Long-term follow-up of intraductal papillary adenoma of the pancreas

Intraductal papillary mucinous tumors of the main pancreatic duct are often considered to be premalignant or malignant, and therefore surgical resection is recommended. We report two autopsy cases of intraductal papillary mucinous tumor of the pancreas following long-term observation. The first patient was an 84-year-old man with early gastric cancer treated by endoscopic mucosectomy. The second patient was a 77-year-old man with hepatocellular carcinoma treated by percutaneous pure ethanol injection and transarterial embolization. In both patients, endoscopic retrograde pancreatography showed a diffusely dilated main pancreatic duct, with intraductal filling defects expressing mucus, as well as dilated side branches. Obvious intramural nodules were not detected. Due to their advanced age and personal requests, both patients were managed conservatively and followed nonoperatively. In the first patient, serial pancreatograms showed progression of the pancreatic duct dilatation. Both patients died of gastric cancer, the first patient 7¹/₂ years and the second, 10 years after first presentation, respectively. Autopsies revealed extensive intraductal papillary adenoma throughout the dilated mucus-filled main pancreatic duct. However, there was no evidence of progression to adenocarcinoma. Based on these observations, we suggest that, in patients with intraductal papillary mucinous tumor of the pancreas without obvious intramural nodules, even if the tumor is in the main pancreatic duct, pancreatectomy may not be mandatory, particularly in the elderly. Received: March 21, 2001 / Accepted: July 6, 2001 Reprint requests to: T. Kamisawa     

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.     

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.     

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.     

Intraductal Papillary Tumors of the Pancreas: Evaluation With Magnetic Resonance Cholangiopancreatography

Objective : We analyzed the findings of intraductal papillary tumors of the pancreas by magnetic resonance cholangiopancreatography (MRCP). Methods : Twelve patients with intraductal papillary tumors (main duct type,   n = 3  ; branch duct type,   n = 8  ; combined type,  n = 1  ) underwent endoscopic retrograde cholangiopancreatography (ERCP) (  n = 11  ) and MRCP, using half-Fourier acquisition single-shot turbo spin-echo (HASTE) sequences (  n = 12  ). Imaging findings were compared with operative and pathological findings in all the patients. Results : Although ERCP failed to fully depict the main pancreatic duct and cystic tumors in six of 11 patients because of copious intraductal mucin, MRCP provided complete images of these structures in all 12 patients. In main duct type tumors, MRCP demonstrated moderate-marked, diffuse dilation of the main pancreatic duct. Branch duct type tumors showed "grape-like" clusters of cysts with no or only mild, diffuse dilation of the main duct. In combined type tumors, MRCP demonstrated a markedly dilated main duct with a large unilocular cyst of the collateral duct. MRCP detected more mural nodules (75%, three of four patients) than ERCP (25%, one of four). MRCP allowed more precise diagnosis of the type, size, and extent of tumors than ERCP, as confirmed by pathologic findings. Conclusion : MRCP demonstrates intraductal papillary tumors less invasively and more completely than ERCP.     

Electronic pancreatoscopy for the diagnosis of pancreatic diseases

OBJECTIVES: Diagnostic pancreatoscopy was performed in 56 patients using the newly developed peroral electronic pancreatoscope (PEPS). The diagnostic value of the PEPS for pancreatic diseases was evaluated. METHODS: The PEPS (external diameter = 2.1 mm, bidirectional tip deflection) was developed with a minute 50,000-pixel interline charge-coupled device. Pancreatoscopy was performed by means of an endoscopic retrograde approach without sphincterotomy. The cases examined were normal (three), chronic pancreatitis (32), pancreatic cancer (eight), and intraductal papillary mucinous tumors (13). RESULTS: Of the 56 cases, 42 (75%) were adequately seen. In normal cases, fine capillary vessels were clearly visualized on the smooth whitish-pink mucosa. Findings in chronic pancreatitis included protein plugs, calcified stones, rough whitish mucosa, scar formation, edema, erythema, and indistinct capillary appearance. All the stenoses of chronic pancreatitis could be differentiated from those of pancreatic cancer with the PEPS. In the pancreatic cancer cases, all patients had stenoses or duct cutoffs; most cases had friable mucosa with erythema and erosive changes, and a single patient had a compressed pancreatic duct wall covered with normal epithelium. In the cases of intraductal papillary mucinous tumors, papillary tumors were visualized with extreme clarity. In the case of adenocarcinoma, the PEPS revealed oval-shaped tumors with spotty redness or villous tumors with dilation of capillary vessels. Moderate acute pancreatitis was recognized after pancreatoscopy in one of the 56 cases (1.8%). CONCLUSIONS: Electronic pancreatoscopy with the PEPS is feasible in most patients and technically safe, and improves diagnostic yield over conventional pancreatoscopy.     

Intraductal papillary mucinous tumor with pancreatobiliary and pancreatodigestive fistulae: a case report

Pancreatobiliary or pancreatodigestive fistulae are rare during the course of intraductal papillary mucinous tumors. The mechanism of the fistula is either malignant involvement or mechanical. We report the case of an 81-year-old patient with intraductal papillary mucinous tumor complicated by fistulae between the main pancreatic duct and common bile duct, duodenum and stomach. These fistulae were treated endoscopically and radiologically by several metallic stents.     

Serous cystadenoma of the pancreas communicating with a pancreatic duct

Summary Conclusion To differentiate serous cystadenoma from other cystic lesions communicating with the pancreatic duct, careful radiological examination is necessary. Background Communication between the cystic cavity and the pancreatic duct in an ordinary serous cystadenoma is uncommon, although it is not uncommon in other cystic lesions, such as pseudocyst, mucinous cystadenoma/adenocarcinoma, and intraductal papillary tumor. Methods A serous cystadenoma of the pancreas communicating with main pancreatic duct in a 76-yr-old male is reported. Results The communication was preoperatively demonstrated by endoscopic retrograde cholangiopancreatography and confirmed by histopathological examination of the resected specimen.     

COMPARISON OF THE HEIGHT OF PAPILLARY TUMOR IN INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM OF THE PANCREAS BETWEEN MEASURED PREOPERATIVE IMAGES AND RESECTED MATERIAL

The height of the mural nodules and papillary tumors in main pancreatic duct or dilated branch duct is the most important factor for diagnosis of intraductal papillary mucinous neoplasm (IPMN). In this study, the authors compared the height of the papillary lesions and mural nodules between the height of resected tissues and the height detected by the preoperative imaging tools (endoscopic ultrasonography [EUS] and intraductal ultrasonography [IDUS]) in 38 patients with IPMN. In 21 out of 23 cases of adenoma, and in cases with the non‐invasive cancer, the difference of the height of operative and preoperative analysis measured by EUS and IDUS was within 1–2 mm. EUS and IDUS are useful for diagnosis of degree of malignancy in IPMN.     

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 mucinous tumors of the pancreas: the preoperative value of cytologic and histopathologic diagnosis

BACKGROUND: The preoperative diagnosis of intraductal papillary mucinous tumors of the pancreas must be as certain as possible because removal of a large portion of the pancreas or even total pancreatectomy may be necessary. The value of cytologic and histopathologic analysis of specimens obtained by preoperative endoscopic investigations is unknown. The aim of this study was to assess the value of such analyses of specimens obtained by EUS-guided FNA and/or biopsy, or transpapillary biopsy specimens obtained during endoscopic retrograde pancreatography for the diagnosis of intraductal papillary mucinous tumors of the pancreas and for the detection of malignancy. METHODS: Between 1992 and 2001, 42 patients (22 men, 20 women; median age 64 years) underwent surgical resection for intraductal papillary mucinous tumors of the pancreas and had preoperative pancreatic tissue sampling. In the case of isolated dilatation of pancreatic ducts, pancreatic juice was obtained by EUS-guided FNA for cytologic analysis. In the presence of a solid lesion or main pancreatic duct stenosis, biopsy specimens were obtained by EUS-guided FNA biopsy or endoscopic retrograde pancreatography, which permitted histopathologic assessment. The accuracy of cytology and histopathology was evaluated for the following: (1) positive diagnosis of intraductal papillary mucinous tumors of the pancreas and (2) assessment of malignancy, by comparison with histopathologic examination of surgical resection specimens. RESULTS: Cytologic analysis was performed in 19 patients; it was positive in 4 (21%) and noninformative in 15 (79%). Histopathologic analysis was performed in 23 patients; it was positive in 21 (91%) and negative in 2 (9%). Histopathologic analysis yielded a positive result in 83% of patients who did not have extrusion of mucus from a patulous papilla. The sensitivity, specificity, and positive and negative predictive values of histopathologic analysis for the diagnosis of malignancy were, respectively, 44%, 100%, 100%, and 33%. When histopathologic analysis was positive, the tumor grade was similar to that determined by final histopathologic examination in 38% of patients, whereas the grade was underestimated in 62%. No complication occurred as a result of tissue sampling. CONCLUSIONS: The sensitivity of histopathologic analysis of EUS-guided FNA biopsy specimens or transpapillary biopsy specimens is 91% for the positive diagnosis of intraductal papillary mucinous tumors of the pancreas with a solid component, which is of particular interest as extrusion mucus from the papilla was absent in most patients. Histopathologic analysis of biopsy specimens of malignant intraductal papillary mucinous tumors of the pancreas often underestimates tumor grade. The result for cytologic analysis of juice obtained from dilated pancreatic ducts is disappointing.     

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.     

A rare ring-shaped anomaly of the main pancreatic duct accompanying a branch duct IPMN

The patient was a 74-year-old female. Screening computed tomography for examination of the abdomen showed a cystic mass in the pancreatic body. Close investigation using endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography revealed a very rare finding: the main pancreatic duct bifurcated at the pancreatic body, and these two ducts converged at the caudal side. A multilocular cystic mass in the pancreatic body and mucus discharge from the orifice of major papilla were observed. There was no protruded lesion in the main pancreatic duct. No findings suggested apparent malignancy. The patient was diagnosed as having hyperplastic intraductal papillary mucinous neoplasm of branch type showing a ring-shaped pancreatic duct, and was placed under follow-up.     

EUS FNA in intraductal papillary mucinous tumors of the pancreas

BACKGROUND/AIMS: There is little information concerning the potential role of fine-needle aspiration guided by endoscopic ultrasonography in the pathologic diagnosis of intraductal papillary mucinous tumors of the pancreas. METHODOLOGY: Patients with an intraductal papillary mucinous tumor of the pancreas suggested by endoscopic ultrasonography underwent fine-needle aspiration guided by endoscopic ultrasonography in order to investigate the presence of mucin and/or cytologic changes consistent with this diagnosis. A group of 111 patients with other pancreatic lesions explored during the same period of time was used as a control group. RESULTS: Fine-needle aspiration guided by endoscopic ultrasonography was safely performed in 19 patients and supported the diagnosis in 17 of them. Nine out of the 17 patients with suspicion of intraductal papillary mucinous tumors of the pancreas went to surgery and this diagnosis was confirmed in the resected specimen in all of them. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of EUS FNA in the diagnosis of IPMT were 82%, 100%, 100%, 92% and 94% respectively. CONCLUSIONS: Fine-needle aspiration guided by endoscopic ultrasonography is a good technique to support the diagnosis of intraductal papillary mucinous tumors of the pancreas and should be considered in this group of patients if pathologic confirmation is judged to be necessary.     

Endoscopic ultrasound characteristics of mucinous cystic neoplasms of the pancreas

OBJECTIVE: Mucinous cystic neoplasms of the pancreas have a more favorable prognosis than ductal adenocarcinoma. Management of a subgroup, intraductal papillary-mucinous neoplasms, is controversial. Endoscopic ultrasound (EUS) with fine-needle aspiration biopsy may emerge as the imaging modality of choice. There are few studies describing the EUS features of these tumors. METHODS: A total of 35 consecutive cases of cystic tumors of the pancreas with an established pathological diagnosis were analyzed for characteristic EUS features. RESULTS: Mucinous cystadenocarcinomas (n = 14) were more likely to be characterized by hypoechoic cystic/solid mass or complex cyst and were frequently associated with a dilated main pancreatic duct. Benign mucinous duct ectasia (n = 6) were characterized by a dilated main pancreatic duct in conjunction with hyperechoic thickening of the duct wall. The two cases of intraductal mucinous hyperplasia additionally showed a hypoechoic mass. Intraductal papillary carcinoma (n = 11) had features in common with mucinous cystadenocarcinoma but also had echogenic foci in the mass and intraductal hyperechoic lesions. The two cases of microcystic cystadenoma showed either a mixed hypoechoic solid/cystic mass or a complex cyst without the additional features seen in mucinous cystadenocarcinoma. CONCLUSIONS: EUS features seem to exist that may help to differentiate cystic neoplasms from adenocarcinoma of the pancreas and, thus, to establish the preoperative diagnosis of cystic tumors of the pancreas.     

Temporary Pancreatic Duct Stenting to Prevent Pancreatitis Induced by Endoscopic Sphincter Dilation

Abstract: To investigate whether prophylactic temporary stenting of the main pancreatic duct would decrease the incidence of pancreatitis after endoscopic sphincter dilation (ESD), we conducted this procedure subsequent to ESD in 13 patients who had common bile duct stones. After ESD and extraction of stones, a stent was placed into the pancreatic duct across the papilla of Vater. The stent was removed endoscopically three days later. Stents were successfully placed in 12 (92.3%) patients. In 11 of the patients, there was no significant elevation of serum amylase values before and after the procedure. The remaining patient, whose stent was identified as occluded, demonstrated elevated serum amylase values. However, there were no other procedure-related complications. Although our report was only limited to 12 cases, our results suggest that temporary pancreatic stenting may help prevent postprocedual pancreatitis. (Dig Endosc 1999; 11:32–36)     

Intraductal mucinous tumors occurring simultaneously in the liver and pancreas

A case of simultaneous intraductal mucinous tumors of the liver and pancreas in a 67-year-old man is described. Abdominal ultrasonography and computed tomography (CT) revealed the presence of cystic lesions with intraluminal septae both in the caudate lobe of the liver and in the uncinate process of the pancreas; these cystic lesions communicated with the hepatic duct and pancreatic duct, respectively. Mucin retention was observed in the cysts, and cholestasis was induced by mucin secretion into the common bile duct. The lesions were resected by left hepatic lobectomy with caudate lobectomy, and segmental pancreatectomy. Both lesions were multilocular cystic tumors with no papillary projections or focal mass effect in their walls. Histologically, both cystic lesions were a mixture of hyperplasia and adenoma lined by low papillary columnar epithelium. There were no cellular or histological features to suggest malignant change. The fibrous intratumor interstitium lacked any mesenchymal or ovarian-like stroma. The hepatic lesion was considered to be of a similar nature to intraductal papillary mucinous tumor (IPMT) of the pancreas. However, the two lesions occurred simultaneously in the liver and pancreas. This case is of interest in regard to the diagnosis and management of mucinous hepatopancreatobiliary lesions. Received: March 16, 2001 / Accepted: September 14, 2001     

Role of SpyGlass-DStm in the preoperative assessment of pancreatic intraductal papillary mucinous neoplasm involving the main pancreatic duct

Background/Objectives

It is often difficult to determine an adequate resection line during pancreatectomy for intraductal papillary mucinous neoplasm involving the main pancreatic duct during partial pancreatectomy. The aim of this study was to evaluate the usefulness of improved peroral pancreatoscopy using SpyGlass-DS^tm in the preoperative assessment of intraductal papillary mucinous neoplasm involving the main pancreatic duct.

EUS in the evaluation of pancreatic cystic lesions

BACKGROUND: The differential diagnosis in pancreatic cystic lesions is often difficult despite the availability of various modern imaging modalities. This study assessed the role of EUS in the following: (1) discrimination of pseudocysts from pancreatic cystic tumors, (2) differential diagnosis between serous cystadenoma and mucinous cystic tumor, and (3) prediction of accompanying malignancy in intraductal papillary mucinous tumor. METHODS: EUS findings in 75 patients with pancreatic cystic lesions (58 cystic tumors, 17 pseudocysts) were evaluated. In the comparison of pseudocysts and cystic tumors, the latter included intraductal papillary mucinous tumor, mucinous cystic tumors, and serous cystadenomas, but not solid-pseudopapillary tumors. RESULTS: In univariate analysis, pseudocysts exhibited echogenic debris and parenchymal changes more often than cystic tumors did (respectively, 29% vs. 6%, p < 0.05; and 65% vs. 4%, p < 0.001). In contrast, septa and mural nodules were found more frequently in cystic tumors than pseudocysts (respectively, 69% vs. 12%, p < 0.001; 56% vs. 12%, p < 0.01). Multivariate analysis revealed that parenchymal changes (odds ratio [OR] = 83.59; p < 0.01); septa (OR = 30.75; p < 0.05); and mural nodules (OR = 21.38; p < 0.05) were independent predictors of differentiation between pseudocysts and cystic tumors. Serous cystadenoma exhibited diverse EUS features, as well as a honeycomb appearance. Mural nodules were found more often in mucinous cystic tumors than in serous cystadenomas (p < 0.05). There were no factors that predicted malignancy in intraductal papillary mucinous tumor. CONCLUSIONS: EUS is a useful complementary imaging method for differentiation of pancreatic cystic lesions.     

Cystic Lesions of the Pancreas

Opinion statement Pancreatic cystadenomas are a group of benign, premalignant, and malignant cystic tumors of the pancreas. Serous cystadenomas are benign lesions that often do not require surgical excision unless they are complicated by bleeding, obstruction, or pain. Mucinous cystadenomas are premalignant lesions that may be surgically excised if there is a concern regarding malignant degeneration. However, it may be difficult to predict the timing and risk of malignant change. Also, it is controversial whether all mucinous cystadenomas should be resected. Cystadenocarcinomas should be surgically managed if they are resectable, that is, there is no evidence of metastatic disease. Intraductal papillary mucinous tumors share many features with mucinous cystadenomas. However, intraductal papillary mucinous tumors arise from the pancreatic duct and are often associated with a dilated pancreatic duct. These lesions are often managed with surgical resection, including total pancreatectomy for diffuse lesions with evidence of localized malignancy.