symptoms of obstruction and subsequently decreased the frequency of recurrent pancreatitis episodes with no further episodes at 6 months follow-up. This case highlights the importance of providing adequate PD drainage to reduce the frequency of recurrent acute pancreatitis in the setting of main duct IPMN, especially if the patient is not a surgical candidate. Also, physicians need to monitor for complications such as fistula formation between the pancreas and surrounding structures in the setting of chronic inflammation due to recurrent episodes of pancreatitis. Early identification of a fistula is important for surgical planning. Furthermore, since recent studies suggested a higher incidence of additional primary malignancies in patients with IPMN of the pancreas compared to the general population, patients may be considered for screening for other primary malignancies.
BACKGROUND Intraductal papillary neoplasm of the bile duct (IPNB) is pathologically similar to intraductal papillary mucinous neoplasm (IPMN). However, there are several significant differences between them. The rate of IPMN associated with extrapancreatic malignancies has been reported to range from 10%-40%, and it may occasionally be complicated with the presence of fistulas. IPMN associated with malignant IPNB is extremely rare and only nine cases have been reported in the literature. CASE SUMMARY We report a 52-year-old man who presented with recurrent cholangitis for nine months. Computed tomography and magnetic resonance cholangiopancreatography showed the common bile duct stricture with dilated pancreatobiliary duct without other abnormal findings. The underlying pathogenesis could not be identified based on the radiologic images. Endoscopic retrograde cholangiopancreatography revealed a pancreatobiliary fistula with dilated main pancreatic duct, biliary stricture with dilated biliary tree, and mucus discharge from the enlarged orifice of the major papilla. The patient underwent SpyGlass cholangiopancreatoscopy due to a suspected mucin-producing biliary neoplasm and indeterminate main pancreatic duct dilatation. Multiple papillary growing neoplasms with vascular images, with the extent of lesions spreading in the biliopancreatic ductal lumens, were identified by SpyGlass. In addition, the presence of a pancreatobiliary fistula was also identified. The patient was diagnosed as having benign IPMN and malignant IPNB with focal invasion by postoperative pathology. Furthermore, varying histological subtypes were present in both IPMN and IPNB. Pylorus-preserving pancreaticoduodenectomy was performed on the patient with excellent results during the 52 month followup period. CONCLUSION We deemed that pancreatography and SpyGlass allowed for an efficient diagnosis of IPMN with pancreatobiliary fistula, whereas the etiology could not be identified by radiologic imaging.
One of the rare complications of low-grade pancreatic neoplasms is fistulization into nearby structures. This often does not present clinically, but is incidentally identified in patients who have been imaged serially to monitor the progression of the disease. In this report, we present an uncommon complication of an intraductal papillary mucinous neoplasm, which developed a spontaneous gastropancreatic fistula in a patient who was conservatively managed. The clinical course, imaging features, and management of this case are discussed.
Background:
Intraductal papillary-mucinous neoplasms (IPMNs) are potentially malignant intraductal epithelial neoplasms that sometimes penetrate into other organs. To the best of our knowledge, no report has yet described a case with penetration into the spleen. We recently encountered a case of IPMN with penetration of the stomach and spleen that was successfully treated by total pancreatectomy.
Case presentation:
A 70-year-old female visited our hospital with a complaint of fever and abdominal pain. Contrast-enhanced computed tomography (CT) revealed dilatation of the main pancreatic duct in the entire pancreas and penetration into the stomach and spleen. Upper gastrointestinal endoscopy revealed mucin extruding from four openings of the fistula in the stomach. No malignancy was detected based on cytology of the mucin. Inflammation markers and tumor markers (CEA, CA19-9) were elevated in the blood. The pre-operative diagnosis was IPMN of main pancreatic duct type penetrating into the stomach and spleen. A total pancreatectomy and splenectomy were performed, combined with distal gastrectomy including resection of the fistulas between the pancreas and stomach. No postoperative complications were noted. Histopathological examination of the resected specimen revealed atrophy of the pancreatic parenchyma, and the main duct of the pancreas was filled with mucin. Mucin-producing malignant tumor cells were detected in the epithelium of the main pancreatic duct with no signs of invasion. No malignancy was found at the fistulas between the pancreas and stomach or spleen. The patient was finally diagnosed with non-invasive intraductal papillary-mucinous carcinoma (IPMC) of main pancreatic duct type. Mechanical penetration was suspected as a mechanism of the penetration. The patient remained disease-free without evidence of recurrence more than 15 months after the operation.
Conclusion:
Though IPMNs sometimes penetrate into other adjacent organs, penetration into two organs, including the spleen, is rare. The rare case of IPMC penetrating into the stomach and spleen presented here was treated successfully by total pancreatectomy.
Introduction:
Intraductal papillary mucinous neoplasms (IPMNs) occasionally involve formation of fistulas with other adjacent organs. Pancreatobiliary fistulas associated with IPMNs are rare, but affected patients often develop obstructive jaundice and cholangitis.
Presentation of case:
A 79-year-old man was referred to our hospital for evaluation of abnormal biliary enzymes. Contrast-enhanced computed tomography and endoscopic retrograde cholangiopancreatography demonstrated multiple cystic lesions with septa in the pancreatic head and fistulas between the cystic lesions and common bile duct. The clinical diagnosis was pancreatobiliary fistula associated with a mixed-type IPMN and accompanying obstructive jaundice. The patient underwent subtotal stomach-preserving pancreaticoduodenectomy. The resected specimen showed fistulas between the cystic lesions and common bile duct. Histopathological examination showed that the main and branch ducts of the pancreatic head were dilated and filled with mucus. The epithelia of the pancreatic ducts revealed papillary proliferation and an invasive adenocarcinoma arising from an intraductal neoplasm. Immunohistochemistry examination showed CDX2- and MUC2-positive reactions. The final diagnosis was an intraductal papillary mucinous carcinoma of the intestinal-type. The patient remained disease-free for 9 months postoperatively.
Discussion:
The causes of death in patients who have pancreatobiliary fistulas associated with IPMNs without resection are cholangitis or hepatic insufficiency. Nonoperative treatment is limited for cases with obstructive jaundice. It is necessary to prevent obstructive jaundice and cholangitis due to a large quantity of mucinous material.
Conclusions:
Surgical resection should be considered, if possible, in patients with pancreatobiliary fistulas associated with IPMNs. A better prognosis is expected with prevention of obstructive jaundice or cholangitis.
Background:
A 68-year-old asymptomatic patient was incidentally diagnosed with an intraductal papillary mucinous neoplasia (IPMN) of the pancreas with a pancreaticogastric fistula. He had a history of a right sided nephrectomy due to a renal cell carcinoma 9 years before. The patient underwent an uneventful total pancreatectomy and wedge resection of the stomach.
Methods:
The patient's medical history was studied and compared to recent literature via PubMed.
Results:
Pathohistological evaluation confirmed a mixed type IPMN of an intestinal subtype with pancreaticogastric fistula.
Conclusion:
Pancreaticogastric fistula due to benign IPMN is extremely rare. Surgical resection including wedge resection of the stomach is the treatment of choice.
Evidence-based guidelines on the management of pancreatic cystic neoplasms (PCN) are lacking. This guideline is a joint initiative of the European Study Group on Cystic Tumours of the Pancreas, United European Gastroenterology, European Pancreatic Club, European-African Hepato-Pancreato-Biliary Association, European Digestive Surgery, and the European Society of Gastrointestinal Endoscopy. It replaces the 2013 European consensus statement guidelines on PCN. European and non-European experts performed systematic reviews and used GRADE methodology to answer relevant clinical questions on nine topics (biomarkers, radiology, endoscopy, intraductal papillary mucinous neoplasm (IPMN), mucinous cystic neoplasm (MCN), serous cystic neoplasm, rare cysts, (neo)adjuvant treatment, and pathology). Recommendations include conservative management, relative and absolute indications for surgery. A conservative approach is recommended for asymptomatic MCN and IPMN measuring <40 mm without an enhancing nodule. Relative indications for surgery in IPMN include a main pancreatic duct (MPD) diameter between 5 and 9.9 mm or a cyst diameter ≥40 mm. Absolute indications for surgery in IPMN, due to the high-risk of malignant transformation, include jaundice, an enhancing mural nodule >5 mm, and MPD diameter >10 mm. Lifelong follow-up of IPMN is recommended in patients who are fit for surgery. The European evidence-based guidelines on PCN aim to improve the diagnosis and management of PCN.
Pancreatobiliary fistulas associated with intraductal papillary mucinous neoplasms (IPMN) often develop obstructive jaundice and cholangitis; thus, early diagnosis is important. However, computed tomography and cholangiography, the current methods for detecting pancreatobiliary fistulas, are not always effective. We previously reported a case of IPMN-associated pancreatobiliary fistula and proposed a potential new diagnostic marker: the "pig-nose" appearance of the duodenal papilla, which results from dilated pancreatic and bile ducts and can be visualized via endoscopy. In this study, we report another three cases of IPMN-associated pancreatobiliary fistulas detected by a different technology, intraductal ultrasonography (IDUS). As with our previously reported case, we confirmed the utility of the "pig-nose" appearance and IDUS in the diagnosis of IPMN-associated pancreatobiliary fistulas. In addition, we found it difficult to manage biliary obstruction that resulted from the flow of mucinous material through pancreatobiliary fistulas. The obstruction was treated with endoscopic nasal biliary drainage (ENBD), but this was not always successful. In two of our cases, additional treatment with a large diameter fully covered metal stent failed to improve jaundice. Therefore, we conclude that standard endoscopic stenting may not be effective, and that alternative endoscopic methods or surgery may be necessary.
Background:
Intraductal papillary mucinous neoplasm (IPMN) is a rare tumor that originates in the pancreatic duct. The diagnosis of benign, borderline or malignant to IPMN is significant in terms of making an appropriate treatment plan and prognosis. This article summarizes our clinical experience of a case report and discussion by literature review. Methods and case report: A 73 year old male patient was admitted for an occupying lesion of the pancreas. The magnetic resonance cholangiopancreatography (MRCP) scan considered IPMN, endoscopic retrograde cholangiopancreatography (ERCP) also confirmed diagnosis of IPMN. Both the biliary and pancreatic duct stents were replaced, but we did not obtain any evidence by cytological evaluation. One month later, ERCP and intraductal ultrasonography (IDUS) showed infiltrating growth of the tumor. Endoscopic ultrasonography guided fine-needle aspiration was performed at the same time, and pathological diagnosis was suggested as borderline IPMN.
Results:
In the absence of pathological support, the patient presented with the clinical diagnosis of infiltrating intraductal papillary mucinous adenocarcinoma (IPMC) and was recommended for surgery. However, the patient and his family refused surgery, and were discharged. Subsequently, the patient died 6.5 months (197 days) following first diagnosis.
Conclusions:
Currently, the definition and classification of IPMN is done by specification, although there remain some difficulties in diagnosing its subtypes. For diagnostic purposes, CT, MRCP, ERCP, IDUS, EUS and EUS-FNA can all be applied. Cytological negative pathology might not completely rule out malignancy, and would still require further examination and follow-up.
Intraductal papillary mucinous neoplasm (IPMN) accounts for 20-50% of all cystic neoplasms of the pancreas. Rarely, IPMN, whether benign or malignant, can fistulize into adjacent organs like duode-num, stomach or common bile duct. IPMN can be associated with other diseases like Peutz-Jeghers syndrome and familial adeno-matous polyposis. Association with adult polycystic kidney disease (ADPKD) is extremely rare. We report a case of a 60-year-old male with a large IPMN in the head of the pancreas diagnosed by magnetic resonance imaging, endoscopic ultrasound and cyst fluid analysis. It was complicated by fistula formation into the second part of the duo-denum. Patient was simultaneously having adult polycystic kidney disease. There is only one case report of uncomplicated IPMN with ADPKD in the literature so far. And even rarer, there is no any case report of fistulizing IPMN with ADPKD reported so far, to the best of our knowledge.
Intraductal papillary mucinous neoplasms (IPMNs) are benign cystic lesions of the pancreas with recognised premalignant potential. An occasional feature of IPMNs is fistula formation to surrounding organs. This report describes a case of a pancreaticogastric fistula from a main duct IPMN that produced the complete resolution of the patient's symptoms.
We report on a case of a 74 year old man who was diagnosed with a recurrence of non-invasive carcinoma of intraductal papillary mucinous neoplasm (non-invasive IPMN) by postoperative gastroscopy (GS). A pylorus preserving pancreatico duodenectomy for IPMN in the pancreatic head was performed. A histopathological study revealed non-invasive adenocarcinoma. At first, the local recurrence of the tumor around the superior mesenteric artery circumference was diagnosed and disappeared with gemcitabine. Later, the GS showed the elevated lesion with mucin hypersecretion in the remnant stomach. The lesion had a central dip and a fistula common to the pancreas was confirmed on fisterography. We diagnosed a recurrence of IPMN and administered chemotherapy again. However, he died of his original illness. There are no reports of postoperative recurrence of IPMN checked by GS. It should be remembered that the elevated lesion of the remnant stomach is considered as one of the recurrent patterns of IPMN.
Invasive intraductal papillary-mucinous neoplasms (IPMNs) of the pancreas may be associated with pancreaticogastric fistulas as shown by case reports. We report the case of a benign IPMN associated with pancreaticogastric and pancreaticoduodenal fistulas. A 70-year-old woman was admitted with intestinal obstruction. Computed tomography and MRI showed a large dilatation of the main pancreatic duct (>1 cm) with intraductal nodules, and pancreaticogastric and pancreaticoduodenal fistulas. Several features in imaging were present to support a malignant IPMN, so that the patient underwent a pancreaticoduodenectomy. The histopathological examination of the surgical specimen showed a benign IPMN. This case proves that a benign IPMN can cause pancreaticogastric and pancreaticoduodenal fistulas, probably resulting from mechanical factors.
Fistula formation has been reported in intraductal papillary-mucinous neoplasms (IPMNs) with or without invasion of the adjacent organs. The presence or absence of invasion is mostly determined by postoperative histological examination rather than by preoperative work-up.
A 72 year-old Japanese woman showed remarkable dilatation of the main pancreatic duct (MPD) in the distal region of the pancreas. Subsequent ERCP also showed MPD dilatation, after which the patient suffered moderate pancreatitis. A subsequent gastroscopy revealed a small ulceration that had not been observed in a gastroscopy performed 3 months prior. Mucinous discharge from the ulceration suggested it might be the orifice of a fistula connected to the MPD. En bloc resection including the distal region of the pancreas, spleen, stomach and part of the transverse colon was performed under the pre- and intraoperative diagnosis of an invasive malignant IPMN. However, histopathology revealed the lesion to be of "borderline malignancy" without apparent invasion of the stomach. Light microscopy showed inflammatory cellular infiltrates (mainly neutrophils) around the pancreatogastric fistula, but there was no evidence of neoplastic epithelia lining the fistulous tract.
This case highlights that a pancreatogastric fistula can develop after acute inflammation of the pancreas in the absence of cancer invasion. Further information regarding IPMN-associated fistulae is necessary to clarify the pathogenesis, diagnosis, appropriate surgical intervention and prognosis for this disorder.
Intraductal papillary mucinous neoplasm (IPMN) sometimes forms fistulas with other organs due to high pressure of pancreatic duct filled with huge amount of mucus. Pancreatobiliary fistula may cause obstructive jaundice due to the mucus and it is hard to manage the jaundice by endoscopic biliary stenting because of high viscosity of the bile. We report a case of IPMN with pancreatobiliary fistula managed by endoscopic ultrasound (EUS)-guided hepaticogastrostomy (HGS). The patient was 87 years old and presented with obstructive jaundice. As a transpapillary biliary stent was considered to have a risk of migration due to the absence of bile duct stenosis, a nasobiliary catheter was placed as an initial drainage. However, the catheter was frequently obstructed by mucus. The patient was intolerable for surgery because of his age. Considering the intrahepatic bile was serous, EUS-HGS was performed and jaundice improved successfully. This case study revealed that EUS-HGS might be a therapeutic option for obstructive jaundice caused by an IPMN-associated pancreatobiliary fistula.
Intraductal papillary mucinous neoplasms (IPMNs) occasionally form a fistula to adjacent organs, resulting in obstructive jaundice and cholangitis due to mucus obstruction. Although some procedures such as endoscopic nasobiliary drainage are attempted, they often do not work adequately because of high mucus viscosity. Herein, we report the case of an 87-year-old man with obstructive cholangitis treated by endoscopic septotomy and mucus suction with direct peroral cholangioscopy using conventional endoscopy. The patient incidentally showed a branched-type IPMN in the pancreatic head on computed tomography (CT) approximately 10-years ago. Although the patient’s tumor had grown slowly and he occasionally developed cholangitis, he did not want surgery. He was admitted to our hospital because of cholangitis by mucus obstruction with a PB fistula. Endoscopic retrograde cholangiopancreatography (ERCP) and septotomy were performed. Septotomy made the duodenal papilla a large orifice, thereby facilitating spontaneous drainage of mucus. In addition, conventional endoscopy with a large working channel enabled direct access into the orifice and smooth mucus suction, thereby alleviating his cholangitis. In conclusion, septotomy and direct peroral cholangioscopy using conventional endoscopy could be useful to control biliary tract infection and obstructive jaundice due to mucus obstruction from an IPMNs with PB fistula.
Pancreatic cysts are very common with the majority incidentally identified. There are several types of pancreatic cysts; some types can contain cancer or have malignant potential, whereas others are benign. However, even the types of cysts with malignant potential rarely progress to cancer. At the present time, the only viable treatment for pancreatic cysts is surgical excision, which is associated with a high morbidity and occasional mortality. The small risk of malignant transformation, the high risks of surgical treatment, and the lack of high-quality prospective studies have led to contradictory recommendations for their immediate management and for their surveillance. This guideline will provide a practical approach to pancreatic cyst management and recommendations for cyst surveillance for the general gastroenterologist.
The management of intraductal papillary mucinous neoplasm (IPMN) continues to evolve. In particular, the indications for resection of branch duct IPMN have changed from early resection to more deliberate observation as proposed by the international consensus guidelines of 2006 and 2012. Another guideline proposed by the American Gastroenterological Association in 2015 restricted indications for surgery more stringently and recommended physicians to stop surveillance if no significant change had occurred in a pancreatic cyst after five years of surveillance, or if a patient underwent resection and a non-malignant IPMN was found. Whether or not it is safe to do so, as well as the method and interval of surveillance, has generated substantial debate. Based on a consensus symposium held during the meeting of the International Association of Pancreatology in Sendai, Japan, in 2016, the working group has revised the guidelines regarding prediction of invasive carcinoma and high-grade dysplasia, surveillance, and postoperative follow-up of IPMN. As the working group did not recognize the need for major revisions of the guidelines, we made only minor revisions and added most recent articles where appropriate. The present guidelines include updated information and recommendations based on our current understanding, and highlight issues that remain controversial or where further research is required.
Fistula as a complication of pancreatic intraductal papillary mucinous neoplasms (IPMN) is rare and may involve different adjacent organs, sometimes, several organs at the same time. Our patient had a pancreatico-gastric fistula, discovered at work-up for IPMN, which required extensive surgery.
Intraductal papillary mucinous neoplasm (IPMN) of the pancreas runs along a large spectrum from benign adenoma to invasive carcinoma.(1) Fistulas between the pancreas and adjacent organs, such as the duodenum, bile duct, and stomach, have been reported,(2-4) but benign pancreaticogastric fistulas have rarely been reported.(5) Herein, we report a patient who was diagnosed with main duct type pancreatic IPMN complicated with spontaneous pancreaticogastric fistula formation. This article is protected by copyright. All rights reserved.
The objectives of this study were to determine the frequency with which intraductal papillary mucinous pancreatic neoplasms (IPMNs) show fistulization to adjacent organs and to describe the multidetector row computed tomography (MDCT) and magnetic resonance imaging (MRI) findings for this specific complication.
A retrospective analysis of the clinical and imaging files of all patients with IPMNs who were followed over 8 years by our department was performed to identify those with fistula formation. Two radiologists determined the type of IPMN, the number and size of visible fistulas, the involved adjacent organs, the pancreatic location and the presence of imaging findings suggestive of malignant transformation of the IPMN. Histological correlation was also performed.
A total of 423 patients were included. Fistula formation was present in 8 patients (1.9%). The corresponding IPMNs were of the main duct type (n=4; 50%), the branch duct type (n=1; 13%) or the mixed type (n=3; 38%). In half of the cases, these tumors were discovered incidentally. A total of 26 fistulas (1-7 per patient) were identified. These fistulas involved the duodenum (65.4%), stomach (19.2%), common bile duct (11.5%) and colon (3.8%). All patients had fistulas to the duodenum. All fistulas appeared to develop from a malignant IPMN based on the imaging studies, but two of the five available samples did not exhibit atypia (a quarter of all fistulas). In 50% of cases, the IPMN was of the intestinal form.
Fistulas are uncommon complications of IPMNs, regardless of malignant transformation of the IPMNs. Fistulas appear to predominate among malignant main-duct IPMNs, are generally multiple and affect several organs, and their preferential target is the duodenum. However, fistulas do not adhere to a strict criterion of malignancy.
Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.
The international consensus guidelines for management of intraductal papillary mucinous neoplasm and mucinous cystic neoplasm of the pancreas established in 2006 have increased awareness and improved the management of these entities. During the subsequent 5 years, a considerable amount of information has been added to the literature. Based on a consensus symposium held during the 14th meeting of the International Association of Pancreatology in Fukuoka, Japan, in 2010, the working group has generated new guidelines. Since the levels of evidence for all items addressed in these guidelines are low, being 4 or 5, we still have to designate them "consensus", rather than "evidence-based", guidelines. To simplify the entire guidelines, we have adopted a statement format that differs from the 2006 guidelines, although the headings are similar to the previous guidelines, i.e., classification, investigation, indications for and methods of resection and other treatments, histological aspects, and methods of follow-up. The present guidelines include recent information and recommendations based on our current understanding, and highlight issues that remain controversial and areas where further research is required.
Intraductal papillary mucinous neoplasm (IPMN) of the pancreas occasionally penetrates to others organs. We present a case of IPMN penetrating to the stomach and the common bile duct.
A 75-year-old man was admitted to the hospital because of epigastric pain. Computed tomography (CT) showed a papillary tumor protruding into the markedly dilated main pancreatic duct and splenic vein obstruction. The tumor was diagnosed as IPMN arising in the main duct, but he rejected surgery and he was followed without treatment. One year later, gastroduodenoscopy revealed gastropancreatic fistula and we were able to pass an endoscope through the fistula and directly examine the lumen of the main pancreatic duct and the papillary tumor adjacent to the fistula. Absence of malignant cells on histopathology suggested mechanical penetration rather than invasive penetration. CT showed splenic vein reperfusion due to decreased inner pressure of the main pancreatic duct. Two and a half years later, CT revealed biliopancreatic fistula formation. Endoscope biliary drainage was performed but failed. Despite jaundice, he is still ambulatory and seen in the clinic three years after the first admission.
We have experienced a case of IPMN penetrating to the stomach and the common bile duct that has taken a slow course. It represents the importance of distinguishing mechanical penetration from invasive penetration as well as mechanical splenic vein obstruction from splenic vein invasion.
Here, we report a case of a pancreatobiliary (PB) fistula caused by an intraductal papillary mucinous neoplasm (IPMN) of the pancreas. The PB fistula was suspected after endoscopic retrograde cholangiopancreatography (ERCP) and diagnosed after direct visualization with a direct peroral cholangioscopy and pancreatoscopy by using an ultra-slim endoscope. No previous reports exist on the precise diagnosis of a PB fistula with direct peroral cholangioscopy and pancreatoscopy. In our case report, a 69-year-old man underwent an ERCP because of a pancreatic head mass and biliary tract obstruction. During ERCP, a fistula between the common bile duct (CBD) and main pancreatic duct (MPD) was suspected. After endoscopic sphincterotomy, we examined both the CBD and MPD with an ultra-slim videoendoscope (GIF-N260; Olympus Optical Co, Tokyo, Japan) under direct visualization and biopsy of the mass. The analysis of the biopsy specimen confirmed this mass to be an IPMN of the pancreas. When we examined the CBD, one fistula with copious mucin secretion was identified at the distal CBD. In conclusion, direct peroral cholangioscopy and pancreatoscopy using the ultra-slim endoscope is an efficient tool for diagnosis of PB fistula and pancreatic IPMN.
To correlate the CT and MR images with pathologic findings on intraductal papillary mucinous neoplasms (IPMNs) complicated with intraductal hemorrhage, perforation, and fistula.
We retrospectively evaluated the CT (n = 5), MR imaging (n = 4), and pathological features of five IPMN patients complicated with intraductal hemorrhage (n = 5), perforation (n = 1), and fistula into the duodenum and jejunum (n = 1).
Intraductal hemorrhage could be detected as high attenuation on non-contrast CT in two of the five cases, and as high signal intensity on fat-suppressed T1-weighted MR images in all four of the cases. Perforation and fistula could be recognized on CT images. In all IPMNs, denuded epitheliums were observed pathologically. Dissolution of the duct wall and extension of mucinous materials were seen at the area of denuded epithelium. Perforations and fistula, without evidence of cancer invasion, were recognized in the dissolved duct wall. Pathogenesis of the perforations and fistula formations appeared to be related to excessive pressure in the dilated ducts and autodigestion of enzyme-rich fluids.
Complications with IPMN could be recognized on CT and fat-suppressed T1-weighted MR images. Intraductal hemorrhage might be predictive sign of perforation and fistula formation.
Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas present more commonly in the elderly. This report describes a case of IPMN in a 36-year-old man who presented with obstructive jaundice and weight loss. The initial investigation by computed tomography scan revealed a cystic lesion in the head of pancreas fistulating into the duodenum and the common bile duct (CBD). Subsequent endoscopic retrograde cholangiopancreatography revealed a low CBD stricture with proximal filling defects. Mucin was observed extruding from the biliary orifice following an endoscopic sphincterotomy. A classic Whipple's pancreatoduodenectomy was performed to excise the lesion. A histological examination of the lesion confirmed the presence of a malignant IPMN of the pancreas complicated by pancreatobiliary and pancreatoduodenal fistulae.
This study assessed the mechanism of fistula formation in intraductal papillary mucinous neoplasm (IPMN) of the pancreas.
A total of 274 patients with IPMN who had been diagnosed by endoscopic retrograde cholangiopancreatography and endoscopic ultrasonography (EUS) at our center were enrolled. The patients with IPMN which had fistula formation into other organs were investigated retrospectively as to (1) clinical prevalence and the organs penetrated by IPMN, (2) analysis of the mechanism of fistula formation by immunohistopathological study, (3) efficacy of EUS in progression assessment, and (4) prognosis.
Among the subjects, fistula formation into other organs was observed in 18 patients (6.6%) and into 28 organs. There were 7 patients (39%) in whom multiple organs were penetrated. Of 16 patients who had undergone investigation of the expression of mucin markers, 94% had an intestinal-type tumor. Of 9 patients who had undergone surgery or autopsy, 67% showed mechanical penetration without invasion around the fistula. Only papillary protrusions were seen by EUS in 4 of these patients with noninvasive papillary adenocarcinoma showing mechanical penetration. All 5 patients who had pancreatic parenchymal invasion showed a mass with a mixed-echo pattern in addition to papillary protrusions shown by EUS, corresponding to colloid carcinoma.
There were 2 processes in the development of fistulas in IPMN. Of those patients showing fistula formation, 94% had intestinal-type IPMN, and 67% showed mechanical penetration. Delineation of a mass with the mixed-echo pattern suggested an invasive penetration due to colloid carcinoma.
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 neoplasm (IPMN) of the pancreas may extend to other organs. However, it is rare for a histopathologically benign IPMN to rupture other organs, particularly multiple organs. There has been no report of a benign IPMN rupturing both the stomach and duodenum.
We experienced a very rare case and make personal remarks based on bibliographical consideration.
Case report.
National Defense Medical College.
A patient with IPMN.
EGD, ERCP, and pancreatoduodenectomy.
We report a case of benign IPMN of the pancreas extending to two adjacent organs. A 77-year-old male who was diagnosed as having IPMN by CT, MRI, upper GIF, and ERCP underwent pancreatoduodenectomy for a mass of 4.2 cm in diameter. Pathological examinations revealed that the IPMN was composed of adenoma. Intraluminal nodular growth was observed in the duodenal gland tissue, and abnormal growth was observed in the fistula to the stomach. According to a literature review based on PubMed data up until March 2009, it is rare for a benign IPMN to penetrate two adjacent organs.
We describe an unusual case of biliopancreatic fistula, free perforation, and subsequent abscess formation within the lesser peritoneal sac associated with intraductal papillary mucinous carcinoma (IPMC). A 71-year-old man presented with general fatigue and loss of appetite that had persisted for 1 month. Abdominal computed tomography (CT) revealed findings consistent with an intraductal papillary mucinous neoplasm (IPMN) of the pancreas, accompanied by abscess formation in the bursa omentalis. Gastrointestinal fiberscopy revealed a swollen papilla of Vater expanded by sticky mucus, and a communication between the pancreatic duct and bile duct was demonstrated by the injection of indigo carmine solution into the pancreatic duct. Percutaneous transhepatic abscess drainage (PTAD) was performed on the day of admission. After this procedure, the patient was managed for 1 month and supported nutritionally with glycemic control for diabetes mellitus. After admission, the patient had an episode of obstructive jaundice that was treated by retrograde biliary drainage. Pancreaticoduodenectomy with lymph node dissection was then performed. Pathological examination revealed IPMN with patchy, scattered carcinoma of the pancreatic head and uncinate process with the formation of a biliopancreatic fistula. Bile duct epithelium in the area of the biliopancreatic fistula demonstrated atypical papillary epithelium suggestive of tumor invasion.
Intraductal papillary-mucinous neoplasms (IPMN) were officially introduced into the TNM classification in 1996. Based on a two-center database, we reevaluated histopathological findings, clinicopathological pattern, predictive markers for malignancy, and outcome.
Between 1996 and 2006, a total of 1424 pancreatic resections were performed in the University Hospitals Dresden and Mannheim. Pathologists of both institutions reviewed the IPMN diagnoses and other with cystic or solid tumor diagnoses. All possible markers, such as diabetes, jaundice, etc., were analyzed for prediction of malignancy. We performed a survival analysis based on the morphologic classification to determine the prognosis of IPMN.
There were 43 patients of primarily diagnosed IPMN along with 1174 patients with diagnoses, such as ductal adenocarcinoma. In 207 patients, the diagnoses revealed other cystic or small solid tumors. A histopathological review of the latter patients revealed 54 IPMNs, resulting in a total of 97 IPMN patients (29 noninvasive, 68 invasive). All IPMN patients had a median survival of 36 months. Recurrence occurred more frequently in invasive IPMN. Predictive markers of malignancy were pain, preoperative weight loss, jaundice, and elevated CA 19.9. The strongest independent prognostic factor was invasive growth. The survival analysis revealed excellent prognosis for noninvasive IPMN.
Since the introduction of IPMN in 1996, even specialized centers have had to deal with a learning curve. By reevaluating all cystic or small solid tumors, centers can improve and their patients' treatment can be optimized. Because the preoperative diagnostic methods are not sensitive enough to differentiate between benign and malignant lesions, surgery is advocated for all main duct IPMN, because they have a high malignant potential. For branch duct IPMN, surgery is advocated if the lesion is symptomatic, >3 cm, or has enlarged nodules.
Although there have been recent reports of mucin-producing tumor of the pancreas, there has been no thorough clinicopathological analysis of a large number of cases.
Two hundred forty four cases of mucin-producing tumor of the pancreas from Japanese, European and American reports, together with 15 cases of our own, were analyzed clinicopathologically.
Mucin-producing tumor of the pancreas was found in 177 males and 82 females (M:F = 2.2:1). The mean age was 65.5 years. Jaundice, diabetes mellitus and a past history of pancreatitis were found in 15%-19% of the cases. The tumor was most frequently (62%) found in the head of the pancreas. Pathologically, hyperplasia or adenoma was found in 58 cases, and adenocarcinoma in 160 cases. Five-year-survival rate by the Kaplan-Meier method was 82.6% in all of the cases and postoperative survival curve was much better in cases with this type of carcinoma than in cases with ordinary pancreatic duct cell carcinoma (5-year-survival rate: 82.6% vs 17.3%). Serum tumor markers such as CEA or CA 19-9 were not effective in differentiating between benign and malignant, or in determining the degree of cancerous spread, while cytology of the pancreatic juice and biopsy of the tumor could contribute to the diagnosis.
Mucin-producing tumor has unique clinicopathological characteristics, such as the dilated main pancreatic duct or branches, dilatation of the orifice of the papilla of Vater, or good prognosis. Since a diagnosis for benign or malignant is very difficult in some cases, methods for distinguishing benign from malignant lesions or for determining cancerous spread, such as molecular biological techniques, should be established.
An 81-year-old woman was admitted with epigastric pain and weight loss. She had been diagnosed with an intraductal papillary mucosal tumor (IPMT) 7 years previously, but had refused surgery for religious reasons. Esophagogastroduodenoscopy revealed a nodular, elevated lesion that was discharging mucin into the duodenal bulb and posterior wall of the upper body of the stomach. Endoscopic ultrasonography, abdominal computed tomography, and endoscopic retrograde cholangiography were carried out, and a highly invasive IPMT with simultaneous invasion of the stomach and duodenum was diagnosed.
Intraductal papillary-mucinous tumor of the pancreas is occasionally accompanied by biliopancreatic fistula. However, it is difficult to show the inflow of mucin produced by the tumor into the common bile duct. To confirm the biliopancreatic fistula, the mucin-rich fraction was purified from the bile and stained with antimucin antibodies. Western blot analysis showed characteristic smear staining patterns for mucin molecules with three types of antimucin antibodies. Immunohistochemical analysis with the antibody showed significant signals of the cancer cells and the luminal content of the dilated pancreatic duct. These results showed that the bile contained an abundance of mucin, which was produced by the primary pancreatic tumor. In cases with intraductal papillary-mucinous tumor of the pancreas, biochemical analysis of mucin molecules in the bile can be of clinical use in consideration of pathological process of tumor progression.
Non-inflammatory cystic lesions of the pancreas are increasingly recognized. Two distinct entities have been defined, i.e., intraductal papillary mucinous neoplasm (IPMN) and mucinous cystic neoplasm (MCN). Ovarian-type stroma has been proposed as a requisite to distinguish MCN from IPMN. Some other distinct features to characterize IPMN and MCN have been identified, but there remain ambiguities between the two diseases. In view of the increasing frequency with which these neoplasms are being diagnosed worldwide, it would be helpful for physicians managing patients with cystic neoplasms of the pancreas to have guidelines for the diagnosis and treatment of IPMN and MCN. The proposed guidelines represent a consensus of the working group of the International Association of Pancreatology.
We report a pancreatobiliary fistula caused by an intraductal papillary-mucinous pancreatic neoplasm (IPMN), manifesting as obstructive jaundice. Computed tomography showed dilatation of the bile duct and main pancreatic duct, with multiple cystic masses in the head of the pancreas. Endoscopic retrograde pancreatocholangiography showed a patulous papilla with mucin secretion. Contrast enhancement outlined amorphous material obstructing the lower part of the common hepatic duct. Pancreatogram and magnetic resonance cholangiopancreatography showed diffuse dilatation of the main pancreatic duct and side branches without communication with the adjacent organs or duct. We performed pancreaticoduodenectomy for IPMN of the pancreatic head and a tumor-like lesion in the lower common bile duct (CBD). Macroscopically, impacted thick mucus protruded into the CBD from the pancreas via a pancreatobiliary fistula. Histologic examination revealed a pancreatobiliary fistula caused by intraductal papillary-mucinous carcinoma of the pancreas with mucin hypersecretion, an adenoma without interstitial infiltration, and isolated implantation of an IPMN in the bile duct mucosa around the fistula.