ABSTRACT
BACKGROUND/AIMS: Precursor lesions of small duct type intrahepatic cholangiocarcinoma (small duct iCCA) have not been clarified so far. We hypothesised that precursor lesions may be frequently distributed in the background liver of small duct iCCA. METHODS AND RESULTS: We determined by histology the presence of bile duct adenomas and von Meyenburg complexes as candidate precursor lesions in the background liver of small duct iCCA, with other primary liver carcinomas as control. Subjects included 28 patients with small duct iCCA, 29 with large duct iCCAs, 60 with combined hepatocellular-cholangiocarcinoma (Comb) and 40 with hepatocellular carcinoma (HCC). The prevalence of bile duct adenomas in the background liver was significantly higher in small duct iCCA (35.7%) compared to other primary liver carcinomas (Comb, 4.9%; 10%, HCC) (P < 0.01). The prevalence of bile duct adenomas was significantly associated with the presence of von Meyenburg complexes and ductal plate malformation-like patterns in small duct iCCAs and Combs. Von Meyenburg complexes were detected in 11 small duct iCCA (39.3%), five large duct iCCAs (17.2%), 10 Comb (16.4%) and 13 HCC (33.3%), respectively (P > 0.05). Small duct iCCAs showed altered expression of ARID1A (46.4%), p53 (39.3%), PBRM1 (14.3%), IMP3 (85.7%) and EZH2 (82.1%), whereas these markers were negative in bile duct adenomas. CONCLUSION: Bile duct adenomas may be precursor lesions of small duct iCCAs. Alteration of ARID1A, p53 or PBRM1 may be involved in the carcinogenesis of small duct iCCAs.
Subject(s)
Adenoma, Bile Duct/complications , Cholangiocarcinoma/etiology , Adenoma, Bile Duct/diagnosis , Adenoma, Bile Duct/pathology , Adult , Aged , Aged, 80 and over , Bile Duct Neoplasms/pathology , Bile Ducts, Intrahepatic/pathology , Biomarkers, Tumor , Carcinoma, Hepatocellular/diagnosis , Carcinoma, Hepatocellular/etiology , Carcinoma, Hepatocellular/pathology , Cholangiocarcinoma/diagnosis , Cholangiocarcinoma/pathology , Diagnosis, Differential , Female , Humans , Liver/pathology , Liver Neoplasms/pathology , Male , Middle AgedABSTRACT
AIMS: Both homozygous and heterozygous α1 -antitrypsin (AAT) deficiency patients are at risk of developing hepatocellular carcinoma (HCC), but also of developing cholangiocarcinoma and combined HCC and cholangiocarcinoma. The aim of our study is to report a series of bile duct adenomas (BDAs) and intrahepatic cholangiocarcinoma (ICCs) in adult AAT deficiency patients, observed in our institution over a 5-year period. Our observational study includes a detailed investigation of their immunohistochemical profile and BRAF V600E mutation status. METHODS AND RESULTS: Eleven biliary lesions from five AAT deficiency patients (six BDAs from three cirrhotic patients with other concurrent liver diseases; three BDAs and two ICCs from two non-cirrhotic patients) were identified between 2010 and 2015 during routine histological investigation. Most BDAs expressed CD56, EpCAM, CD133, and CA19-9, similarly to hepatic progenitor cells (HPCs), and carried the BRAF V600E mutation (87.5%). One ICC showed a similar immunohistochemical profile but no evidence of the BRAF V600E mutation. CONCLUSIONS: Most of the biliary proliferations in AAT deficiency patients have an appearance of BDA with an HPC-related immunohistochemical profile. Their frequent BRAF V600E mutations support their neoplastic nature, but not necessarily their progression to ICC. We believe that this may depend on the patient genotype, or require a different pathway or a second mutational hit for malignant transformation. We postulate that BDA represents a heterogeneous group of biliary lesions, and that those associated with AAT deficiency may constitute a group of their own.
Subject(s)
Adenoma, Bile Duct/complications , Bile Duct Neoplasms/complications , Bile Ducts, Intrahepatic/pathology , Cholangiocarcinoma/complications , Proto-Oncogene Proteins B-raf/genetics , alpha 1-Antitrypsin Deficiency/complications , Adenoma, Bile Duct/genetics , Adenoma, Bile Duct/pathology , Bile Duct Neoplasms/genetics , Bile Duct Neoplasms/pathology , Cholangiocarcinoma/genetics , Cholangiocarcinoma/pathology , DNA Mutational Analysis , Female , Humans , Immunohistochemistry , Male , Middle Aged , MutationABSTRACT
Morphologic features and neoplastic potentials of bile duct adenoma (BDA) and von Meyenburg complex (VMC)-like duct arising in chronic liver disease were unknown. Thirty-five BDAs and 12 VMC-like duct lesions were observed in 39 cases with chronic liver disease. BDAs were divided into the EMA-cytoplasmic type (n = 14) and EMA-luminal type (n = 21). EMA-cytoplasmic BDA composed of a proliferation of cuboidal to low-columnar cells forming an open lumen with NCAM(+)/MUC6(-), resembling an interlobular bile duct. EMA-luminal BDA showed uniform cuboidal cells with narrow lumen, and NCAM(++)/MUC6(++), resembling a ductular reaction. VMC-like duct showed positive MUC1 expression and negative MUC6. The expression of S100P, glucose transporter-1 (GLUT-1) and insulin-like growth factor II mRNA-binding protein 3 (IMP-3) were not detected in three lesions. p16 expression was higher than those of the ductular reaction, and the Ki67 and p53 indexes were very low (<1.0%). Large-sized EMA-luminal BDA shows sclerotic stroma. We classified small nodular lesions of ductal or ductular cells in chronic hepatitis and cirrhosis into the following groups: BDA, interlobular bile duct type; BDA, ductular/peribiliary gland type; and VMC-like duct. They may be reactive proliferation rather than neoplastic lesions.
Subject(s)
Adenoma, Bile Duct/pathology , Bile Duct Neoplasms/pathology , Bile Ducts, Intrahepatic/pathology , Hamartoma/pathology , Hepatitis/pathology , Adenoma, Bile Duct/complications , Adenoma, Bile Duct/metabolism , Aged , Bile Duct Neoplasms/complications , Bile Ducts, Intrahepatic/metabolism , Female , Hamartoma/complications , Hepatitis/complications , Humans , Liver Cirrhosis/pathology , Male , Middle AgedABSTRACT
The purpose of this study was to report the magnetic resonance imaging (MRI) features of multiple biliary hamartoma (MBH) and to correlate them with histopathology. MRI features of 11 patients with MBH proven by histology were retrospectively reviewed and correlated to histopathology. MBH presented as multiple, tiny, and uniformly distributed lesions in all cases. All were hypointense on T1-weighted images and hyperintense on T2-weighted images relative to the liver parenchyma. Mural nodules were identified in 10 of 11 (91%) cases. They were isosignal on T1-weighted images, intermediate signal on T2-weighted images. Gadolinium-enhanced images showed mural nodule enhancement in 9 of 10 patients (90%) or a peripheral rim-like enhancement of the whole lesion in one case (9%). MBH were present in all liver specimens. In the six patients examined at MR cholangiography, the lesions lacked communication with the biliary tree. At histopathology, the mural nodule corresponded to an endocystic polypoid projection made of conjunctive septa. Three (27%) patients had associated focal nodular hyperplasia, and 1 (9%) had concomitant cholangiocarcinoma. MRI features allowed diagnosis of MBH with accuracy. Their recognition, especially the mural nodule, may help avoid misdiagnosis.
Subject(s)
Bile Ducts, Intrahepatic/abnormalities , Hamartoma/diagnosis , Magnetic Resonance Imaging , Adenoma, Bile Duct/complications , Adenoma, Bile Duct/diagnosis , Adult , Aged , Bile Duct Neoplasms/complications , Bile Duct Neoplasms/diagnosis , Bile Ducts, Intrahepatic/pathology , Female , Gadolinium , Hamartoma/congenital , Hamartoma/pathology , Humans , Liver/pathology , Male , Middle AgedABSTRACT
No disponible
Subject(s)
Male , Middle Aged , Humans , Hemobilia/complications , Hemobilia/diagnosis , Hemobilia/surgery , Hemorrhage/complications , Hemorrhage/etiology , Adenoma/etiology , Ureterostomy/methods , Endoscopy, Digestive System/methods , Tomography, Emission-Computed/methods , Urinary Bladder Neoplasms/complications , Gastrointestinal Hemorrhage/complications , Hematemesis/complications , Ventricular Fibrillation/complications , Cardiopulmonary Resuscitation/methods , Adenoma/complications , Hematemesis/diagnosis , Angiography/methods , Abdomen/pathology , Abdomen , Adenoma, Bile Duct/complicationsABSTRACT
Enteroenteric intussusception is a condition in which full-thickness bowel wall becomes telescoped into the lumen of distal bowel. In adults, there is usually an abnormality acting as a lead point, usually a Meckels' diverticulum, a hamartoma or a tumour. Duodeno-duodenal intussusception is exceptionally rare because the retroperitoneal situation fixes the duodenal wall. The aim of this report is to describe the first published case of this condition. A patient with duodeno-duodenal intussusception secondary to an ampullary lesion is reported. A 66 year-old lady presented with intermittent abdominal pain, weight loss and anaemia. Ultrasound scanning showed dilated bile and pancreatic ducts. CT scanning revealed intussusception involving the full-thickness duodenal wall. The lead point was an ampullary villous adenoma. Congenital partial (type II) malrotation was found at operation and this abnormality permitted excessive mobility of the duodenal wall such that intussusception was possible. This condition can be diagnosed using enhanced CT. Intussusception can be complicated by bowel obstruction, ischaemia or bleeding, and therefore the underlying cause should be treated as soon as possible.
Subject(s)
Adenoma, Bile Duct/complications , Common Bile Duct Neoplasms/complications , Duodenal Obstruction/etiology , Duodenum/abnormalities , Intussusception/etiology , Aged , Duodenal Obstruction/diagnosis , Female , Humans , Intussusception/diagnosis , RotationABSTRACT
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.
Subject(s)
Adenoma, Bile Duct/complications , Adenomatous Polyposis Coli/complications , Ampulla of Vater , Common Bile Duct Neoplasms/complications , Pancreatitis/etiology , Acute Disease , Adenoma, Bile Duct/surgery , Adult , Common Bile Duct Neoplasms/surgery , Female , Humans , Pancreaticoduodenectomy , Pancreatitis/surgery , RecurrenceABSTRACT
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.
Subject(s)
Adenoma, Bile Duct/complications , Bile Duct Neoplasms/complications , Bile Ducts, Intrahepatic/pathology , Mucins/metabolism , Pancreatitis/etiology , Adenoma, Bile Duct/pathology , Adenoma, Bile Duct/surgery , Bile Duct Neoplasms/pathology , Bile Duct Neoplasms/surgery , Bile Ducts, Intrahepatic/surgery , Humans , Jaundice, Obstructive/etiology , Jaundice, Obstructive/pathology , Jaundice, Obstructive/surgery , Male , Middle Aged , Pancreatitis/pathology , Pancreatitis/surgery , Treatment OutcomeABSTRACT
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.
Subject(s)
Adenoma, Bile Duct/complications , Adenoma, Liver Cell/complications , Adenoma, Bile Duct/diagnostic imaging , Adenoma, Bile Duct/surgery , Adenoma, Liver Cell/diagnostic imaging , Adenoma, Liver Cell/surgery , Adult , Female , Humans , Preoperative Care , Tomography, X-Ray ComputedSubject(s)
Adenoma, Bile Duct/diagnosis , Bile Duct Neoplasms/diagnosis , Bile Ducts, Intrahepatic , Adenoma, Bile Duct/complications , Adenoma, Bile Duct/diagnostic imaging , Bile Duct Neoplasms/complications , Bile Duct Neoplasms/diagnostic imaging , Bile Ducts, Intrahepatic/diagnostic imaging , Bile Ducts, Intrahepatic/pathology , Biopsy , Hepatitis C/complications , Humans , Magnetic Resonance Imaging , Male , Middle Aged , UltrasonographySubject(s)
Adenoma, Bile Duct/pathology , Bile Duct Neoplasms/pathology , Gallbladder Diseases/diagnosis , Liver Diseases/diagnosis , Polyarteritis Nodosa/diagnosis , Abdominal Pain/diagnosis , Abdominal Pain/etiology , Adenoma, Bile Duct/complications , Adenoma, Bile Duct/surgery , Aged , Bile Duct Neoplasms/complications , Bile Duct Neoplasms/surgery , Bile Ducts, Intrahepatic , Biopsy, Needle , Dilatation, Pathologic/pathology , Female , Follow-Up Studies , Gallbladder Diseases/complications , Humans , Immunohistochemistry , Laparotomy , Liver Diseases/complications , Polyarteritis Nodosa/complications , Severity of Illness Index , Treatment OutcomeABSTRACT
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.
Subject(s)
Bile Ducts, Intrahepatic/pathology , Biliary Tract Neoplasms/pathology , Hepatitis C/pathology , Liver Cirrhosis/pathology , Papilloma/pathology , Adenocarcinoma/complications , Adenocarcinoma/secondary , Adenocarcinoma/surgery , Adenoma, Bile Duct/complications , Adenoma, Bile Duct/pathology , Adenoma, Bile Duct/surgery , Aged , Biliary Tract Neoplasms/complications , Biliary Tract Neoplasms/surgery , Fatal Outcome , Hepacivirus/isolation & purification , Hepatitis C/complications , Humans , Liver Cirrhosis/complications , Liver Cirrhosis/surgery , Lung Neoplasms/secondary , Male , Papilloma/complications , Papilloma/surgeryABSTRACT
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.
Subject(s)
Adenoma, Bile Duct/complications , Bile Duct Neoplasms/complications , Bile Ducts, Intrahepatic , Common Bile Duct/pathology , Adenoma, Bile Duct/pathology , Adenoma, Bile Duct/surgery , Bile Duct Neoplasms/pathology , Bile Duct Neoplasms/surgery , Bile Ducts, Intrahepatic/pathology , Bile Ducts, Intrahepatic/surgery , Cholangiopancreatography, Endoscopic Retrograde , Female , Humans , Middle AgedABSTRACT
Biliary cystadenoma is a rare cause of obstructive jaundice. We report a case of a 78-year-old Japanese man with biliary cystadenoma presenting repetitive abdominal pain and jaundice. Ultrasound sonography revealed a hyperechoic mass in the left lateral lobe of the liver. Histological examination revealed a biliary cystadenoma. Intracystic hemorrhage was assumed to be the cause of obstruction of the bile ducts.
Subject(s)
Adenoma, Bile Duct/complications , Bile Duct Neoplasms/complications , Bile Ducts, Intrahepatic , Cholestasis, Intrahepatic/etiology , Cystadenoma/complications , Aged , Humans , MaleABSTRACT
Biliary cystadenoma is a rare tumor of the liver. We describe a biliary cystadenoma of the left lobe of the liver with intracystic gallstone formation. This is the first report of stone formation in biliary cystadenoma of the liver.
Subject(s)
Adenoma, Bile Duct/complications , Bile Duct Neoplasms/complications , Bile Ducts, Intrahepatic/pathology , Cholelithiasis/complications , Cystadenoma/complications , Adenoma, Bile Duct/pathology , Bile Duct Diseases/complications , Bile Duct Diseases/pathology , Bile Duct Neoplasms/pathology , Cholelithiasis/pathology , Cystadenoma/pathology , Female , Humans , Middle AgedABSTRACT
BACKGROUND: In hepatocellular carcinoma (HCC), a high prevalence of hepatitis C virus antibody (anti-HCV) has been reported, indicating that it may be an important etiologic factor in the pathogenesis of HCC. In this study, the authors investigated the prevalence of anti-HCV in HCC patients, as well as the same prevalence in patients with cholangiocarcinoma (CC) and combined hepatocellular-cholangiocarcinoma (combined HCC-CC), to study the clinicopathologic features of anti-HCV-positive cases. METHODS: The authors examined 141 patients with primary liver cancer who were pathologically diagnosed as having HCC (121 cases), CC (13 cases), or combined HCC-CC (7 cases). Hepatitis B surface antigen (HBsAg) and anti-HCV were measured in these patients. RESULTS: Of 121 HCC cases, 85 (70.3%) were found to be anti-HCV positive, 16 (13.2%) were HBsAg positive, and 5 (4.1%) were both anti-HCV and HBsAg positive. In 13 cases with CC and in 7 with combined HCC-CC examined, 4 (30.8%) and 5 (71.4%), respectively, were anti-HCV positive. CONCLUSIONS: The anti-HCV-positive rate was high in combined HCC-CC as well as in HCC. These three types of primary liver cancer, which were anti-HCV positive, shared two common features: male dominance and high incidences of complication with liver cirrhosis.
Subject(s)
Adenoma, Bile Duct/immunology , Carcinoma, Hepatocellular/immunology , Hepacivirus/immunology , Hepatitis Antibodies/blood , Liver Neoplasms/immunology , Adenoma, Bile Duct/complications , Adult , Aged , Carcinoma, Hepatocellular/complications , Female , Hepatitis B Surface Antigens/analysis , Hepatitis C/complications , Hepatitis C/transmission , Humans , Liver Cirrhosis/complications , Liver Neoplasms/complications , Male , Middle Aged , Sex Factors , Transfusion ReactionABSTRACT
The role of intraduodenal bile in regulation of plasma cholecystokinin (CCK) levels were investigated in patients with obstructive jaundice under external bile diversion and under physiological bile flow into the duodenum by internal bile drainage. Basal plasma CCK levels determined by a specific and sensitive bioassay in patients under external bile drainage (2.2 +/- 0.2 pmol/liter; mean +/- SE) were significantly higher than those in control subjects (1.0 +/- 0.3 pmol/liter). In control subjects, the peak CCK response (6.2 +/- 0.7 pmol/liter) to a test meal was seen at 45 min, whereas that in patients under external bile drainage, it was seen at 20 min after a test meal (17.6 +/- 3.2 pmol/liter; P < 0.01 vs controls). After peak response, plasma CCK levels in controls gradually decreased, but remained significantly elevated during a 3-hr observation period. In patients under bile diversion, the test meal caused a prompt plasma CCK peak, with a transient fall followed by a continuous rise until 180 min postprandially. In six patients, external bile diversion was changed to internal biliary drainage with a stent tube within two weeks to maintain physiological bile flow into the duodenum. Internal bile drainage normalized basal (0.9 +/- 0.2 pmol/liter) as well as meal-stimulated CCK release (peak value: 5.0 +/- 0.8 pmol/liter). These results demonstrate that endogenous bile exerts tonic inhibition on basal and postprandial plasma CCK levels in humans.
Subject(s)
Bile/physiology , Cholecystokinin/blood , Eating/physiology , Adenoma, Bile Duct/complications , Adult , Aged , Ampulla of Vater , Bile Duct Neoplasms/complications , Cholestasis/blood , Cholestasis/etiology , Common Bile Duct Neoplasms/complications , Drainage/methods , Female , Humans , Male , Middle Aged , Pancreatic Neoplasms/complications , Time FactorsABSTRACT
The authors present a case of Caroli's disease complicated with multiple lithiasis and cholangiocarcinoma. They analyse the etiopathogenesis and the anatomopathologic features of this uncommon disease and express some diagnostic and therapeutic considerations.