[A case of locally recurrent breast cancer difficult to differentiate from nodular fasciitis].

Breast-conserving surgery was performed on a 78-year-old woman for left breast cancer 5 years previously (invasive ductal carcinoma, T1cN2M0, stage IIIA, ER[+], PR[-], HER2[-]). Chemotherapy, radiotherapy, and hormonal therapy were administered. A left subclavian tumor was detected, and an excisional biopsy was performed. Histological examination showed spindle cells, different from primary breast cancer histology, and nodular fasciitis was diagnosed negative cytokeratin and vimentin immunostaining results. After 12 months, a mass had developed in the same region, and reoperation was performed for resection. Similar spindle cells were observed, but they tested positive for cytokeratin. Carcinoma was diagnosed and thought to be locally recurrent breast cancer. Despite postoperative chemotherapy, the patient experienced bone and lung metastasis and a third local recurrence. She died 13 months following the last surgery. Recurrent breast cancer sometimes displays different histology from the initial cancer, and mimics stromal tumors in certain cases.

Differentiation of autoimmune pancreatitis from pancreatic cancer by diffusion-weighted MRI.

OBJECTIVES: We sought to clarify the clinical utility of diffusion-weighted magnetic resonance imaging (DWI) for differentiating autoimmune pancreatitis (AIP) from pancreatic cancer. METHODS: Thirteen AIP patients underwent DWI before therapy, and six of them underwent DWI after steroid therapy. The extent and shape of high-intensity areas were compared with those of 40 pancreatic cancer patients. Apparent diffusion coefficient (ADC) values were calculated in the AIP area before and after steroid therapy in pancreatic cancer patients and in a normal pancreatic body. RESULTS: On DWI, AIP and pancreatic cancer were detected as high-signal intensity areas. The high-intensity areas were diffuse (n=4), solitary (n=6), and multiple (n=3) in AIP patients, but all pancreatic cancer patients showed solitary areas (P<0.001). A nodular shape was significantly more frequent in pancreatic cancer, and a longitudinal shape was more frequently found in AIP (P=0.005). ADC values were significantly lower in AIP (1.012+/-0.112 x 10(-3) mm(2)/s) than in pancreatic cancer (1.249+/-0.113 x 10(-3) mm(2)/s) and normal pancreas (1.491+/-0.162 x 10(-3) mm(2)/s) (P<0.001). Receiver operating characteristic analysis yielded an optimal ADC cutoff value of 1.075 x 10(-3) mm(2)/s to distinguish AIP from pancreatic cancer. After steroid therapy, high-intensity areas on DWI disappeared or were markedly decreased, and the ADC values of the reduced pancreatic lesions increased almost to the values of normal pancreas. CONCLUSIONS: DWI is useful for detecting AIP and for evaluating the effect of steroid therapy. ADC values were significantly lower in AIP than in pancreatic cancer. An ADC cutoff value may be useful for distinguishing AIP from pancreatic cancer.

Pancreaticobiliary maljunction.

Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a junction of the pancreatic and bile ducts located outside the duodenal wall, usually forming a markedly long common channel. In PBM patients, this anomaly allows regurgitation between the pancreatobiliary and biliopancreatic tract. Since hydrostatic pressure within the pancreatic duct is usually higher than that in the common bile duct, pancreatic juice frequently refluxes into the bile duct. As a result, pancreatic enzyme levels are generally very high in the bile and there is a related high incidence of biliary cancer. PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst [CCC]) and PBM without biliary dilatation (maximal diameter of the bile duct

Biliary lesions associated with autoimmune pancreatitis.

BACKGROUND/AIMS: Characteristic radiological features of biliary lesions in patients with autoimmune pancreatitis (AIP) have not yet been identified. METHODOLOGY: Bile duct lesions and their relationships to other clinical findings were assessed in 43 AIP patients. RESULTS: Of the 43 AIP patients, 34 (79%) had bile duct stenosis. In all the 34 patients, the lower bile duct was involved; in 21 of these, only the lower bile duct was involved, and in 13 patients, there was widespread wall thickening of the middle and upper bile duct where stenosis was not obvious on cholangiography. Furthermore, 4 patients with extensive bile duct involvement also had stenosis of the intrahepatic bile duct. All patients with bile duct involvement had involvement of the head portion of the main pancreatic duct. None of the 6 patients with involvement of only the body and/or tail portion of the main pancreatic duct had bile duct involvement. Gallbladder wall thickening was more frequently noted in patients with extensive bile duct involvement (p < 0.01). Serum IgG4 levels were significantly more elevated in patients with extensive bile duct involvement (p < 0.05). CONCLUSIONS: AIP patients with extensive bile duct involvement characterized by widespread wall thickening of the bile duct may have more active disease.

Frequent and significant K-ras mutation in the pancreas, the bile duct, and the gallbladder in autoimmune pancreatitis.

OBJECTIVES: To assess the relationship between autoimmune pancreatitis (AIP) and pancreatic cancer, we analyzed K-ras mutation in the pancreatobiliary tissues of patients with AIP. METHODS: An analysis of K-ras mutation and an immunohistochemical study were performed on the pancreas of 8 patients with AIP and 10 patients with chronic alcoholic pancreatitis and on the common bile duct and the gallbladder of 9 patients with AIP. K-ras mutation was analyzed in the pure pancreatic juice from 3 patients with AIP. RESULTS: High-frequency K-ras mutation (2+ or 3+) was detected in the pancreas of all the 8 patients and in the pancreatic juice of the other 2 patients. The mutation in codon 12 of the ras gene was GAT in all the 10 patients. High-frequency K-ras mutation was detected in the common bile duct of 5 patients with AIP and in the gallbladder epithelium of 4 patients with AIP. The K-ras mutation was detected in the fibroinflammatory pancreas, the bile duct, and the gallbladder, with abundant infiltrating IgG4-positive plasma and Foxp3-positive cells of patients with AIP with elevated serum IgG4 levels. CONCLUSIONS: Significant K-ras mutation occurs most frequently in the pancreatobiliary regions of patients with AIP. Autoimmune pancreatitis may be a risk factor of pancreatobiliary cancer.

Serum IgG4 concentrations and IgG4-related sclerosing disease.

BACKGROUND: Based on histological and immunohistochemical examinations of various organs of patients with autoimmune pancreatitis (AIP), a new clinicopathological entity, IgG4-related systemic disease, was proposed. This study aimed to clarify clinical utility of serum IgG4 levels in differentiating AIP from other pancreatobiliary diseases, clinical utility of serum IgG4 levels in differentiating Mikulicz's disease from other salivary gland disorders, as well as in identifying other IgG4-related diseases. METHODS: Serum IgG4 levels were measured in 468 patients. RESULTS: The median serum IgG4 level was significantly greater in AIP (301.5mg/dl) than in other pancreatobiliary diseases (p<0.01). Using the cutoff value of 119 mg/dl that was determined on the basis of this study's ROC curve data, the sensitivity and specificity to distinguish AIP from pancreatic cancer were 82.1% and 94.8%, respectively. The median serum IgG4 level was significantly greater in Mikulicz's disease (357.0mg/dl) than in other salivary gland diseases (p<0.01). Of 75 patients with elevated serum IgG4 levels, 15 had diseases other than pancreatobiliary and salivary gland diseases. CONCLUSIONS: Serum IgG4 levels were useful for diagnosing AIP and Mikulicz's disease. Some diseases with serum IgG4 level elevations may be lesions of IgG4-related systemic disease without manifestations of AIP and Mikulicz's disease.

Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis.

AIM: To clarify the characteristic features of biliary lesions in patients with autoimmune pancreatitis (AIP) and compare them with those of primary sclerosing cholangitis (PSC). METHODS: The clinicopathological characteristics of 34 patients with sclerosing cholangitis (SC) associated with AIP were compared with those of 4 patients with PSC. RESULTS: SC with AIP occurred predominantly in elderly men. Obstructive jaundice was the most frequent initial symptom in SC with AIP. Only SC patients with AIP had elevated serum IgG4 levels, and sclerosing diseases were more frequent in these patients. SC patients with AIP responded well to steroid therapy. Segmental stenosis of the lower bile duct was observed only in SC patients with AIP, but a beaded and pruned-tree appearance was detected only in PSC patients. Dense infiltration of IgG4-positive plasma cells was detected in the bile duct wall and the periportal area, as well as in the pancreas, of SC patients with AIP. CONCLUSION: SC with AIP is distinctly different from PSC. The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.

Endocrine cells in ampullary carcinoma.

BACKGROUND/PURPOSE: As ampullary carcinoma originates from four anatomical regions, it may have different behaviors depending on its origin. We documented the presence of endocrine cells found in ampullary carcinoma, and we studied the clinicopathological implications of their presence. METHODS: We immunohistochemically examined the presence of an endocrine component in 62 surgically resected specimens of ampullary carcinoma, and we studied the clinicopathological differences between endocrine component-positive cases and endocrine component-negative cases. RESULTS: Endocrine cells were detected in 16 cases (26%); 11 cases had many endocrine cells, and five cases had scattered endocrine cells. Serotonin-positive cells were detected in all 16 cases, in which six cases had many positive cells. Several somatostatin-positive cells were detected in three cases. Endocrine cells were detected in ampulloduodenal polypoid lesions (two cases) and ampullopancreaticobiliary ducts (14 cases). The histology of 15 of the 16 endocrine component-positive ampullary carcinomas was the intestinal type. Pancreatic invasion and lymph node involvement were observed less frequently in endocrine component-positive cases (P < 0.01). There were no significant differences with respect to immunoreactivity for carbohydrate antigen (CA) 19.9, carcinembryonic antigen (CEA), and p53 overexpression, and K-ras mutations. CONCLUSIONS: Endocrine component-positive ampullary carcinoma seemed to be derived from the ampullopancreaticobiliary common duct or the ampulloduodenum, and to behave less aggressively than endocrine component-negative carcinoma.

Biliopancreatic reflux-pathophysiology and clinical implications.

The common bile duct and the main pancreatic duct open into the duodenum, where they frequently form a common channel. The sphincter of Oddi is located at the distal end of the pancreatic and bile ducts; it regulates the outflow of bile and pancreatic juice. In patients with a pancreaticobiliary maljunction, the action of the sphincter does not functionally affect the junction. Therefore, in these patients, two-way regurgitation (pancreatobiliary and biliopancreatic reflux) occurs. This results in various pathological conditions of the biliary tract and the pancreas. Biliopancreatic reflux could be confirmed by: operative or postoperative T-tube cholangiography; CT combined with drip infusion cholangiography; histological detection of gallbladder cancer cells in the main pancreatic duct; and reflux of bile on the cut surface of the pancreas. Biliopancreatic reflux occurs frequently in patients with a long common channel. Although the true prevalence, degree, and pathophysiology of biliopancreatic reflux remain unclear, biliopancreatic reflux is related to the occurrence of acute pancreatitis. Obstruction of a long common channel easily causes bile flow into the pancreas. Even if no obstruction is present, biliopancreatic reflux can still result in acute pancreatitis in some cases.

Diagnosis and clinical implications of pancreatobiliary reflux.

The sphincter of Oddi is located at the distal end of the pancreatic and bile ducts and regulates the outflow of bile and pancreatic juice. A common channel can be so long that the junction of the pancreatic and bile ducts is located outside of the duodenal wall, as occurs in pancreaticobiliary maljunction (PBM); in such cases, sphincter action does not functionally affect the junction. As the hydropressure within the pancreatic duct is usually greater than in the bile duct, pancreatic juice frequently refluxes into the biliary duct (pancreatobiliary reflux) in PBM, resulting in carcinogenetic conditions in the biliary tract. Pancreatobiliary reflux can be diagnosed from elevated amylase level in the bile, secretin-stimulated dynamic magnetic resonance cholangiopancreatography, and pancreatography via the minor duodenal papilla. Recently, it has become obvious that pancreatobiliary reflux can occur in individuals without PBM. Pancreatobiliary reflux might be related to biliary carcinogenesis even in some individuals without PBM. Since few systemic studies exist with respect to clinical relevance and implications of the pancreatobiliary reflux in individuals with normal pancreaticobiliary junction, further prospective clinical studies including appropriate management should be performed.

Serum IgG4 levels and extrapancreatic lesions in autoimmune pancreatitis.

OBJECTIVE: Serum IgG4 levels are frequently elevated in patients with autoimmune pancreatitis (AIP). AIP is sometimes associated with various extrapancreatic lesions. This study examined whether there is a correlation between serum IgG4 levels and associated extrapancreatic lesions in AIP patients. METHODS: Serum IgG4 levels were measured in 40 AIP patients before therapy. In these patients, four associated extrapancreatic lesions (sclerosing cholangitis, sclerosing cholecystitis, sclerosing sialadenitis, and retroperitoneal fibrosis) and clinical factors, such as age, sex ratio, enlargement of the pancreas, and initial symptoms, were retrospectively assessed. RESULTS: The mean serum IgG4 level of the 40 AIP patients was 411+/-448 mg/dl. On the basis of the receiver operator characteristic curve data, the optimal cutoff value for the serum IgG4 to distinguish between AIP patients with and without extrapancreatic lesions was 220 mg/dl; 18 (78%) of 23 patients whose serum IgG4 was more than or equal to 220 mg/dl had extrapancreatic lesions, whereas four (24%) of 17 patients whose serum IgG4 was less than 220 mg/dl had extrapancreatic lesions (P=0.0011). No significant differences between the two groups in age, sex, the frequency of pancreatic enlargement or obstructive jaundice, and associated sialadenitis, retroperitoneal fibrosis, and diabetes mellitus were identified. Sclerosing cholangitis and cholecystitis were more frequent in patients with serum IgG4 levels of more than or equal to 220 mg/dl than in those with a lower serum IgG4 level (P=0.0002 and 0.0204, respectively). The number of associated extrapancreatic lesions was significantly greater in patients with a high-serum IgG4 level. CONCLUSION: AIP patients with serum IgG4 levels of more than or equal to 220 mg/dl frequently have extrapancreatic lesions.

Strategy for differentiating autoimmune pancreatitis from pancreatic cancer.

OBJECTIVES: It is of utmost importance that autoimmune pancreatitis (AIP) be differentiated from pancreatic cancer (PC) because some AIP cases undergo unnecessary laparotomy or pancreatic resection on suspicion of PC. This study aimed to develop an appropriate strategy for differentiating between AIP and PC. METHODS: Clinical, serological, and radiological features of 17 AIP patients forming a masslike lesion on pancreas head and 70 patients with pancreatic head cancer were compared. RESULTS: Numerous findings can be used to distinguish between AIP and PC, and the following are more likely in AIP: fluctuating jaundice; elevated serum IgG4 levels; delayed enhancement of the enlarged pancreas and a capsule-like low-density rim on computed tomography; long or skipped narrowed portion with side branches of the main pancreatic duct without upstream dilatation on endoscopic retrograde pancreatography, extrapancreatic lesions, such as stenosis of the intrahepatic bile duct, salivary gland swelling, and retroperitoneal mass; and responsiveness to steroid therapy. CONCLUSIONS: In elderly male patients presenting with obstructive jaundice and a pancreatic mass, AIP should be considered in the differential diagnosis. Based on a combination of clinical, serological, and radiological findings, AIP can be differentiated from PC. An algorithm for management of patients with a masslike lesion on pancreas head is presented.

Carcinoma arising in congenital choledochal cysts.

BACKGROUND/AIMS: Congenital choledochal cyst is almost always associated with pancreaticobiliary maljunction and is sometimes associated with biliary carcinoma. This study aimed to investigate the clinicopathological features and surgical treatment of choledochal cyst associated with carcinoma arising in the cyst wall. METHODOLOGY: Relationship of the clinicopathological findings and surgical treatment of 8 patients with histologically proven carcinoma that had developed in the choledochal cyst wall were examined to determine mode of tumor spread, multicentric tumor origins, and coincidence with other neoplastic lesions. RESULTS: Papillary adenocarcinoma (n = 5) had different clinicopathological features than tubular adenocarcinoma (n = 3). Radiologically, papillary adenocarcinoma presented as an eccentrically located polypoid mass in the cyst, while with tubular adenocarcinoma, there was evidence of bile duct stenosis with irregular thickening of the bile duct wall. Papillary adenocarcinoma was associated with extensive superficial spread (n = 1), synchronous (n = 2), or metachronous (n = 2) multicentric tumors. Pancreatoduodenectomy with (n = 1) or without (n = 1) hepatic lobectomy, and repeated resection by hepatic lobectomy (n = 2) were performed for these circumstances of tumors. Extensive or repeated resections allowed 3 patients to live longer than 2 years. Tubular adenocarcinoma was associated with scirrhously infiltrative spread and a poor prognosis. CONCLUSIONS: Papillary adenocarcinoma frequently occurs in the choledochal cyst wall. Since aggressive resection offers survival benefits in papillary adenocarcinoma arising in the choledochal cyst wall, the presence of superficial spread and multicentric tumors should be identified and taken into account when planning surgery. Patients require close monitoring so that recurrent carcinoma of the remnant bile duct can be identified early.

A new diagnostic endoscopic tool for autoimmune pancreatitis.

BACKGROUND: Autoimmune pancreatitis (AIP) is a type of pancreatitis that is presumed to have an autoimmune etiology, and is currently diagnosed based on a combination of clinical, laboratory, and imaging studies. Although it is of utmost importance that AIP be differentiated from pancreatic cancer, AIP is sometimes difficult to differentiate from locally advanced pancreatic cancer. OBJECTIVE: To find a useful new method to diagnose AIP. DESIGN: Prospective study from June 2004 to September 2007. SETTING: Single public hospital. PATIENTS: Ten AIP patients, 10 patients with pancreatic cancer, and 10 patients with papillitis. INTERVENTIONS: Immunoglobulin (Ig)G4-immunostaining of biopsy specimens obtained from the major duodenal papilla. MAIN OUTCOME MEASUREMENTS: Number of immunohistochemically identified cells per high-power field (HPF) in each specimen were counted. RESULTS: Significant infiltration of IgG4-positive plasma cells (> or = 10/HPF) was observed in the major duodenal papilla of all 8 AIP patients with pancreatic head involvement. Moderate infiltration of IgG4-positive plasma cells (4-9/HPF) was detected in 1 patient with pancreatic head cancer, but there were rare (< or = 3/HPF) IgG4-positive plasma cells infiltrating the major duodenal papilla in 2 AIP patients who only had pancreatic body and/or tail involvement, 9 patients with pancreatic cancer, and 10 patients with papillitis. In the 3 AIP patients in whom biopsy specimens were retaken after steroid therapy, the number of IgG4-positive plasma cells decreased from significant to moderate in 2 patients and to < or = 3/HPF in 1 patient. LIMITATIONS: Small sample size. Endoscopists were not blinded to clinical information. CONCLUSIONS: IgG4 immunostaining of biopsy specimens obtained from the major duodenal papilla may be useful for supporting a diagnosis of AIP with pancreatic head involvement.

Sex-based differences in gallbladder cancer associated with pancreaticobiliary maljunction.

BACKGROUND/AIMS: Gallbladder cancer predominantly affects women; this sex-based difference is influenced by factors such as gallstones, sex hormone and genetic susceptibility. Gallbladder cancer is also frequently associated with pancreaticobiliary maljunction (PBM) without biliary dilatation. We examined sex-based differences in gallbladder cancer associated with PBM. METHODOLOGY: With a particular focus on gender differences, we retrospectively compared clinicopathological findings between 44 patients (9 men, 35 women) with PBM without biliary dilatation and 232 patients (60 men, 172 women) with gallbladder cancer that was not associated with PBM. RESULTS: Gallbladder cancer was detected in 75% of patients with PBM without biliary dilatation. Among PBM patients, gallbladder cancer was significantly more common in women than in men [29/35 (83%) us. 4/9 (44%), p<0.05]. Both men and women with gallbladder cancer associated with PBM were significantly younger at the time of diagnosis than patients with gallbladder cancer without PBM (p<0.01). Gallstones were detected in only 10% of women with gallbladder cancer with PBM, while gallstones were detected in 63% of women with gallbladder cancer without PBM (p<0.01). CONCLUSIONS: Gallbladder cancer occurs very frequently in patients with PBM without biliary dilatation, and women appear to be at a significantly higher risk than men.

Pancreas divisum in pancreaticobiliary maljunction.

BACKGROUND/AIMS: Pancreaticobiliary maljunction (PBM) and pancreas divisum (PD) are congenital anomalies that develop in the embryo at an early stage. They are possibly the result of bile and pancreatic duct misarrangement. We investigated the configuration of the pancreatic duct in patients with PBM and its clinical implications. METHODOLOGY: In 84 PBM patients, the configuration of the pancreatic duct and the presence of biliary cancer were documented. Patency of Santorini's duct was determined fluoroscopically or by dye-injection endoscopic retrograde pancreatography. Bile amylase levels were measured in 10 patients. RESULTS: Incomplete PD was detected in 8 (9.5%) of the 84 PBM patients. All of the 8 patients had a patent Santorini's duct, and only 1 patient had gallbladder cancer. The frequency of associated gallbladder cancer and the bile amylase level were significantly lower in PBM patients with a patent Santorini's duct than in PBM patients with a nonpatent Santorini's duct. CONCLUSIONS: PBM is sometimes associated with incomplete PD. In PBM patients with an incomplete PD, the incidence of cancer of the biliary tract may be lower, since pancreatic juice reflux into the bile duct might be reduced by the flow of pancreatic juice into the duodenum through Santorini's duct.

The incidence of pancreatic and extrapancreatic cancers in Japanese patients with chronic pancreatitis.

BACKGROUND/AIMS: Although an association between chronic pancreatitis and malignancies has been reported in the Western literature, in Japan there have been few reports that have dealt with this issue. We investigated the incidence of pancreatic and extrapancreatic cancers in Japanese patients with chronic pancreatitis. METHODOLOGY: We studied 170 Japanese patients with definite chronic pancreatitis with respect to the occurrence of pancreatic and extrapancreatic cancers during follow-up and compared the incidence with that reported in the Western literature. RESULTS: The patients developed 29 cancers including 5 pancreatic cancers. Four patients had two different types of cancer. The extrapancreatic cancer incidence (24/170: 14.1%) was significantly higher than in the West (8.3%, p < 0.01). The major organs in which cancer developed were stomach (n=9), pancreas (n=5), esophagus (n=4), colon (n=3), lung (n=2) and hemopoietic tissue (n=2). The overall incidence (8.2%) of associated cancers of the digestive system including, stomach, intestine, liver, biliary duct, and gallbladder, was significantly higher than in the West (1.3%, p < 0.01). CONCLUSIONS: The risk of extrapancreatic cancers during the course of chronic pancreatitis is significantly increased in Japan than in Western countries. In particular, cancers of the digestive system are frequently associated with chronic pancreatitis in Japan.

Pancreatographic investigation of embryology of complete and incomplete pancreas divisum.

OBJECTIVES: To investigate embryology of complete and incomplete pancreas divisum (PD). METHODS: We analyzed anatomy of the pancreatic duct system in 44 cases of complete PD and 41 cases of incomplete PD. Under the concept of branch fusion between the ventral and dorsal pancreatic ducts, incomplete PD was divided into 3 types: fusion of the extreme end of the upper branch of the ventral pancreatic duct with the dorsal pancreatic duct (type 1); fusion of the lower branch of the dorsal pancreatic duct with the lower branch of the ventral pancreatic duct (type 2); and fusion of the lower branch of the dorsal pancreatic duct with the ventral pancreatic duct (type 3). RESULTS: Complete PD showing no ventral pancreatic duct radiologically might be induced by complete regression of the ventral pancreatic duct near the orifice in the pancreas with communication between the ventral and dorsal pancreatic ducts. Embryological mechanism of incomplete PD (type 1, n = 16; type 2, n = 10; and type 3, n = 15) was confirmed by measurement in pancreatograms. CONCLUSIONS: Some cases of complete PD might be induced by complete regression of the ventral pancreatic duct near the orifice. Embryology of incomplete PD can be accounted by concept of branch fusion.

Acute pancreatitis and a long common channel.

BACKGROUND: As sphincter action does not functionally affect the union in pancreaticobiliary maljunction (PBM), two-way regurgitation occurs. We investigated the features of acute pancreatitis associated with patients displaying a long common channel. METHODS: We reviewed 3210 endoscopic retrograde cholangiopancreatograms. PBM was diagnosed in 107 patients with a long common channel in which communication between the pancreatic and bile duct was maintained even during sphincter contraction. High confluence of pancreaticobiliary ducts (HCPBD) was diagnosed in 60 patients with a common channel >or=6 mm long in which communication between the two ducts was occluded with sphincter contraction. RESULTS: Of patients with PBM, four had acute pancreatitis. Etiology of acute pancreatitis in the two patients was suspected to involve impaction in a long common channel. Of patients with HCPBD, 17 displayed acute pancreatitis. Three patients had a history of excessive alcohol intake and 10 patients were associated with choledocholithiasis or cholecystitis. The remaining four patients had no history of them. CONCLUSIONS: Acute pancreatitis occurs in 13% of patients with a long common channel. Obstruction of a long common channel by stones easily induces bile flow into the pancreas. Even if no obstruction is present, biliopancreatic reflux induces acute pancreatitis in some patients.

Biliary carcinoma risk in patients with pancreaticobiliary maljunction and the degree of extrahepatic bile duct dilatation.

BACKGROUND/AIMS: Pancreaticobiliary maljunction (PBM) carries a high risk of biliary carcinoma. This study aimed to examine the biliary complications of patients with PBM in relation to the degree of extra-hepatic bile duct dilatation. METHODOLOGY: Ninety-eight cases of PBM could be divided into 5 groups according to the maximum diameter of the extrahepatic bile duct: < or = 10mm, 11-15mm, 16-20mm, 21-30mm, > or = 31mm. The clinicopathological findings of biliary carcinomas associated with PBM were compared with 232 cases of gallbladder carcinoma and 159 cases of bile duct carcinoma that were not associated with PBM. RESULTS: Gallbladder carcinoma occurred in 36 of 65 patients (55%) with PBM whose maximum diameter of the extrahepatic bile duct was < or = 30mm, but no gallbladder carcinoma occurred in patients with PBM whose diameter was > or = 31mm. Bile duct carcinoma occurred in 6 of 52 patients (12%) with PBM whose diameter was > or = 21mm, but no bile duct carcinoma occurred in patients with PBM whose diameter was < or = 20mm. The age at diagnosis of the patients with gallbladder or bile duct carcinoma associated with PBM was significantly younger than those without PBM (p<0.01). CONCLUSIONS: PBM with an extrahepatic bile duct diameter < or = 30mm is associated with a high risk of gallbladder carcinoma. PBM with an extrahepatic bile duct diameter > or = 21mm is associated with a high risk of bile duct carcinoma. Prophylactic cholecystectomy is recommended for patients with PBM without biliary dilatation.