A Comprehensive Approach to the Management of Benign and Malignant Ampullary Lesions: Management in Hereditary and Sporadic Settings.

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.

Imaging features of bile duct adenoma: case series and review of literature.

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.

Magnetic Resonance Imaging Findings of Intrahepatic Bile Duct Adenoma: A Report of 4 Cases.

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.

Bile duct adenoma: a case report and literature review.

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 neoplasm arising from peribiliary glands connecting with the inferior branch of the bile duct of the anterior segment of the liver.

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.

Imaging findings of intrahepatic bile duct adenoma (peribiliary gland hamartoma): a case report and literature review.

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.

An immunohistochemical profile of the so-called bile duct adenoma: clues to pathogenesis.

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.

Papillary adenoma arising in the left hepatic duct: an unusual tumour in an uncommon location.

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.

Relapsing acute pancreatitis due to ampullary adenoma in a patient with familial adenomatous polyposis.

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.

Magnetic resonance findings of bile duct adenoma with calcification.

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.

Pancreatitis complicating mucin-hypersecreting common bile duct adenoma.

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.

[Hepatobiliary cystoadenomas]. / Cistoadenomas hepatobiliares.

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.

Cistoadenomas hepatobiliares / Hepatobiliary cystoadenomas

Los cistoadenomas hepatobiliares son tumores quísticos infrecuentes. Su diagnóstico preoperatorio es difícil ya que con frecuencia se confunden con otras lesiones quísticas más comunes. Son lesiones con potencial maligno, de ahí que su tratamiento consista en la extirpación quirúrgica. Los datos de laboratorio, la historia clínica y las imágenes radiológicas deben orientarnos hacia su diagnóstico. Se presentan dos de estos infrecuentes casos y se discuten tanto los modos de presentación como los tipos histológicos y el tratamiento que precisan estas lesiones (AU)

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 (AU)

Malignant intrahepatic biliary papillomatosis associated with viral C cirrhosis.

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.