Extrahepatic biliary cystadenocarcinoma arising from the left hepatic duct

A 54-year-old man, who had no clinical symptoms, underwent a routine health checkup at our hospital. Abdominal ultrasonography disclosed a well demarcated tumor containing a solid portion occupying the dilated left hepatic duct and a cystic portion expanding into the parenchyma of the left hepatic lobe, with mild dilatation of the intrahepatic bile ducts. These findings were later confirmed by computed tomography (CT) and magnetic resonance imaging. Endoscopic retrograde cholangiography revealed a complete defect at the level of the left hepatic duct, while drip infusion cholangiographic-CT (DIC-CT) disclosed a defect of the left hepatic duct only, with the distal portions of the left intrahepatic ducts being visualized on the image. Hepatic angiography revealed light stains in the solid portion in the parenchymal phase. At left lobectomy, a multiloculated polyp-like tumor was found arising from the left hepatic duct and expanding into the parenchyma of the left hepatic lobe. Microscopically, all the lining cells in the cysts and the tumor cells in the solid portion showed the features of papillary adenocarcinoma. In this patient with extrahepatic biliary cystadenocarcinoma, DIC-CT was useful in identifying the site of origin of the tumor, and hepatic angiography was also useful in differentiating this rare malignant tumor from benign cystadenoma. (Received July 11, 1997; accepted May 22, 1998)     

Biliary cystadenocarcinoma followed up as benign cystadenoma for 10 years

We describe a case of biliary cystadenocarcinoma that showed a longterm clinical course. A 69-year-old Japanese man was admitted to our hospital because of abdominal discomfort. The patient had been diagnosed with benign cystadenoma of the liver at another hospital in 1987 and had been followed up for 10 years. Abdominal ultrasonography (US) and computed tomography (CT) scan demonstrated a unilocular cystic lesion, which included multiple conspicuous papillary protrusions in the left hepatic lobe. Left lateral segmentectomy was performed, and intraoperative cholangiography revealed a communication between the cystic tumor and intrahepatic bile duct. The tumor contained clear mucinous fluid and enfolded multiple yellowish papillary projections on the cystic wall. Histological examination showed the tumor to be biliary cystadenocarcinoma. The patient is doing well 4 years after the operation.     

A special growth manner of intrahepatic biliary cystadenoma

We report a case of a 56-year-old woman with intrahepatic biliary cystadenoma (IBC) accompanying a tumor embolus in the extrahepatic bile duct, who was admitted to our department on October 13, 2008. Imaging showed an asymmetry dilation of the biliary tree, different bile signals in the biliary tree, a multiloculated lesion and an extrahepatic bile duct lesion with internal septation. A regular left hemihepatectomy en bloc was performed with resection of the entire tumor, during which a tumor embolus protruding into the extrahepatic bile duct and originating from biliary duct of segment 4 was revealed. Microscopically, the multiloculated tumor was confirmed to be a biliary cystadenoma with an epithelial lining composed of biliary-type cuboidal cells and surrounded by an ovarian-like stroma. An aggressive en bloc resection was recommended for the multiloculated lesion. Imaging workup, clinicians and surgeons need to be aware of this different presentation.     

Intraductal oncocytic papillary neoplasm of the bile duct: the first case of peribiliary gland origin

We report herein the first case of intraductal oncocytic papillary neoplasm of the bile duct arising from a peribiliary gland of the left hepatic duct. The patient was a 63-year-old Japanese man. Radiological and cholangioscopic examinations revealed intraductal tumor of the left hepatic duct. After pathological diagnosis of adenocarcinoma by cholangioscopic biopsy, a surgical hepatobiliary resection was performed. Pathological examination revealed papillary tumor in the left hepatic duct. Histologically, the tumor was identified as papillary neoplasm comprising oncocytic cells and delicate fibrovascular cores. Interestingly, this tumor originated from the cystic space in the bile duct wall. This cystic space was histologically identified as a cystically dilated peribiliary gland. Carcinoma in situ was observed in this cystic peribiliary gland at the bottom of the tumor, but not on any areas of biliary epithelium. This case suggests that intraductal papillary neoplasm can arise from both biliary epithelium and peribiliary glands.     

Definitive diagnosis of a duplicate gallbladder can only be made intraoperatively: report of a case

Duplicated gallbladders are rare congenital anomalies that are important in clinical practice as they may cause clinical, surgical, and diagnostic problems. Here, we describe the case of a 79-year-old female patient who presented with acute cholangitis. Abdominal ultrasonography, endoscopic ultrasonography, computed tomography, and magnetic resonance imaging revealed an intrahepatic cystic lesion, suggesting communication with the intrahepatic bile duct; no evidence of a polypoid lesion within the cystic lesion was observed. Based on these findings, intrahepatic cholangiectasis, intrahepatic bile duct cystadenoma, and the presence of a duplicated gallbladder were suspected, and surgery was performed. During surgery, a tube inserted into the common bile duct from a cystic duct facilitated intraoperative cholangiography, which indicated the presence of a duplicated gallbladder. Thus, we believe that a duplicated gallbladder should be an additional consideration when typical gallbladder disease symptoms are present under certain circumstances. A multimodal imaging approach can help to establish the diagnosis preoperatively or intraoperatively.     

Intrahepatic biliary cystadenoma

Summary A 24-year-old white woman presented with abdominal discomfort associated with a firm, nonpulsatile hepatic mass. Radiological examination, which included radionuclide liver scan, abdominal ultrasonography, computed tomography, and hepatic angiography, demonstrated a large, globular, multilocular cyst arising from the left lobe of the liver. A left hepatic lobectomy was performed, and gross examination of the specimen confirmed the cystic nature of the lesion. Histologically, the multiple locules were lined by a single layer of tall cuboidal to columnar cells, which were comparable to those of native biliary tract epithelium. There was little evidence of cellular pleomorphism, and invasion into the underlying mesenchymal stroma was not present. A diagnosis of intrahepatic biliary cystadenoma was made. The clinical, radiological, and pathological findings of this neoplasm are presented and its differential diagnosis discussed.     

Resected cystadenoma of the common bile duct

Biliary cystadenoma in the extrahepatic bile ducts is a very rare tumor. A 62-year-old woman with jaundice was admitted to our hospital. Imaging studies revealed a 4-cm cystic lesion around the hepatic hilum, compressing the common bile duct (CBD). When laparotomy was performed, a cystic tumor was detected in the hepatic hilum, filling the lumen of the CBD. Bile duct resection that included the tumor was performed, followed by biliary reconstruction. Microscopically, the cyst wall was lined by a single layer of cuboidal epithelial cells, covering an ovarian-like stroma. The degree of atypia was low and warranted the diagnosis of cystadenoma.     

Mucin-producing biliary papillomatosis associated with gastrobiliary fistula

We report a case of mucin-producing biliary papillomatosis in a 78-year-old woman. Abdominal ultrasound (US) and computed tomography (CT) showed wall thickening and dilatation of the intrahepatic bile duct (IHBD), as well as a nodular lesion, 1.2 cm in diameter, in the left branch of the IHBD. Gastric endoscopy revealed excretion of bile-containing mucin on the anterior wall of the body of the stomach. Endoscopic ultrasonography (EUS) showed gastrobiliary fistula and discharge of mucin into the stomach. Needle biopsy of the biliary tumor revealed papillary proliferation, but no malignant cells were recognized histologically. Therefore this patient was diagnosed as having mucin-producing biliary papillomatosis forming gastrobiliary fistula. She did not present with obstructive jaundice, probably because of the fistula. She is alive, without obstructive jaundice, 16 months after the diagnosis without having had surgery. This is, to our knowledge, the first reported case of biliary papillomatosis forming gastrobiliary fistula and with the patient free of obstructive jaundice. (Received May 22, 1998; accepted Aug. 28, 1998)     

Laparoscopic Anatomical Left Hepatectomy for Intrahepatic Bile Duct Papillary Mucinous Cystadenoma With Intraoperative Vascular Repair: A Case Report

Laparoscopic hepatectomy has been widely performed for patients with benign liver tumors such as hepatic hemangioma, focal nodular hyperplasia, and hepatic adenoma.We here present a case of a 78-year-old female patient who was initially admitted to our department due to fever and jaundice for 2 days. Abdominal enhanced computed tomography scan showed intrahepatic and extrahepatic bile duct dilatation with liver atrophy of left lobe. Unenhanced nodules were seen within the left intrahepatic bile duct. Ultrasonography revealed intrahepatic and extrahepatic bile duct dilatation with viscous fluid, tubular adenoma? Tumor markers including alpha fetoprotein, carcinoembryonic antigen, and CA19-9 were normal. Preoperative total bilirubin was 64.4 mmol/L.Laparoscopic anatomical left hepatectomy and common bile duct exploration were performed. In this procedure, a lot of mucus was seen within the common bile duct and left intrahepatic bile duct. No bile duct stones were found after the exploration. During parenchymal transection, intraoperative hemorrhage from middle hepatic vein was met, and we repaired middle hepatic vein by laparoscopic suture (5-0 Prolene). No air embolism and hypotension were met. This operation took 232 minutes and estimated blood loss was 300 mL. Postoperative ultrasonography indicated a normal outflow of middle hepatic vein and there was no stricture. The patient''s postoperative course was uneventful and was discharged on the 6th day after surgery. Postoperative pathological diagnosis was intrahepatic bile duct papillary mucinous cystadenoma.     

Long-term growth of intrahepatic papillary neoplasms: A case report

BACKGROUND Intraductal papillary neoplasm of the bile duct(IPNB) is a type of tumor that presents in the intra-or extrahepatic bile ducts. Cystic-type intrahepatic IPNB often mimics simple liver cysts, making the diagnosis difficult. Because the growth of IPNB is slow, careful follow-up and timely therapeutic intervention is recommended. There are few reports with a follow-up period longer than a decade; thus, we report the case of a patient with an IPNB that grew for over 13 years.CASE SUMMARY A 65-year-old man was diagnosed, 13 years prior with a cystic hepatic tumor with abnormal imaging findings. The targeted tumor biopsy results showed no malignancy. Biannual follow-up examinations were performed because of the potential for malignancy. The cystic lesions showed gradual enlargement over 11 years and a 4 mm papillary proliferation appeared on the cyst wall, which is compatible with IPNB. The tumor was observed for another 2 years because of the patient's wishes. The imaging findings showed enlargement to 8 mm and a new 9 mm papillary proliferation of the cystic tumor. Contrast-enhanced ultrasonography showed hyperenhancement during the arterial phase in both cyst walls, indicating intraductal tumor progression in both tumors. Thus, liver segment 8 subsegmentectomy was performed. The pathological findings indicated that the tumors contained mucin, and high-grade atypia was observed in the papillary lesions, showing IPNB.CONCLUSION The development of IPNB should be monitored in patients with cystic lesions and ultrasonography are useful tool for the evaluation.     

Biliary cystadenocarcinoma associated with atrophy of the left hepatic lobe and hepatolithiasis mimicking intrahepatic cholangiocarcinoma: a case report

Biliary cystadenocarcinoma and its benign counterpart, biliary cystadenoma, are rare hepatic cystic tumors arising from the hepatobiliary epithelium. We report the case of a 68-year-old Taiwanese woman who presented initially with acute cholangitis. A series of imaging studies including abdominal ultrasound, computerized tomography, endoscopic retrograde cholangiopancreatography, and percutaneous transhepatic cholangiography showed bilateral intrahepatic duct (IHD) and common bile duct (CBD) stones with IHD and CBD dilatation, and an ill-defined tumor within the atrophied left hepatic lobe. The patient underwent surgical resection of the tumor and choledocholithotomy. The pathologic diagnosis was biliary cystadenocarcinoma. We review this rare disease entity and discuss its unusual radiologic features mimicking intrahepatic cholangiocarcinoma.     

DIC-CT findings of biliary cystadenocarcinoma communicating with the bile duct: a case report

Among the intrahepatic cystic diseases except Caroli's disease, only biliary cystadenoma/cystadenocarcinoma may communicate with the bile duct. We present a case of biliary cystadenocarcinoma in which drip infusion cholangiographic-computed tomography demonstrated communication between an intrahepatic cyst and the biliary system preoperatively. Drip infusion cholangiographic-computed tomography, a simple and noninvasive examination, is useful for differentiating biliary cystadenoma/cystadenocarcinoma from other intrahepatic cystic lesions.     

Multicystic biliary hamartoma mimicking intrahepatic cholangiocarcinoma: report of a case

Multicystic biliary hamartoma (MCBH) is a rare cystic disease of the liver. A 69-year-old man was referred to our hospital with radiographic abnormality. Physical examination of the patient was unremarkable, and he denied any previous medical, travel, or surgical history. An abdominal computed tomography (CT) scan demonstrated a 3-cm low-density lesion in segment 3 of the liver, with dilation of the intrahepatic bile duct. The peripheral site of this lesion was slightly enhanced in the arterial phase. In the portal phase, the peripheral site was enhanced more clearly and showed a honeycomb-like dilated bile duct. Ultrasonography also revealed that the lesion was an irregularly shaped mass. On magnetic resonance imaging (MRI), T1-weighted images revealed a low-density mass and T2-weighted images revealed a dappled-density mass with honeycomb-like dilated bile duct and dilation of major intrahepatic bile duct. The patient was diagnosed with intrahepatic cholangiocarcinoma (ICC) and underwent left hepatectomy. However, pathological findings revealed that the lesion was MCBH. Our case highlights the potential difficulties in differentiating between MCBH and ICC under such circumstances.     

A case of a mucin-producing bile duct tumor diagnosed over the course of 6 years

We report a case of a mucin-producing intraductal papillary neoplasm of the intrahepatic bile duct (M-IPNB) diagnosed over a period of 6 years. A 64-year-old man underwent follow-up evaluations for an abdominal aortic aneurysm at our hospital. In 2009, a computed tomography (CT) scan revealed a simple hepatic cyst in segment 3 of the liver. Annual CT scans initially showed almost no change in the size or shape of the cyst. The cystic lesion, which measured 5 cm in 2014, had increased to 11 cm by 2015, and a solid component was detected within the cyst. A biliary cystic tumor was suspected and we performed a left lateral hepatectomy. Pathological examination showed that the papillary lesion in the cyst included adenocarcinoma and adenoma components. We diagnosed M-IPNB in 2015. Identification of the solid component of the cyst, as well as an increase in cyst diameter in the image analyses, was critical for diagnosis of M-IPNB.     

Intrahepatic Biliary Papillomatosis Arising in Nonobstructive Intrahepatic Biliary Dilatations Confined to the Hepatic Left Lobe

Papillomatosis of intrahepatic bile ducts is a rare pathologic entity. We describe herein a case of papillomatosis of intrahepatic bile ducts associated with nonobstructive dilatations of intrahepatic bile ducts of the hepatic left lobe. The patient, a 70-yr-old female, complained of right upper abdominal pain. Cholangiograms revealed multiple cystic dilatations of the left intrahepatic bile ducts. The right intrahepatic bile ducts were almost normal. The patient underwent left lobectomy of the liver. Grossly, intrahepatic bile ducts of the left lobe were cystically dilated and contained a little mucinous fluid. The inner surface of the dilated ducts was mildly granular or papillary. Microscopically, the walls of the dilated intrahepatic ducts were diffusely covered by columnar epithelial cells with papillary configurations. There was mild atypia of the epithelial cells, but no invasion of the epithelial cells into underlying duct walls was found. To the best of our knowledge, this is the first case of papillomatosis of intrahepatic bile ducts associated with nonobstructive dilatations of intrahepatic bile ducts confined to the left lobe. Although the pathogenesis of intrahepatic biliary papillomatosis of our case is unclear, it seems conceivable that biliary irritation associated with the nonobstructive dilatations of intrahepatic bile ducts is causally related to the papillary proliferations of intrahepatic bile duct epithelial cells.     

Biliary Cystadenoma: An Uncommon Cause of Recurrent Cholestatic Jaundice

The clinical history of a patient with intermittent jaundice is presented. The diagnostic work up, including ultrasound, laparoscopy, and endoscopic retrograde cholangiography, revealed a multiloculated cystic mass in the left lobe of the liver. At laparotomy, a multicystic lesion invading the left hepatic duct and protruding into the common bile duct was found. Histological examination revealed a biliary cystadenoma. Complete excision is the treatment of choice of such lesions.     

Adenosquamous carcinoma of the liver arising in biliary cystadenocarcinoma: clinical, radiologic, and pathologic features with review of the literature

Primary biliary cystadenoma, cystadenocarcinoma, squamous carcinoma, and adenosquamous carcinoma of the liver are rare tumors. We describe a middle-aged woman with recurrent fever and a clinical diagnosis of hepatic abscess who proved at laparotomy to have adenosquamous carcinoma of the liver arising in a biliary cystadenocarcinoma. The pathologic features of the tumor and findings on ultrasonography, angiography, and computed tomography are described in detail. The pathogenetic relationships of this unusual tumor to chronic biliary inflammation, biliary cystadenoma, and cystadenocarcinoma and to bile duct malformations (von Meyenburg complexes) are considered.     

Type II congenital biliary dilation (biliary diverticulum) with pancreaticobiliary maljunction successfully treated by laparoscopic surgery: report of a case

Biliary diverticulum or type II congenital biliary dilation accounts for only 1?C2% of all patients with congenital biliary dilation. The association between pancreaticobiliary maljunction (PBM) and this type of anomaly remains unclear. A 40-year-old Japanese woman presented with a history of repeated upper abdominal pain for more than 30?years. Computed tomography showed a cystic lesion (diameter 30?mm) arising from the common bile duct. Magnetic resonance cholangiopancreatography revealed a biliary diverticulum (diameter 33?mm) and the cystic duct entering the diverticulum. There was no dilation of the intrahepatic or extrahepatic ducts. Endoscopic retrograde cholangiopancreatography demonstrated PBM. A diagnosis of type II biliary diverticulum with PBM was made, and the patient underwent laparoscopic resection of the diverticulum combined with cholecystectomy. Pathological examination of the resected specimen showed slight inflammation, but no malignancy in the diverticulum or gallbladder. The patient??s postoperative course was uneventful. To our knowledge, this is the first report of successful laparoscopic resection of biliary diverticulum associated with pancreaticobiliary maljunction.     

Biliary cystadenoma revealed by obstructive jaundice

Biliary cystadenoma is a rare cystic tumour of the liver that can be difficult to differentiate from other types of benign hepatic cysts. We report the case of a 32-year-old woman who presented with obstructive jaundice due to a large cystic lesion of the left hepatic lobe. Resection of the mass revealed a mucinous cystadenoma with protrusion of a pedunculated extension into the left hepatic duct and the common bile duct. We describe the clinical features, the radiological findings, the surgical management and the pathology of this rare entity.     

Idiopathic benign biliary stricture

A rare case of idiopathic benign biliary stricture is reported. A 50-year-old man with liver dysfunction underwent ultrasonography, which revealed dilation of the intrahepatic bile ducts, and endoscopic retrograde cholangiopancreatography, which revealed a short, ring-like stenosis at the junction of the left and right hepatic ducts. Although a benign stricture was suspected, radiologic tests alone were insufficient to make a firm diagnosis. Therefore, a cholecystectomy and resection of the extrahepatic biliary tract were performed. Microscopic examination of the resected specimen demonstrated no evidence of malignancy. The final diagnosis was mild, localized, chronic cholangitis. The patient had not had previous biliary tract surgery, choledocholithiasis, nor did he have a congenital abnormality of the biliary tract, bile duct carcinoma, or pancreatic disease. Since there was no evidence of primary sclerosing cholangitis, the stricture was considered to be idiopathic.