Biliary Tract System Research Articles

The PLGF/c-MYC/miR-19a axis promotes metastasis and stemness in gallbladder cancer.

Gallbladder cancer (GBC) is the most common malignant tumor of the biliary tract system. Epithelial–mesenchymal transition (EMT) plays a vital role in the process of tumor metastasis. Mesenchymal‐like cells can serve as a source of cancer stem cells, which can confer the EMT phenotype. Placental growth factor (PLGF) belongs to the vascular endothelial growth factor family and plays a vital role in cancer. However, the underlying molecular mechanisms about the influence of PLGF on EMT in GBC remain unknown. Here we show that PLGF expression levels were higher in GBC tissues than in normal adjacent tissues and were associated with poor prognosis in GBC patients. Exogenous PLGF enhanced the migration, invasion, and tumorsphere formation of GBC cells. Conversely, knockdown of PLGF decreased the aggressive phenotype of GBC cells. Mechanistically, exogenous PLGF upregulated microRNA‐19a (miR‐19a) expression through the activation of c‐MYC. Moreover, Spearman's correlation analysis showed a positive pairwise correlation among PLGF, c‐MYC, and miR‐19a expression in GBC tissues. Taken together, these results suggest that PLGF promotes EMT and tumorsphere formation through inducing miR‐19a expression by upregulating c‐MYC. Thus, PLGF could be a promising molecular therapeutic target for GBC.

Serum extracellular vesicles—a promising path towards an individualized medicine in primary sclerosing cholangitis and related cholangiocarcinoma

Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the biliary tract system, whose mechanisms of pathogenesis remain elusive. Most importantly, PSC is considered a major medical burden due to its premalignant nature (1). Malignant complications in PSC frequently occur within the hepatobiliary system, arising predominately as cholangiocarcinoma (CCC) (2).

A case of double common bile duct in a deceased donor for transplantation

A double common bile duct is extremely rare among the anatomical variations in the biliary tract system. We report an incidentally encountered case of the double common bile duct and discuss the novel anatomical findings of the accessory common bile duct from the viewpoint of embryology. A unique point of our case is that the accessory common bile duct bifurcated at the level of the intrapancreatic bile duct. There is no similar case in the previous literature among type II double common bile duct in the viewpoint of anatomical findings of the accessory common bile duct. We assume that this asymptomatic anatomical variation may be present more commonly, but not diagnosed.

Adiposity and cancer at major anatomical sites: umbrella review of the literature

Objective To evaluate the strength and validity of the evidence for the association between adiposity and risk of developing or dying from cancer.Design Umbrella review of systematic reviews and meta-analyses.Data...

Features and diagnosis of computed tomography and magnetic resonance imaging on autoimmune pancreatitis

Objective To summarize the features of computed tomography (CT) and magnetic resonance imaging (MRI) of autoimmune pancreatitis (AIP) and investigate the key points of diagnosis and identification. Methods The retrospective and descriptive study was conducted. The clinical data of 21 patients with AIP who were admitted to the Affiliated Hospital of Inner Mongolia Medical University between February 2012 and February 2015 were collected. All the patients underwent plain and enhanced scans of CT and MRI, and magnetic resonanced cholangio-pancreatography (MRCP), and then received hormone therapy. Eleven patients with pancreatic cancer and 11 normal subjects who were diagnosed by MRI in the same period were selected, and apparent diffusion coefficient (ADC) was calculated and compared. Observation indicators: (1) situation of imaging examination: ① pancreatic manifestations: density, signal, atrophy, calcification and enlargement of pancreas, change of pancreatic duct, ② manifestations out of pancreas: changes of biliary tract system and kidney, ③ diffusion weighted imaging (DWI) and ADC: comparisons of ADC among AIP, pancreatic cancer and normal pancreas; (2) diagnosis; (3) treatment and follow-up. The follow-up using outpatient examination and telephone interview was performed to detect the clinical symptoms and signs up to February 2016. Measurement data with normal distribution were represented as ±s. Comparisons among groups were done using one-way ANOVA. Pairwise comparison was analyzed by Dunnett′ T3 test. Results (1) Situation of imaging examination: Of 21 patients, 17 received scan of CT and 11 received scan of MRI (7 combined with scan of CT). ① Pancreatic manifestations: 14 patients had diffuse enlargement of pancreas, with full edge and sausage-like change. Plain scan of CT showed uniform isodense shadow, and enhanced scan showed that reduced enhancement in arterial phase and gradually homogenous enhancement in portal vein phase and lag phase with no enhancement in edge of pancreas. Plain scan of MRI showed lesions were manifested as slight hypointensity on T1 weighted imaging (T1WI), slight hyperintensity on T2WI and hyperintensity on DWI. Enhanced scan of MRI showed delayed enhancement, edge of lesions was manifested as slight hypointensity on T1WI and T2WI, without enhancement. Atrophy and calcification of pancreas: 3 patients had atrophy of pancreatic parenchyma in which scattered calcification were seen. Enlargement of pancreas: 4 patients had localized enlargement of pancreas showing false tumor-like change, including 2 with localized enlargement in head of pancreas. Change of pancreatic duct: MRCP showed that diffuse stenosis, local stenosis and local dilatation of pancreatic ducts were respectively detected in 4, 3 and 1 patients. ② Manifestations out of pancreas: 11 patients had changes of biliary tract system, showing intrahepatic bile duct and common bile duct dilation, partial stenosis and extensive bile duct wall thickening. Enhanced scan of MRI showed there was obvious enhancement of bile duct wall. MRCP of 4 patients showed that the beak-like stenosis was seen in the distal common bile duct. Three patients had kidney changes, enhanced scan of CT showed that kidney demonstrated patch-shape hypodense shadow in arterial phase and homogenous enhancement of patch-shape hypodense shadow in lag phase, and plain scan of MRI showed that kidney lesions demonstrated equal signal on T1WI fat suppression (FS) and patch-shape low signal on T2WI FS. Lesions had gradually homogenous enhancement in substance phase and lag phase. ③ DWI and ADC: lesions in patients with AIP and pancreatic cancer demonstrated high signal on DWI (b=1 000 s/mm2) compared with adjacent tissues (no involvement in pancreas or normal pancreatic parenchyma), ADC of pancreas in patients with AIP, with pancreatic cancer and with normal population was (0.001 30±0.000 35)mm2/s, (0.000 80±0.000 14)mm2/s and (0.001 60±0.000 24)mm2/s, respectively, with a statistically significant difference ( F=30.409, P 0.05). (2) Diagnosis: 11 patients were diagnosed by CT examination, with a diagnostic accuracy of 11/17. Eight patients were diagnosed by MRI examination, with a diagnostic accuracy of 8/11. One patient was misdiagnosed as cancer of pancreatic head by CT and MRI examinations, and 1 was misdiagnosed as cancer in the distal common bile duct. (3) Treatment and follow-up: 21 patients underwent regular hormone therapy, and 40 mg prednisolone was given orally a daily for 3-4 weeks and then gradually reduced to 5 mg up to complete relief of the symptoms. All the 21 patients were followed up for 12-45 months. Of 17 patients with abdominal pain and distension, symptoms of 7 patients disappeared and symptoms of 10 patients decreased or occasionally occurred. Of 10 patients associated with jaundice, symptoms of 7 and 2 patients disappeared and decreased, respectively, and symptoms of 1 patient subsided. Conclusion CT and MRI examinations of pancreas demonstrate sausage-like and false tumor -like changes, the non-neoplastic bile and pancreatic duct stenosis combined with IgG4 related diseases in other organs is an important imaging evidence for diagnosis and differential diagnosis of AIP. Key words: Autoimmune pancreatitis; Tomograghy, X-ray computed; Magnetic resonance imaging; Diagnosis

Cancer incidence in urban Shanghai, 1973-2010: an updated trend and age-period-cohort effects.

BackgroundTo provide a comprehensive overview of temporal trends in cancer incidence during 1973–2010 in urban Shanghai.MethodsThe estimated annual percent changes (EAPCs) for the whole period and for the time segments in age-standardized incidence rates (ASR) were evaluated with Joinpoint analysis. Age-period-cohort (APC) models were modeled to examine the effects of age, period and birth cohort on cancer incidence.ResultsThe overall ASR decreased slightly and significantly in males (EAPC of −0.41) but increased significantly in females (EAPC of 0.57) during 1973–2010 in urban Shanghai. The incidence trend was not linear and varied by time segments. During the most recent 10 years (2001–2010), the ASR in males decreased by 1.65 % per year and stabilized in females. Incidence rates continued to decline during 1973–2010 for esophagus, stomach, and liver cancer in both sexes, as well as male lung cancer and cervix cancer. It should be noted that it was the first time to document a significant decline in lung cancer incidence among males during 1973–2010 with EAPC of −0.58 %, and a notable upward for cervix cancer since 1996 with EAPC of 8.94 %. Unfavorable trends in incidence were observed for the most common cancer sites in the 38 years period: colorectum, gallbladder & biliary tract, pancreas, kidney, bladder, brain & central nervous system (CNS), thyroid, non-Hodgkin’s lymphoma (NHL), prostate, female breast, corpus uteri, and ovary. APC analysis showed age, period and birth cohort yielded different effects by cancer sites.ConclusionsThe observed trends primarily reflect dramatic changes in socioeconomic development and lifestyles in urban Shanghai over the past four decades.

Innervation of Extrahepatic Biliary Tract, With Special Reference to the Direct Bidirectional Neural Connections of the Gall Bladder, Sphincter of Oddi and Duodenum in Suncus murinus, in Whole-Mount Immunohistochemical Study.

Sphincter of Oddi dysfunction is one of the most important symptoms in post-cholecystectomy syndrome. Using either electrical or mechanical stimulation and retrogradely transported neuronal dyes, it has been demonstrated that there are direct neural pathways connecting gall bladder and the sphincter of Oddi in the Australian opossum and the golden hamster. In the present study, we employed whole-mount immunohistochemistry staining to observe and verify that there are two different plexuses of the extrahepatic biliary tract in Suncus murinus. One, named Pathway One, showed a fine, irregular but dense network plexus that ran adhesively and resided on/in the extrahepatic biliary tract wall, and the plexus extended into the intrahepatic area. On the other hand, named Pathway Two, exhibiting simple, thicker and straight neural bundles, ran parallel to the surface of the extrahepatic biliary tract and passed between the gall bladder and duodenum, but did not give off any branches to the liver. Pathway Two was considered to involve direct bidirectional neural connections between the duodenum and the biliary tract system. For the first time, morphologically, we demonstrated direct neural connections between gall bladder and duodenum in S.murinus. Malfunction of the sphincter of Oddi may be caused by injury of the direct neural pathways between gall bladder and duodenum by cholecystectomy. From the viewpoint of preserving the function of the major duodenal papilla and common bile duct, we emphasize the importance of avoiding kocherization of the common bile duct so as to preserve the direct neural connections between gall bladder and sphincter of Oddi.

Bronchobiliary fistula from foreign body reaction or cholelithiasis

Bronchobiliary fistula (BBF) is an abnormality between the bronchial tree and biliary tract system. Misdiagnosis and delayed treatment might have a high mortality rate. Although some characteristics relevant to BBF have been introduced, difficulties and controversies in diagnosis and treatment need further investigation. In the present study, we summarize the diagnostic and surgical treatment experience of a 65-year-old male patient who presented with an irritating cough and biliptysis. As he had a history of aortic valve replacement surgery and schistosomial cirrhosis, the provisional diagnosis was BBF, which might be secondary to a foreign body reaction or liver cirrhosis and chronic cholecystitis and cholelithiasis. During the surgery, it was discovered that the right lobe of the liver was significantly atrophied with irregular contours, and the gallbladder was adjacent to the diaphragm with a fistula between the fundus of the gallbladder and the right semidiaphragm. These results confirmed that the BBF in this patient resulted from cholelithiasis associated with serious schistosomial cirrhosis. He was successfully treated with a right hepatic lobe resection and Roux-en-Y hepaticojejunostomy. The diaphragm defect was repaired by imbrication of the repaired liver and lower lobe's edges. A high index of clinical suspicion can be generated by patients who present with recurring irritating cough and production of bile-like sputum. The manifestations and responses to conservative treatment are crucial in clinical judgment, and surgical intervention is the primary treatment choice when other approaches have failed or there are complications from the underlying disease.

Second Primary Cancers Following Colorectal Cancer

See Article on Page 18-22 Compared with the general population, individuals with a previous colorectal cancer have an elevated risk of a second primary cancer [1, 2]. Possible reasons for this phenomenon could be (1) an overall greater predisposition to cancer due to the same environmental and genetic factors that contributed to the first primary cancer [3] and (2) the second cancers possibly being related to the effects of treatment for the first cancer [4]. Lynch syndrome is an autosomal, dominant, inherited disorder of cancer susceptibility caused by germline mutations in DNA mismatch repair (MMR) genes. Mutation carriers are prone to developing cancers of the colon, rectum, endometrium, ovary, stomach, small intestine, urinary tract, kidney, biliary tract, and nervous system, and the diagnosis of these cancers generally occurs at a younger age than it does for non-MMR-related cancers [5]. Second cancers following colorectal cancer have essentially two types of patterns: Lynch-syndrome-associated cancers and cancers with no features of Lynch syndrome, which include breast, pancreas, prostate, and thyroid cancers [6-8]. An increased surveillance strategy could lead to earlier identification and removal of both premalignant lesions and early-stage tumors. Any patients diagnosed with colorectal cancer at a young age are at high risk of having a second colorectal cancer and should be followed-up at regular intervals [6]. Furthermore, in surveillance of these patients, all Lynch-syndrome-related sites should be considered. However, given the limited evidence for the efficacy of screening for breast, pancreas, prostate, and thyroid cancers [8, 9], further studies are needed to verify the validity of screening for these cancers in Lynch syndrome.

Primary sclerosing cholangitis is associated with autoreactive IgA antibodies against biliary epithelial cells

Objective. Primary sclerosing cholangitis (PSC) is a chronic liver disease characterized by progressive destruction of the biliary tract system. Autoimmune reactions have been suggested to play a role in the etiology and pathogenesis of PSC, and a large number of different autoantibodies have been reported in PSC patients. However, the role of IgA, being the dominant immunoglobulin in bile and transported there via biliary epithelial cells, is still incompletely understood in PSC. The aim of this study was to evaluate the presence of autoreactive IgA in PSC patients. Material and methods. Cultures of biliary epithelial cells from healthy human liver tissue were established. Serum was collected from a total of 81 PSC patients and 42 healthy controls and tested for reactivity against biliary epithelial cells using flow cytometry. Patient characteristics were correlated with experimental findings. Results. The results showed that a majority of investigated PSC patients had autoreactive IgA against biliary epithelial cells, whereas these antibodies were almost absent in healthy individuals. Presence of autoreactive IgA in the PSC patients was not associated to gender, age, duration of disease, concomitant inflammatory bowel disease, or cholangiocarcinoma. Instead, the levels of autoreactive IgA correlated with higher total serum IgA levels and autoreactive IgA-positive patients progressed faster to a clinical endpoint (death or liver transplantation) compared to autoreactive IgA-negative patients. Conclusions.The findings provide new insights into the role of IgA in PSC patients and opens up for future studies addressing pathogenic mechanisms of autoantibodies directed against biliary epithelial cells.

Potential Therapeutic Targets for the Primary Gallbladder Carcinoma: Estrogen Receptors

Gallbladder carcinoma, the most frequent malignant neoplasm of the biliary tract system, has always been considered to feature late clinical presentation and diagnosis, limited treatment options and an extremely poor prognosis. In recent years, while the incidence of gallbladder cancer has appeared to be on the increase, the available treatment methods have not greatly improved survival of the affected patients. Thus, exploring new therapeutic targets for this devastating disease is an urgent matter at present. Epidemical studies have demonstrated that the incidence of gallbladder carcinoma exhibits a distinct gender bias, affecting females two to three times more than males, pointing to crucial roles of estrogen. It is well known that estrogen acts on target tissues by binding to estrogen receptors (ERs), which are mainly divided into three subtypes, ERα, ERβ and ERγ. ERα and ERβ appear to have overlapping but also unique even opposite biological effects. As important pathogenic mediators, ERs have been considered to relate to several kinds of tumors. In gallbladder carcinoma tissue, ERs have been shown to be positively expressed, and ERs expression levels are associated with differentiation and prognosis of this cancer. Nevertheless, the exact mechanisms of estrogen inducing growth of gallbladder carcinoma remain poorly understood. On the base of the current investigations, we deduce that estrogen participates in promotion of gallbladder carcinoma by influencing the formation of gallstones, stimulating angiogenesis, and promoting abnormal proliferation. Since ERs mediate the carcinogenic actions of estrogen in gallbladder, and therapy targeting ERs may provide new directions for gallbladder carcinoma. Therefore, it should be stressed that ERs are potential therapeutic targets for gallbladder carcinoma.

Quantitative analysis of the breath-holding half-Fourier acquisition single-shot turbo spin-echo technique in abdominal MRI

A consecutive series of 50 patients (28 males and 22 females) who underwent hepatic magnetic resonance imaging (MRI) from August to December 2011 were enrolled in this study. The appropriate parameters for abdominal MRI scans were determined by comparing the images (TE = 90 and 128 msec) produced using the half-Fourier acquisition single-shot turbo spin-echo (HASTE) technique at different signal acquisition times. The patients consisted of 15 normal patients, 25 patients with a hepatoma and 10 patients with a hemangioma. The TE in a single patient was set to either 90 msec or 128 msec. This was followed by measurements using the four normal rendering methods of the biliary tract system and the background signal intensity using the maximal signal intensity techniques in the liver, spleen, pancreas, gallbladder, fat, muscles and hemangioma. The signal-to-noise and the contrast-to-noise ratios were obtained. The image quality was assessed subjectively, and the results were compared. The signal-to-noise and the contrast-to-noise ratios were significantly higher at TE = 128 msec than at TE = 90 when diseases of the liver, spleen, pancreas, gallbladder, and fat and muscles, hepatocellular carcinomas and hemangiomas, and rendering the hepatobiliary tract system based on the maximum signal intensity technique were involved (p < 0.05). In addition, the presence of artifacts, the image clarity and the overall image quality were excellent at TE = 128 msec (p < 0.05). In abdominal MRI, the breath-hold half-Fourier acquisition single-shot turbo spin-echo (HASTE) was found to be effective in illustrating the abdominal organs for TE = 128 msec. Overall, the image quality at TE = 128 msec was better than that at TE = 90 msec due to the improved signal-to-noise (SNR) and contrast-to-noise (CNR) ratios. Overall, the HASTE technique for abdominal MRI based on a high-magnetic field (3.0 T) at a TE of 128 msec can provide useful data.

Liver involvement in melamine-associated nephrolithiasis.

It is currently believed that melamine ingestion can lead to insoluble crystals in an animal's urinary system with subsequent physical obstruction or bladder carcinoma. However, whether melamine can cause injury of other tissues and organs in humans is yet unknown. In this study, we encountered 3 affected children with liver lesions, 2 males and 1 female, and detailed their clinical characterizations. Their ages were respectively 2, 6, and 10 months. Among the 3 patients with liver lesions, only 1 exhibited symptoms of gradual progressive jaundice, abdominal distention, hepatic intumesce, and bilirubin abnormality; the other 2 were asymptomatic. The mechanism associated with liver lesion may, at least in part, be due to physical deposition and blockage of the biliary tract system. Disturbance of the acid-base equilibrium may be another reason that accelerates stone formation in human tissues.

Onset of Apoptosis in the Cystic Duct During Metamorphosis of a Japanese Lamprey, Lethenteron reissneri

A nonparasitic lamprey in Japan, Lethenteron reissneri, stops feeding prior to the commencement of metamorphosis. Resumption of feeding cannot take place due to major alterations in the digestive system, including loss of the gall bladder (GB) and biliary tree in the liver. This degeneration of bile ducts is considered to depend on programmed cell death or apoptosis, but molecular evidence of apoptosis remains lacking. Using terminal deoxynucleotidyl transferase dUTP nick-end labeling (TUNEL) staining and immunohistochemistry with an antibody against active caspase-3, we showed that epithelial cells of the cystic duct (CD) and GB became TUNEL-positive by the early metamorphosing stage. Immunohistochemical staining of active caspase-3, a key mediator in the apoptotic cascade, showed that the apoptotic signal was initiated in the region around the CD in the late larval phase. In later stages, active caspase-3-positive epithelial cells were also observed in the large intrahepatic bile duct (IHBD) and peripheral small IHBDs. At the early metamorphosing stage, bile canaliculi between hepatocytes were dilated and displayed features resembling canaliculi in cholestasis. Onset of apoptosis around the CD, which is the pathway for the storage of bile juice, and progression of apoptosis towards the large IHBD, which is the pathway for the secretion of bile juice, may lead to temporary intrahepatic cholestasis. The present study represents the first precise spatial and temporal analysis of apoptosis in epithelial cells of the biliary tract system during metamorphosis of any lamprey species.

Metachronous cancer of gallbladder and pancreas with pancreatobiliary maljunction

Pancreaticobiliary maljunction is a congenital anomaly in which the junction between the pancreatic duct and the common bile duct is located outside the sphincter of Oddi. It is well known that pancreaticobiliary maljunction is frequently associated with carcinoma of thebiliary tract. We report a case of metachronous cancer of the gallbladder and pancreas associated with pancreaticobiliary maljunction and cystic dilatation of common bile duct in a 68-year-old Tunisian woman who underwent a cholecystectomy for acute cholecystitis. The pancreatic tumor was an adenosquamous carcinoma. Pancreaticobiliary maljunction allows for pancreatobiliary or biliopancreatic reflux which may induce biliary tract carcinoma. Few cases of multifocal cancer associated with this anomaly have been reported. The association with pancreatic carcinoma remains rare. Close attention should be given to both the biliary tract system and pancreas during the long-term follow-up of patients with pancreaticobiliary maljunction, especially after they have undergone a choledochojejunostomy.

A Case of Peutz-Jeghers Syndrome with Intraductal Papillary Mucinous Carcinoma of Pancreas

Peutz-Jeghers syndrome (PJS), which is characterized by multiple hamartomatous polyps of the gastrointestinal tract and mucocutaneous pigmentation, is a rare autosomal dominant disease. This syndrome is often represented as a surgical emergency with complications of the polyps such as intussusception, small bowel obstruction, bleeding, and volvulus. In particular, many studies have reported that patients with this syndrome have a high risk of gastrointestinal or extragastrointestinal malignancy including gastric, duodenal, jejunal, ileal, and colonic carcinoma as well as malignancies involving other organs such as the gallbladder, biliary tract, pancreas, tonsils, breast, and reproductive system. However, there are few reported cases of an association of this syndrome with extraintestinal malignancy. In addition to that, there is no reported case of this syndrome with malignant tumor or intraductal papillary mucinous tumor of pancreas in Korea. We experienced a case of PJS accompanying intraductal papillary mucinous carcinoma of the pancreas, therefore we report this case with literatures reviewed.

Full‐term pregnancies after multiple hepatobiliary operations for choledochal cyst: A case report

Choledochal cysts are rare congenital cystic dilatations of the biliary tree. Choledochal cysts in pregnancy are a rare entity and represent a diagnostic and therapeutic challenge. The authors present a case of two full-term pregnancies in a woman who underwent multiple hepatobiliary operations for a choledochal cyst. The patient had two major problems: a type IV(a) choledochal cyst with atrophy of the left side of the liver and a congenitally dilated pancreatic duct in the head of the gland. She underwent a drainage surgical procedure using a Roux loop of jejunum and a revision surgery in which complete excision of the cyst and hepaticojejunostomy was performed. The surgical management was completed by a left lateral hepatic segmentectomy. During her first pregnancy, she experienced an episode of cholangitis, most probably due to the remaining congenitally dilated pancreatic duct. In succeeding years, the patient presented with recurrent pancreatitis and finally she underwent a Whipple operation. After that, the patient had a second pregnancy without any complications from the biliary tract system. Although choledochal cysts rarely occur in pregnancy, clinicians should be aware of this condition, as delayed or inappropriate therapy may be catastrophic for both the mother and the fetus.

A rare case of periampullary carcinoma with ectopic ending of Vater’s ampulla

A 71-year-old woman was referred to our department complaining of painless progressive jaundice for the last 3 mo. Magnetic resonance imaging and magnetic resonance cholangiopancreatography (MRCP) showed the ectopic hepatopancreatic ampulla draining into the fourth part of the duodenum adjacent to the duodenojejunal flexure; the irregular morphology of the duodenojejunal flexure likely due to a soft tissue mass. Laparotomy confirmed the presence of the abnormal ampulla of Vater located at the fourth part of the duodenum and a soft tissue tumor about 6 cm x 5 cm x 5 cm with a peduncle adjoining the ampulla. Resection of the tumor, including some peripheral tissue, and a Roux-Y loop anastomosis choledochojejunostomy were performed. Pathological examination indicated an intestinal villous adenoma accompanied by severe dysplasia and focal canceration. Periampullary carcinoma with ectopic ending of the Vater's ampulla into the fourth part of the duodenum is rather rare. The embryonic genetic background of this anomaly has not yet been fully explained. It is worth mentioning that MRCP is useful for demonstrating anomalies and anatomic variants of the biliary tract system and pancreatic duct.

Surgical Experience in a Baby With Congenital Broncho-Biliary Fistula

Congenital tracheobiliary and bronchobiliary fistulae are rare malformations in which patent communications exist between the respiratory system and biliary tract, respectively. We present a newborn who was admitted with respiratory distress and bilious tracheal discharge. Investigation revealed a bronchobiliary fistula originating from the left main bronchus, as well as biliary atresia. Excision of the bronchobiliary fistula was successful and the connection between biliary tract and gastrointestinal system was established by performance of a Roux-en-Y cholecysto-jejunostomy. Diagnostic tools, differential diagnosis, and surgical correction strategies are discussed.

Mortality Associated with Pheochromocytoma

A consecutive series of patients (68 females and 53 males) with pheochromocytoma (PC, n=110) or paraganglioma (PG, n=11) were treated at the Sahlgrenska University Hospital (1950-1997). During the observation period (15+/-6 years) 42 patients died versus 23.6 expected in the general population (P<0.001). There was no surgical mortality. Twenty patients died of cardiovascular disease, 11 of other tumors, and 7 of other diseases, but only 4 of PC/PG. The main causes of death in this regional series were cardiovascular diseases and tumor in a ratio of 1.3 versus 2.0 in the general Swedish population. Analysis of the mortality in all patients with clinically diagnosed PC (n=481, 259 women and 222 men) based on the National Cancer Registry (1957-1997) showed that the number of deaths in this cohort was 196 versus 153.4 expected (P<0.001). These patients had almost four times higher risk of dying of a tumor than did the general population (similar risk for females and males). There was no increased risk for cardiovascular death; in fact, the risk was lower than expected for men (22 vs. 38 expected). A second tumor diagnosed subsequent to PC occurred in 68 versus 31 expected. In men tumors of the liver and biliary tract and central nervous system and in women malignant melanoma and cervix carcinoma were most frequent. The results from the national series thus confirm an increased risk of a second tumor and increased tumor-related mortality in patients with PC.