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Purpose of Review: The purpose of this review was to examine the historical roots of endoscopic management of ampullary lesions and explore emerging data on improved techniques, technologies, and outcomes. Of specific interest was answering whether there exists a reasonable body of data to support one resection technique or strategy above others., Recent Findings: Review of recent literature suggests the continued use of endoscopic ampullectomy is a safe and effective means of curative treatment of ampullary adenomas. Complications are relatively infrequent and complete endoscopic resection is possible in a majority of cases, with proper patient and lesion selection. Greater than 2 decades of experience with endoscopic ampullectomy have shown this to be a viable, well-tolerated, and highly effective means of treating ampullary adenomas. While few concrete guidelines exist to advise endoscopists on the ideal technique for resection, experience, patient selection, and prior planning can greatly influence the technical and clinical success of endoscopic ampullectomy.

Purpose: We aimed to evaluate the imaging features of bile duct adenoma (BDA) on ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI)., Methods: Retrospective search in our institution database was performed for histologically confirmed BDA. Their imaging studies before histologic confirmation were reviewed. The search identified seven adults (mean age, 52.9 years) with histologically proven single BDA each. US (n=3), CT (n=5), and MRI (n=3) were performed before histologic confirmation. Additionally, a systematic English literature review for BDA and reported imaging findings since 2000 was also conducted using the following search criteria "bile duct adenoma, peribiliary hamartoma, biliary adenoma, CT, ultrasound, MRI" (date range: 01/01/2000 through 08/31/2016). The imaging findings of those cases reported were summarized and compared with our series., Results: All seven individual nodules were well circumscribed. Five lesions were located in the right hepatic lobe and two in the left hepatic lobe. On US, lesions appeared hypoechoic (n=2) and hyperechoic (n=1). BDA was hypodense on unenhanced CT images (n=1). On MRI, BDA were hypointense on T1 (n=3), hyperintense on T2 (n=3), and hyperintense on diffusion-weighted images (n=2). On contrast-enhanced CT and MRI, BDAs showed arterial phase hyperenhancement that persisted on portal venous/delayed phase images., Conclusion: BDA demonstrates characteristic arterial phase hyperenhancement that persisted into the portal venous and delayed phases on CT and MRI, which may be useful in differentiating from other hepatic lesions.

Intrahepatic bile duct adenoma (BDA) is a rare type of benign hepatic lesions. In this study, 4 cases of BDA diagnosed from surgical resection pathology were examined. Their clinical and magnetic resonance imaging (MRI) data were retrospectively analyzed. The 4 cases (1 men and 3 women) were aged 21 to 55 years without obvious clinical symptoms. Three were identified through routine examination. Three had a history of chronic hepatitis B virus infection. Two cases were accompanied by hepatocellular carcinoma, and one had a higher level of α-fetoprotein. The MRI images of BDA all manifested as peripheral hepatic nodules with abnormal signals. The diameters of the lesions in the 4 cases were 7.7 to 17.0 mm. The MRI images showed slight hypointensity on T1WI and slight hyperintensity on T2WI in all cases, and they showed slight hyperintensity in 2 cases and hyperintensity in 2 cases on diffusion-weighted imaging. Dynamic contrast-enhanced MRI scans show hyperintensity in the arterial phase and slight hyperintensity in the late stage in 3 cases. The other case shows hyperintensity in the arterial and portal phases and isointensity at the delayed phase. During follow-up, 3 cases were recurrence-free. The other case was complicated by the reoccurrence of HCC. In general, BDA shows specific MRI characteristics, and peripheral hepatic nodules show slight hypointensity on T1WI and slight hyperintensity on T2WI. Dynamic contrast-enhanced MRI scans showed obvious enhancement in the arterial phase and continuous enhancement at the late stage.

Background: Bile duct adenoma (BDA) is a comparatively rare disease clinically, therefore, there are relatively few reported cases about it both in China and abroad., Case Presentation: Herein, we present a 51-year-old man, diagnosed preoperatively with enhanced-contrast abdominal computed tomography, as having a nodule in the left hepatic. The patient underwent a liver tumor resection, and the histological examination revealed bile duct adenoma (BDA)., Conclusions: BDA is an extremely rare benign tumor, which is difficult to distinguish BDA from hepatocellular carcinoma definitely preoperatively, surgical resection is needed as a way of treatment.

Intraductal papillary neoplasms of the bile duct are generally thought to arise from neoplastic papillary proliferation of epithelial cells lining the bile duct. We herein report a case with findings that strongly suggested that the biliary cystic tumor might have derived from a peribiliary gland. A 69-year-old female was found to have a cystic lesion with intracystic protrusions at the anterior segment of the right hepatic lobe and underwent hepatic anterior segment resection. Fluoroscopy of the resected specimen injected with contrast medium into the cyst revealed a connection between the cystic lesion and the bile ducts. The cyst was multilocular in appearance. On microscopic examination, the cyst was located within the portal tract of the inferior branch of the anterior segment and connected with the inferior branch of the bile duct. The wall of the hepatic cyst lacked an ovarian-like stroma. The tumor was composed of papillary and glandular components, and the tumor cells were similar to gastric foveolar and pyloric gland epithelia and regarded as adenoma. These tumor cells were positive for MUC 5AC, MUC6, and HIK1083. The tumor was finally diagnosed as an intraductal papillary neoplasm of the bile duct (adenoma, gastric type) arising from a peribiliary gland., (© 2011 The Authors. Pathology International © 2011 Japanese Society of Pathology and Blackwell Publishing Asia Pty Ltd.)

The so-called bile duct adenoma and peribiliary glands are characterized by the expression of two foregut antigens (designated D10 and 1F6) and secretion of acid mucin. On account of this similarity in phenotype and their frequent close association with a large caliber bile duct, it was earlier suggested that bile duct adenoma represent a peribiliary gland hamartoma. Here, we compare the expression of 13 tissue antigens in bile duct adenomas, other benign bile duct lesions, and various foregut-derived tissues, to further investigate the bile duct adenoma phenotype and pathogenesis. Antibodies to 4 intestinal mucins, 3 cytokeratins, and CDX2, were not informative. Five foregut antigens (D10, 1F6, MUC6, MUC5AC, and TFF2) and secretion of acid mucin were of use in distinguishing bile duct adenoma from other hepatic lesions. 1F6 and MUC6 were normally present in bile ductules and canals of Hering, whereas the epithelium lining the larger bile ducts stained focally for D10, MUC5AC, MUC6, or TFF2 in, respectively, 21%, 36%, 43%, and 100% of the livers examined. Thirty-six bile duct adenomas examined were distinguished by expression of MUC6 (94% of bile duct adenoma), MUC5AC (90%), TFF2 (80%), D10 (67%), and 1F6 (61%), and varying degrees of acid mucin secretion (100%). Of 30 bile duct adenoma tested for all 5 antigens, 40% expressed all 5, 27% expressed 4, 17% expressed 3, 13% expressed 2, and 1 expressed only MUC6. Peribiliary glands invariably expressed D10, 1F6, MUC6, and TFF2, and showed acid mucin secretion, with MUC5AC present in the inflamed peribiliary glands of 3/4 livers with recurrent pyogenic cholangitis, but none of the glands of the other 23 normal or diseased livers tested. The acini of pyloric gland metaplasia in gallbladder and terminal ileum also stained for D10, 1F6, MUC6, and TFF2, with MUC5AC focally present in the gastric foveolar metaplasia overlying the pyloric gland metaplasia but not in the metaplastic glands. MUC6 was expressed in 92% of ductular reactions, 1F6 in 42%, and D10 in 25%. Focal expression of MUC6, or TFF2 was observed in 1 or 2 examples of 14 von Meyenburg complexes and 6 polycystic livers, with staining for acid mucin generally obvious only in the glycocalyx of the epithelium of these two types of lesions. The distinguishing feature of so-called bile duct adenoma is their display of the same phenotype as pyloric gland metaplasia. It is concluded that they develop as a localized biliary healing response equivalent to the function of a peribiliary gland or pyloric gland metaplasia in the foregut.

Intrahepatic bile duct adenoma is a rare benign epithelial hepatic tumor derived from bile duct cells. We report the imaging findings of a patient with bile duct adenoma, which appeared as a small heterogeneously enhancing mass with focal small cystic change on CT and MRI. Follow-up images at seven months showed a slight increase in tumor size, which could be partly explained by intratumoral hemorrhage on pathologic examination. Although rare, bile duct adenoma should be considered as a differential diagnosis of a small hypervascular tumor located in the periphery of liver. Focal cystic change and intratumoral hemorrhage may occur.

Bile duct adenomas are rare tumours that arise more frequently in the distal extrahepatic biliary tree. We report the case of a papillary adenoma arising at the junction of the common and left hepatic ducts and review the available literature on this rare entity. A 73-year-old lady presented with a history of mild weight loss and vague upper abdominal pain. Routine blood tests revealed an elevated c-glutaryl transferase, and an ultrasound scan showed gross dilatation of the intrahepatic and extrahepatic biliary tree. Subsequent radiological imaging confirmed biliary dilatation and identified tumour within the left and common hepatic ducts with the provisional diagnosis of cholangiocarcinoma. At laparotomy, there was no evidence of extraductal tumour, and choledochoscopy showed a papillary lesion within the common hepatic and proximal left hepatic ducts. The tumour was excised and the biliary tree was reconstructed. Histological evaluation of the resected specimen confirmed a papillary adenoma with mild dysplasia. This case illustrates that not all biliary tumours are cholangiocarcinomas and referral to a hepatopancreaticobiliary unit for investigation and treatment is mandatory for all cases of obstructive jaundice.

Villous adenomas of the bile ducts are extremely uncommon. We describe a 58-year-old man presenting with clinical signs and laboratory findings of acute pancreatitis and obstructive jaundice. Preoperative investigation demonstrated a dilated papillary orifice with mucus exiting (fish-mouth sign) and a filling defect in the distal common bile duct. He underwent a modified Whipple operation and histological examination of the surgical specimen showed villous adenoma with rich secretion of mucus.

A patient with familial adenomatous polyposis (FAP) presented with a relapsing attack of acute pancreatitis. Evaluation using computed tomography, ultrasonography, and duodenoscopy revealed an ampullary adenoma, which was classified as Spigelman's stage III according to Spigelman's criteria. The patient underwent a pylorus-resected pancreatoduodenectomy, and has had no abdominal pain suggesting acute pancreatitis for 1 year after surgery. Only a few reports of acute pancreatitis due to ampullary neoplasms in patients with FAP are available. Relapsing acute pancreatitis is another surgical indication for premalignant periampullary neoplasms in FAP.

Computed tomographic (CT) and magnetic resonance (MR) appearances of bile duct adenoma (BDA in a patient who underwent partial hepatectomy of segment 8 are presented. BDA showed a ring-shaped hyperdense area suggesting calcification and ring enhancement on CT. It appeared hypointense on both T1- and T2-weighted MR images. Preoperatively, the diagnoses considered included metastatic carcinoma, cholangiocarcinoma, tuberculosis, and rare tumors such as epithelioid hemangioendothelioma. The radiological findings were confirmed by pathological investigation.

Cystoadenomas of the liver are rare cystic tumors that have seldom been reported. Accurate preoperative diagnosis is difficult because they are usually mistaken for more frequent lesions. Due to their malignant potential, complete surgical removal of the lesion is required. Correct diagnosis is guided by clinical history, radiological imaging, and laboratory parameters. We report two of these rare cases and discuss the spectrum of presentation, pathological features and treatment of these tumors.

Biliary papillomatosis is a rare entity characterized by multiple papillary adenomas involving extensive areas of the biliary tract with a great potential for recurrence and malignant transformation. It has been reported in association with Caroli disease and a choledochal cyst. We report herein a case of malignant intrahepatic biliary papillomatosis associated with cirrhosis secondary to hepatitis C. To the best of our knowledge, this is the first report of this association.

Background: Cystic lesions of the liver consist of a heterogeneous group of disorders and may present a diagnostic and therapeutic challenge. Large hepatic cysts tend to be symptomatic and can cause complications more often than smaller ones., Study Design: We performed a retrospective review of adults diagnosed with large (> or = 4 cm) hepatic cystic lesions at our center, over a period of 15 years. Polycystic disease and abscesses were not included., Results: Seventy-eight patients were identified. In 57 the lesions were simple cysts, in 8 echinococcal cysts, in 8 hepatobiliary cystadenomas, and in 1 hepatobiliary cystadenocarcinoma. In four patients, the precise diagnosis could not be ascertained. Mean size was 12.1 cm (range, 4 to 30 cm). Most simple cysts were found in women (F:M, 49:8). Bleeding into a cyst (two patients) and infection (one patient) were rare manifestations. Percutaneous aspiration of 28 simple cysts resulted in recurrence in 100% of the cases within 3 weeks to 9 months (mean 4(1/2) months). Forty-eight patients were treated surgically by wide unroofing or resection (laparoscopically in 18), which resulted in low recurrence rates (11% for laparoscopy and 13% for open unroofing). Four of the eight patients with echinococcal cysts were symptomatic. All were treated by open resection after irrigation of the cavity with hypertonic saline. There was no recurrence during a followup period of 2 to 14 years. Hepatobiliary cystadenomas occurred more commonly in women (F:M, 7:1) and in the left hepatic lobe (left:right, 8:0). Seven were multiloculated. All were treated by open resection, with no recurrence, and none had malignant changes. Cystadenocarcinoma was diagnosed in a 77-year-old man, and was treated by left hepatic lobectomy., Conclusions: Large symptomatic simple cysts invariably recur after percutaneous aspiration. Laparoscopic unroofing can be successfully undertaken, with a low recurrence rate. Open resection after irrigation with hypertonic saline is a safe and effective treatment for echinococcal cysts. Hepatobiliary cystadenomas have predilection for women and for the left hepatic lobe. Malignant transformation is an uncommon but real risk. Open resection is a safe and effective treatment for hepatobiliary cystadenoma, and is associated with a low recurrence rate.

Background and Study Aims: The mucin-hypersecreting bile duct tumor, which closely resembles an intraductal papillary mucinous tumor (IPMT) of the pancreas, is rare, and its clinical features are not well known. We report our experience of nine patients with this type of tumor, and analyze the data in order to elucidate its clinicopathologic characteristics., Patients and Methods: Between 1995 and 1998, nine consecutive patients (four men, five women; mean age 54) who were diagnosed as having a mucin-hypersecreting bile duct tumor were enrolled in this study., Results: Recent or previous attacks of biliary pain and acute cholangitis were reported by most of the patients. Characteristics included a widely open ampulla of Vater with extrusion of mucin, and a diffuse dilated intrahepatic and extrahepatic bile duct with amorphous filling defects on cholangiogram. On cholangioscopic examination, a papillary mass or minute mucosal lesion was found in the dilated bile duct containing thick viscid mucin. Nine patients were referred for an operation, and curative resection was performed in eight of them. Using histologic examination, well differentiated adenocarcinoma in the background of benign hyperplasia and adenoma was documented in all patients except one, who showed pure adenoma., Conclusion: The mucin-hypersecreting bile duct tumor can be characterized by a striking homology with IPMT of the pancreas in clinical, radiologic, and pathologic features.

Background: Biliary cystadenomas are rare cystic tumours that arise in the liver or less frequently in the extrahepatic biliary system. They are commoner in middle-aged women, their most favoured site is the right hepatic lobe., Methods: Case report and review of the literature., Results: We present only the second case of an intrahepatic cystadenoma causing luminal obstruction of the common bile duct. Clinical presentation is often non-specific and can prove to be a diagnostic challenge., Conclusion: Wide local excision of biliary cystadenomas is recommended, with regular radiological follow-up., (Copyright 2000 S. Karger AG, Basel)

Background/aims: To evaluate the phenomenon and the potential reasons for protrusion and growth of hepatobiliary cystadenoma into the extrahepatic bile ducts in our patients, accomplished by a review of the data regarding hepatobiliary cystadenomas published elsewhere., Methodology: In a retrospective open study conducted over the last eight years, five patients with hepatobiliary cystadenoma and one patient with hepatobiliary cystadenocarcinoma were operated on. All the patients were females aged between 25 to 61 years. Diagnostic procedures, laboratory, operative and histopathological findings and treatment were evaluated., Results: Most of our patients were found to have hepatobiliary cystadenoma located in the left surgical liver. In three out of five patients with HBC mesenchymal stroma was histologically detected. In two of the three, protrusion and growth into the extrahepatic bile ducts was found., Conclusion: Considering the pathogenesis, location and the morphology of HBC, the mesenchymal stroma may present the competent potential for intraductal progression of the tumor. Radical excision should be performed for successful treatment of hepatobiliary cystadenomas, because of the potential for reoccurrence.

Hepatobiliary cystadenoma is an uncommon lesion that is difficult to diagnose preoperatively. Here we report a 34-year-old woman who presented with enlargement of a cyst that had been observed for the previous 6 months. Diagnostic imaging revealed a 7-cm diameter cystic mass with irregular multiple septation in her liver. All laboratory test results were normal except for serum carbohydrate antigen (CA) 19-9 (62.5 U/ml). Because of the malignant potential and the history of enlargement, a complete surgical excision was performed. The patient was discharged after a good recovery; 2 months after surgery her serum CA19-9 level had returned to normal (32.9 U/ml). Regardless of the diagnostic modalities used, cystadenoma and cystadenocarcinoma cannot be differentiated with accuracy. Therefore complete surgical resection is the recommended therapy.

Three cases of intrahepatic biliary cystadenoma with mesenchymal stroma and one case of biliary cystadenocarcinoma are presented. Their immunohistochemical features and the surgical treatment are discussed together with a brief review of the literature. The benign cystadenomas stained positive for cytokeratin and CA 19-9 in the epithelium of the cyst wall. Mesenchymal stromal cells were strongly positive for a-SMA and moderately positive for desmin. The epithelium of the cystadenocarcinoma, however, was positive only for cytokeratin and the stroma only for a-SMA. Our findings indicate that biliary cystadenomas seem to be of primitive hepatobiliary origin. Furthermore, the malignant variant cystadenocarcinoma may loose its immunoreactivity for CA 19-9 and desmin.

Background: Multiple biliary papillomatosis is a rare tumour of the biliary tract but is capable of multicentric malignant transformation. The complete removal of these tumours is difficult because it involves a field change of the biliary tract., Methods: Exploration with choledochoscopy is a prerequisite for thorough assessment of the extent of involvement. Incorporation of laser ablation via a laser-delivering fibre with choledochoscopy permits complete removal of the tumour., Results: Repeated laser therapy via choledochoscopy through the T-tube tract enables complete ablation of the tumour which changes the outlook of the prognosis. This is confirmed on subsequent cholangiography., Conclusions: Laser therapy via choledochoscopy is a satisfactory mode of treatment for multiple biliary papillomatosis. It enables complete and precise ablation of the tumour which certainly changes the prognosis of this condition.

We report a case of biliary cystadenoma in a 40 years female who presented with an epigastric abdominal mass. The preoperative diagnosis was made by means of US, CT and MRI. Lack of calcifications and the serological tests ruled out hydatid disease. Total excision by shelling out the lesion from the liver parenchyma was performed. We recommend complete surgical removal as the treatment of choice due to the malignant potential and the high recurrence rate after partial resections.

Biliary cystadenomas are rare neoplasms usually found in the liver. These neoplasms have a strong tendency to recur and undergo malignant transformation, and so differentiating between cystadenomas and other cystic lesions of the liver is very important. We describe herein the characteristics of these neoplasms and report the first case of an intrahepatic biliary cystadenoma being demonstrated by intraoperative cholangiography. In our case, intraoperative cholangiography was very useful in differentiating a cystadenoma or cystadeno-carcinoma from other cystic mass lesions of the liver. Not only did it reveal a communication between the intrahepatic bile duct and the cystadenoma, but it also allowed obtain fluid for cytology.

Objective: The aim of this investigation was to describe the clinical features, diagnosis, pathologic characteristics, and optimal surgical management for patients with extrahepatic biliary cystadenomas., Summary Background Data: Extrahepatic biliary cystadenomas are rare epithelial neoplasms. The clinical features and optimal surgical management for these lesions have not been defined clearly. The usual presenting symptom is jaundice. These lesions should be considered premalignant and necessitate resection. Sporadic case studies have reported instances of recurrence with local excision. To the authors' knowledge, this study represents the largest collected single series of extrahepatic biliary cystadenomas and reviews previously reported cases., Methods: The authors reviewed and reported their institutional experience from 1950 to 1993 in treating seven patients with extrahepatic biliary cystadenomas as well as 19 previously reported cases in the literature., Results: A strong female predominance (96.3% of patients reviewed) was associated with extrahepatic biliary cystadenomas. Obstructive jaundice was the most common presenting symptom (85%). Abdominal pain occurred in 50% of patients; other symptoms included fever and hemobilia. The most common site of occurrence was the common hepatic duct (32%). Papillary cystadenoma with foci of invasive adenocarcinoma, thus supporting the malignant potential of cystadenomas, occurred in one patient. Local excision from the wall of the bile duct was performed in 18 patients and was associated with 50% recurrence within a mean follow-up of 13 months (range, 4-24 months). No recurrence was reported after formal sleeve resection and bilioenteric reconstruction., Conclusions: Extrahepatic biliary cystadenomas can become malignant, and in this study, local surgical excision was associated with a 50% local recurrence rate. Sleeve resection with negative histologic resection margins followed by bilioenteric reconstruction, therefore, is recommended.

We report on a case of 61 year old female who presented with upper abdominal discomfort. Ultrasound and computerized tomography scan revealed a liver cyst resembling a hydatid cyst. Signs of mild biliary obstruction were present in laboratory tests. At operation, a cystadenoma of the left liver protruding into the common bile duct was found. Left hepatectomy with resection of the common bile duct and right side cholangiojejunostomy were performed.

Like in adenomas of the colon, an adenoma-carcinoma sequence is expected from adenomatous growths in the papilla of Vater. Papilloadenomas must therefore be resected. Imaging techniques have a decisive influence on the choice of operative technique and consequently on perioperative mortality. Using conventional endoscopic ultrasonography, detection of lesions smaller than 10 mm in diameter is rare. The probes available up to now for intraductal sonography with an external diameter of between 3.4 and 12 mm were too rigid and could be inserted transpapillarily in only a few cases. The recent development, however, of high-frequency, extremely flexible ultrasound catheters with an external diameter of 1.17 mm makes intraductal examination of the pancreatic ductal system possible. We present a case of a 66 year-old female patient, in whom, for the first time, a histologically adenomatous lesion of the papilla of Vater could be examined with intraductal sonography using the endoscopic retrograde, transpapillary approach. As there was no evidence of infiltrative growth, the patient underwent a modified duodenum-preserving resection of the pancreatic head. Histological examination of the resected specimen confirmed the preoperative intraductal sonography findings. Since conventional endosonography did not detect the lesion in this case, intraductal sonography with flexible, high-frequency catheters as presented here, clearly may have a role in extending the possibilities of preoperative examination, especially of focal lesions in the periampullary region.

The clinical and radiological findings of 5 patients with a total of 7 hepatobiliary cystadenomas are reported. In all cases sonography and computed tomography were performed, in three cases angiography was carried out, and in one case magnetic resonance imaging was used. On sonography and computed tomography hepatobiliary cystadenomas exhibit relatively typical findings as a multicystic, space-occupying lesion with septation and papillary mucosal nodes; hence the diagnosis can be made preoperatively in most cases. For differential diagnosis, the malignant form, hepatobiliary cystadenocarcinoma has to be considered which can only be distinguished from the benign form by histology.

The medical and necropsy records of 41 cats diagnosed with nonlymphomatous hepatobiliary (NLHB) masses, including neoplasia and cysts, were reviewed. Overall, benign masses (n = 27) were more common than malignant ones (n = 14). The single most common malignancy was cholangiocellular carcinoma. The median age at diagnosis was significantly lower (P < .01) for cats with malignant rather than benign disease. Clinical signs associated with hepatobiliary neoplasia were usually vague and included lethargy, vomiting, and anorexia, often present for at least 2 weeks before presentation. Benign masses were an incidental finding in significantly more (P < .01) of the cases than were malignant masses. Median values for alanine aminotransferase (ALT), aspartate aminotransferase (AST), and total bilirubin were significantly higher (P < .05) in cats with malignant versus benign masses. The prognosis for malignant disease was poor, with 86% of the cats dying or being euthanatized during hospitalization. Cats with benign disease that underwent exploratory celiotomy were more likely to recover and warranted a more favorable prognosis than cats with malignant tumors. Factors associated with malignancy included age at presentation, presence of clinical signs at presentation, and specific serum chemistry changes.

The case of a 12-year-old boy with the diagnosis of intrahepatic biliary duct cystadenoma is presented. A critical review of the literature revealed less than 100 cases described at present. Differential diagnosis with other intrahepatic cystic lesions of varied etiology is discussed too.

Biliary cystadenoma is a rare benign neoplasm usually found in the parenchyma of the right lobe of the liver. The lesions are usually septated and represent less than 5 percent of non-parasitic cysts of biliary origin. Malignant degeneration to biliary cystadenocarcinoma is well documented, and recurrence is the rule following incomplete excision.

Background: Operative ultrasonography for orienting the direction of transection of the liver is often useful in obtaining an adequate disease-free surgical margin. We have devised a new technique for hepatectomy guided by needles inserted under ultrasonographic guidance., Methods: One hundred two hepatectomies were performed between January 1987 and September 1991, and the hepatectomy with this technique was begun in January 1989., Results: In 10 of 29 limited hepatectomies performed in the first phase of the period in which this technique was not available, disease-free surgical margin of less than 1 cm was left because of inadequately directed division. Disease-free surgical margin of more than 1 cm was left in 18 of 23 limited hepatectomies in the second phase of the period in which this technique was available. In the other five operations where disease-free surgical margin of less than 1 cm was left, carcinomas were located too close to the major hepatic vessels. The average blood loss during the limited hepatectomies was reduced by this technique. Two-year and 3-year survival of patients undergoing hepatectomy for hepatocellular carcinoma were more favorable in the second phase than in the first phase., Conclusions: Although the difference between the two groups was not significant, this technique is useful in performing adequate transection of the liver.

Objectives: To elucidate the clinical and pathologic features of extrahepatic cholangiocarcinomas and to identify prognostic variables in patients treated surgically., Design: Retrospective review of clinical records of patients undergoing surgical exploration for cholangiocarcinoma, with univariate and multivariate analyses of the clinical and pathologic factors that influenced patient survival., Setting: Mayo Clinic, Rochester, Minn., Study Participants: One hundred seventy-one patients undergoing operative intervention for diagnostic, palliative, or curative reasons between 1976 and 1985. Follow-up was complete until death or for a minimum of 5 years for surviving patients., Intervention: A curative surgical resection was performed in 29% of patients, while the remainder underwent tumor biopsy or a palliative procedure., Main Outcome Measure: Patient survival following operative treatment., Results: The operative mortality in this patient cohort was 5% and median survival was 13 months. Overall 5-year survival was 16%, with 44% of patients having a curative resection still alive at 5 years. Using univariate analysis, curative resection, tumor stage, Eastern Cooperative Oncology Group performance status, total bilirubin concentration, lymph node status, liver invasion, tumor morphology, tumor grade, and site of tumor origin were significant determinants of prognosis. Using the Cox proportional hazards model for multivariate analysis, curative resection, Eastern Cooperative Oncology Group performance status, total bilirubin concentration, and tumor grade were the only variables predictive of patient outcome. A curative resection of a proximal cholangiocarcinoma had a similar chance of providing long-term survival as a curative distal ductal resection., Conclusions: Although the tumor extent and the patient's overall health will affect outcome, curative resection for cholangiocarcinoma at all sites should be undertaken since this treatment offers the best chance for long-term survival.

A review of seven hepatic resections in six patients undergoing maintenance hemodialysis is presented. These cases consisted of hepatocellular carcinoma in four, and cholangiocellular carcinoma, myelolipoma and focal nodular hyperplasia in one each. The last preoperative hemodialysis was undertaken within 24 h prior to the operation with heparin. Intraoperatively, infused solutions containing no potassium, along with strict attention to preventing overhydration, allowed us to manage the patients without hemodialysis on the day of the operation. No specific intra-operative complications related to hemodialysis were noted. Postoperative hemodialysis was performed on the first or second day after operation, using nafamstat mesilate, a synthetic protease-inhibiting agent. The morbidity rate in the hemodialyzed patients was 85.7% (6/7), which was significantly higher than that in the non-hemodialyzed patients who underwent hepatic resections in our hospital. Fluid collection in the pleural and/or peritoneal cavities was frequent and difficult to control, but transient. Our experience suggests that hepatic resection is an acceptable procedure for hemodialyzed patients, when used in conjunction with careful perioperative management.

It is reported that a prompt increase in the arterial ketone body ratio (acetoacetate/beta-hydroxybutyrate), which reflects the mitochondrial redox state of the liver graft, is a decisive prerequisite for graft survival in clinical liver transplantation. To contrast the rôle of hepatic mitochondrial redox state in partial hepatectomy with that in hepatic replacement, the changes in the ketone body ratio were investigated in 107 cases of hepatectomy. The ketone body ratio in uneventful cases (n = 70) in the first three days after hepatectomy was significantly higher than that in eventful cases. In the uneventful cases, the ketone body ratios were all increased to above 1.0 within two days after hepatectomy, except in diabetics, whose preoperative values did not reach 1.0 under oral glucose load. However, 20 (22.7%) out of 88 cases whose ketone body ratios promptly increased after hepatectomy had mild to moderate complications thereafter. It is suggested that the recovery of hepatic mitochondrial redox state is also a prerequisite in partial hepatectomy, where a reduced and often damaged liver confronts systemic metabolic load.

Recent progress has been made in identifying patients with malignant primary liver tumors who have the best chance of long-term survival after orthotopic liver transplantation. Patients with UICC stages I or II hepatocellular carcinoma, the fibrolamellar variant of hepatocellular carcinoma, or epithelioid hepatic hemangioendothelioma are acceptable candidates for orthotopic liver transplantation. Carefully selected patients with neuroendocrine tumors metastatic to the liver may also have long-term palliation with liver transplantation. Therapeutic strategies are currently being developed to minimize disease recurrence after liver transplantation for patients with advanced-stage hepatocellular carcinoma confined to the liver or with cholangiocarcinoma. However, published survival rates for most patients with malignant primary liver tumors remain suboptimal, and significant progress in preventing disease recurrence is required before liver transplantation can be widely applied to most patients with primary malignant liver tumors.

Thirty-one patients underwent limited hepatic resection with Roux-en-Y biliary-enteric anastomosis and placement of biliary stents for cholangiocarcinoma of the hepatic duct bifurcation (Klatskin tumor). Resection included wide tumor excision and bile duct resection at the liver hilum without major hepatic resection and was undertaken in all patients unless precluded by intraoperative evidence of vascular invasion. All patients were operated on by a single surgeon during the 10 years between 1981 and 1991. Similar procedures were performed for both curative (n = 17) and palliative (n = 14) treatment of this disease entity. In this series, the overall mean postoperative survival of these patients was 17 months. The mean postoperative survival of patients undergoing surgery with curative intent was 21 months in contrast to 12 months for those undergoing planned palliation. One patient in this series has been alive for more than 6 years with no evidence of disease. Five patients experienced major postoperative complications (16%), and there were two perioperative deaths (6%). This retrospective review supports an aggressive surgical approach in patients with Klatskin tumor.

An interportal communicating branch was found at the hepatic bifurcation in two (2.4%) out of 83 hepatectomized cases of carcinoma of the biliary tract. The first case was a 54-year-old male with intrahepatic bile duct cancer, and the second was a 64-year-old female with gallbladder cancer. No other anatomical anomalies were found in the portal vein, hepatic artery, or biliary tract.

To evaluate whether intraoperative autologous transfusion increases the risk of hematogenous dissemination of tumor we reviewed the risk of lung metastasis as well as disease-free and long-term survival of patients who underwent resection of malignant hepatic neoplasms with this technique. A retrospective review of patients undergoing liver resection for malignant disease revealed 39 patients in whom intraoperative autologous transfusion was used. The 2-year actuarial survival in the patients in this series, as calculated with the Kaplan-Meier method, was predicted to be 75%. Two-year actuarial disease-free survival was predicted to be 28%, and the risk of developing lung metastasis at 3 years was estimated to be 40%. The predicted overall survival and risk of recurrence in this series compare favorably with published data for patients in whom intraoperative autologous transfusion was not used.

Self-expandable metallic biliary endoprostheses (= "stents") are an accepted therapeutic alternative in the palliative treatment of malignant biliary obstruction. The physiological direction of bile flow is re-established for a varying period of time. Sonography is an acutely available modality for the evaluation of symptomatic patients who are carriers of such endoprostheses. The cause of recurrent obstructive jaundice can be diagnosed quickly and a new intervention can be planned carefully.