Follicular lymphoma of the extrahepatic bile duct mimicking cholangiocarcinoma

We report a case of a 33-year old man who presented with symptoms and signs of an acute biliary tract obstruction with jaundice and abdominal pain. Diagnostic imaging studies revealed a biliary stricture of the hepatic confluence, and a mass at the hepatic hilum which obstructed the extrahepatic bile duct from the outside. At laparotomy, there was 3-cm-size nodule at the hilum which presented with a rubbery consistency. We performed extrahepatic bile duct resection and right and left hepaticojejunostomy. Histological examination of the resected specimen revealed follicular lymphoma, which consisted of medium cleaved follicle-like cells, grade 1 of 3 according to the revised European-American classification of lymphoid neoplasms proposed by International Lymphoma Study Group. Postoperative follow-up of more than 1 year has been completely uneventful, without any symptoms or signs of disease recurrence. This is the second case report of follicular lymphoma of the extrahepatic bile duct.     

Familial occurrence of congenital bile duct cysts

Congenital bile duct cysts are now a well-documented anomaly of the biliary tree, and have become more common in Japan. Familial occurrence of congenital bile duct cysts, however, is extremely rare, with only six reported cases in the literature. We report a familial pattern of congenital bile duct cysts in a mother and her daughter. A 33-year-old female was admitted for evaluation of right upper quadrant abdominal pain and fever 6 days after an uneventful delivery of her second child. A com- puted tomography (CT) and ultrasound scan (US) revealed an obstructed biliary tract. Percutaneous transhepatic biliary drainage was then performed, and a cholangiogram revealed a Scholtz type B choledochocele without an anomalous connection of the pancreaticobiliary ducts. Endoscopic US demonstrated that the choledochocele was associated with a stone in the cyst. A pylorus-preserving pancreatoduodenal resection was performed, and a histological study revealed that the choledochocele was lined by biliary mucosa without evidence of malignancy. The newborn infant had an abdominal tumour. An US and CT revealed a congenital bile duct cyst. An operation was performed and the intraoperative cholangiogram showed an Alonso-Lej type I congenital bile duct cyst with an anomalous connection of the pancreaticobiliary ducts. Whether congenital bile duct cysts are hereditary remains to be elucidated.     

Right hepatic lobectomy for bile duct injury associated with major vascular occlusion after laparoscopic cholecystectomy

A 57-year-old woman underwent laparoscopic cholecystectomy (LC) for cholelithiasis. Continuous bile leak was observed beginning on the first postoperative day. Postoperative endoscopic retrograde cholangiography revealed bile leak through the common hepatic duct, and severe stenosis of the hepatic confluence. A total of three percutaneous transhepatic biliary drainage (PTBD) catheters were inserted to treat obstructive jaundice and cholangitis. The patient was referred to our hospital for surgery 118 days after LC. Cholangiography through the PTBD catheters demonstrated a hilar biliary obstruction. Celiac arteriography revealed obstruction of the right hepatic artery, and transarterial portography showed occlusion of the right anterior portal branch. On the basis of the cholangiographic and angiographic findings, we performed a right hepatic lobectomy with hepaticojejunostomy to resolve the bile duct obstruction and address the problem of major vascular occlusion. The patient's postoperative recovery was uneventful and she remains well 25 months after hepatectomy. We discuss a treatment strategy for bile duct injury suspected after LC, involving early investigation of the biliary tree and prompt intervention.     

Ectopic hepatocellular carcinoma arising in the bile duct

We report a case of ectopic hepatocellular carcinoma arising in the bile duct. A 72‐year‐old woman was transferred to our hospital with fever, abdominal pain, and jaundice. Contrast‐enhanced computed tomography revealed a round mass, measuring 25 mm in diameter, in the bile duct. The mass was causing obstructive jaundice. Endoscopic retrograde cholangiography showed a 27 mm × 21‐mm round defect in the superior bile duct. These findings led to a diagnosis of bile duct tumor, and the patient underwent extrahepatic bile duct resection and biliary reconstruction. Gross examination of the tumor showed a fibrous capsule and a stalk arising from the bile duct mucosa. The tumor was diagnosed histopathologically as well‐differentiated hepatocellular carcinoma arising in the bile duct.     

Metachronous double cancer of the gallbladder and common bile duct

We report a rare case of metachronous double cancer of the biliary tract. At age 59 years, a man had undergone a cholecystectomy and resection of the liver bed for gallbladder cancer pathologically diagnosed as papillary adenocarcinoma, in 1997. Four years later, he was admitted to our hospital with jaundice. At first, we suspected lymph node metastasis of the gallbladder cancer along the common bile duct. But abdominal computed tomography demonstrated circular wall thickness of the common bile duct, so primary bile duct cancer was strongly suspected. Thus, extended right hepatectomy and pancreaticoduodenectomy were performed after right portal vein embolization. The pathological diagnosis of the resected specimen was well-differentiated tubular adenocarcinoma, and this case was clarified to be metachronous double cancer. A review of the literature regarding double cancer of the biliary tract is presented following this case report. We showed that half of 30 cases of double cancer of the biliary tract were not associated with pancreaticobiliary maljunction, including all 6 metachronous cases.     

Primary malignant peripheral nerve-sheath tumor of the common bile duct

Primary malignant peripheral nerve-sheath tumors of the common bile duct are extremely rare. To our knowledge, the published literature contains no previous case report of this disease. Here we report on a 58-year-old Japanese woman with a primary malignant peripheral nerve-sheath tumor of the common bile duct, which was completely resected. A hypoechoic mass was identified in the hepatic hilus, using ultrasonography and computed tomography. Endoscopic retrograde cholangiography revealed a smooth stricture and deviation of the common bile duct. Laparotomy exposed a firm mass around the common bile duct that had not invaded the surrounding tissues. Partial resection of the common bile duct and cholecystectomy were performed as the treatment of choice. The final histopathological diagnosis was malignant peripheral nerve-sheath tumor arising from the wall of the common bile duct.     

Long-term prognosis of patients with endoscopically treated postoperative bile duct stricture and bile duct stricture due to chronic pancreatitis

Aim:  To compare the outcome of endoscopic therapy for postoperative benign bile duct stricture and benign bile duct stricture due to chronic pancreatitis, including long-term prognosis.
Methods:  The subjects were 20 patients with postoperative benign bile duct stricture and 13 patients with bile duct stricture due to chronic pancreatitis who were 2 years or more after initial therapy. The patients underwent transpapillary drainage with tube exchange every 3 to 6 months until being free from the tube. Successful therapy was defined as a stent-free condition without hepatic disorder.
Results:  Endoscopic therapy was successful in 90% (18/20) of the patients with postoperative bile duct stricture. The stent was removed (stent free) in 100% (20/20) of the patients, but jaundice resolved in only 10% (2/20) of patients while biliary enzymes kept increasing. Restricture occurred in 5% (1/20) of the patients, but after repeat treatment the stent could be removed. In patients with bile duct stricture due to chronic pancreatitis the therapy was successful in only 7.7% (1/13) of the patients; the stent was retained in 92.3% (12/13) of the patients during a long period. Severe acute pancreatitis occurred in 3.0% (1/33) of the patients as an accidental symptom attributable to endoscopic retrograde cholangiopancreatography (ERCP); however, it remitted after conservative treatment.
Conclusion:  Our results further confirm the usefulness of endoscopic therapy for postoperative benign bile duct strictures and good long-term prognosis of the patients.     

Peripheral portal vein-oriented non-dilated bile duct puncture for percutaneous transhepatic biliary drainage

AIM: To evaluate the efficacy of peripheral portal vein(PV)-oriented non-dilated bile duct(BD) puncture for percutaneous transhepatic biliary drainage(PTBD).METHODS: Thirty-five patients with non-dilated BDs underwent PTBD for the management of various biliary disorders, including benign bilioenteric anastomotic stricture(n = 24), BD stricture(n = 5) associated with iatrogenic BD injury, and postoperative biliary leakage(n = 6). Under ultrasonographic guidance, percutaneous transhepatic puncture using a 21-G needle was performed along the running course of the peripheral targeted non-dilated BD(preferably B6 for right-sided approach, and B3 for left-sided approach) or along the accompanying PV when the BD was not well visualized. This technique could provide an appropriate insertion angle of less than 30° between the puncture needle and BD running course. The puncture needle was then advanced slightly beyond the accompanying PV. The needle tip was moved slightly backward while injecting a small amount of contrast agent to obtain the BD image, followed by insertion of a 0.018-inch guide wire(GW). A drainage catheter was then placed usinga two-step GW method. RESULTS: PTBD was successful in 33(94.3%) of the 35 patients with non-dilated intrahepatic BDs. A rightsided approach was performed in 25 cases, while a left-sided approach was performed in 10 cases. In 31 patients, the first PTBD attempt proved successful. Four cases required a second attempt a few days later to place a drainage catheter. PTBD was successful in two cases, but the second attempt also failed in the other two cases, probably due to poor breath-holding ability. Although most patients(n = 26) had been experiencing cholangitis with fever(including septic condition in 8 cases) before PTBD, only 5(14.3%) patients encountered PTBD procedure-related complications, such as transient hemobilia and cholangitis. No major complications such as bilioarterial fistula or portal thrombosis were observed. There was no mortality in our series.CONCLUSION: Peripheral PV-oriented BD puncture for PTBD in patients with non-dilated BDs is a safe and effective procedure for BD stricture and postoperative bile leakage.     

Early bile duct cancer detected by direct peroral cholangioscopy with narrow-band imaging after bile duct stone removal

Cholangioscopy not only enables the direct visualization of the biliary tree, but also allows for forceps biopsy to diagnosis early cholangiocarcinoma. Recently, some reports have suggested the clinical usefulness of direct peroral cholangioscopy (POC) using an ultra-slim endoscope with a standard endoscopic unit by a single operator. Enhanced endoscopy, such as narrow band imaging (NBI), can be helpful for detecting early neoplasia in the gastrointestinal tract and is easily applicable during direct POC. A 63-year-old woman with acute cholangitis had persistent bile duct dilation on the left hepatic duct after common bile duct stone removal and clinical improvement. We performed direct POC with NBI using an ultra-slim upper endoscope to examine the strictured segment. NBI examination showed an irregular surface and polypoid structure with tumor vessels. Target biopsy under direct endoscopic visualization was performed, and adenocarcinoma was documented. The patient underwent an extended left hepatectomy, and the resected specimen showed early bile duct cancer confined to the ductal mucosa.     

Magnetic resonance cholangiographic assessment of a delayed radiation-induced bile duct stricture

Radiation-induced bile duct strictures are rare since bile ducts are considered to be resistant in radiation injury. We report a case of bile duct stenosis where evidence is presented that bile duct stricture was the result of radiation injury and which illustrates the major contribution of magnetic res-onance cholangiography in biliary tract disease evaluation.     

Metachronous bile duct cancer nine years after resection of gallbladder cancer

We report a rare case of a 74-year-old man with metachronous gallbladder cancer and bile duct cancer who underwent curative resection twice, with the operations nine years apart. At the age of 65 years, the patient underwent a cholecystectomy and resection of the liver bed for gallbladder cancer. This was a welldifferentiated adenocarcinoma, with negative resection margins （T2NOM0, stage Ⅰ B）. Nine years later, during a follow-up examination, abdominal computed tomography and MRCP showed an enhanced 1.7 cm mass in the hilum that extended to the second branch of the right intrahepatic bile duct. We diagnosed this lesion as a perihilar bile duct cancer, Bismuth type Ⅲ a, and performed bile duct excision, right hepatic Iobectomy and Roux-en-Y hepaticojejunostomy. The histological diagnosis was a well-differentiated adenocarcinoma with one regional lymph node metastasis （TINIM0, stage Ⅱ B）. Twelve months after the second operation, the patient is well, with no signs of recurrence. This case is compared with 11 other cases of metachronous biliary tract cancer published in the world medical literature.     

Intraductal papillary neoplasm of the bile duct extending superficially from the intrahepatic to extrahepatic bile duct

Intraductal papillary neoplasm of the bile duct (IPNB) or liver is a recently noted rare disease, and its pathogenesis remains unclear. Here we present a case of IPNB with an interesting morphology, which was treated by resection of the right hemiliver and extrahepatic bile duct. A 79-year-old woman was found to have a high alkaline phosphatase level and slight dilatation of the right intrahepatic bile duct on imaging studies. The right intrahepatic bile duct became dilated over a 2-year period; however, no solid mass could be detected, and tumor markers were not elevated. Hepatic resection was scheduled because a mucin-producing bile duct carcinoma of the liver was suspected. A right hemihepatectomy was conducted, and the extrahepatic bile duct was also resected after malignant cells were found in the surgical stump of the right bile duct and in the bile itself. Macroscopically, diffuse dilatation of the intrahepatic bile duct was noted, but no solid component or mucin within the duct was found. Histopathological findings revealed carcinoma in situ, IPNB, in the majority of intrahepatic bile ducts, with no lymph node metastasis, and it extended continuously to the epithelium of the common bile duct. No tumor recurrence or biliary dilatation was observed at follow-up 2 years after surgery. It is important to consider malignancy in the presence of a dilated bile duct and in the absence of any cause of occlusion. Complete resection of IPNB results in a good prognosis and no recurrence.     

Value of peroral cholangioscopy for mucin-producing bile duct tumor

Mucin-producing bile duct tumor is a rare biliary tract tumor. Despite the development of modern diagnostic technologies such as ultrasonography, and angiography, the precise determination of this tumor is difficult because of ambiguity caused by the abundant mucin secreted by the tumor and/or by the superficial mucosal spread of the tumor along the bile duct. Given these problems, selective percutaneous transhepatic biliary drainage (PTBD) and percutaneous transhepatic cholangioscopy (PTCS) are indispensable for the accuracy staging of this cancer. But, PTCS has a risk of a rare but serous complications, seeding metastasis at the sinus tract of PTBD, whereas the retrograde approach minimizes this risk. We report the case of a patient who underwent the successful resection of a mucin-producing bile duct tumor. The tumor was diagnosed preoperatively as originating in the left caudate lobe after ERC and peroral cholangioscopy (POCS).     

Insemination of bile duct carcinoma to the liver after insertion of percutaneous biliary endoprosthesis

Percutaneous transhepatic biliary drainage is widely used to relieve bile duct obstruction which can be caused by bile duct or pancreas carcinomas. Although the incidence is low, insemination of carcinoma along the transhepatic catheter tract is considered to be a serious complication of percutaneous transhepatic biliary drainage. The authors present a case of intrahepatic insemination of bile duct carcinoma along the catheter that subsequently underwent a curative resection consisting of pancreaticoduodenectomy and right hepatic lobectomy. It is suggested that a percutaneous biliary endoprosthesis through the tumor should be avoided in patients in whom a possible curative resection can be considered. External biliary drainage should only be performed in order to minimize the manipuIation of the tumor in such patients.     

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.     

Double cancers of the gallbladder and bile duct associated with anomalous choledochopancreatic duct junction

A rare case of double cancers of the gallbladder and bile duct associated with anomalous choledo-chopancreatic duct junction (ACPDJ) is reported. The patient was a 61-year-old Japanese woman who with presented right upper quadrant abdominal pain. Liver function tests results were normal. Computed tomography showed a polypoid lesion in the gallbladder, and endoscopic retrograde cholangiopancreatography (ERCP) demonstrated ACPDJ and irregular wall of the inferior bile duct. A diagnosis of double cancers of the gallbladder and bile duct was made and a pancreaticoduodenectomy and liver bed resection was performed. His topathological examination showed papillary adenocarcinoma of the gallbladder and mucosal adenocarcinoma of the bile duct. The patient is in good health 15 months after the operation and shows no signs of recurrence. A review of the literature is presented.     

Choledochocele associated with superficial spreading cancer with cholesterolosis of the bile duct

A 74-year-old woman, who had suffered from acute cholangitis, was referred to our department for further evaluation of the biliary tree. A diagnosis of choledochocele was made by endoscopic ultrasonography and endoscopic retrograde cholangiopancreatography. A small flat elevation with cholesterolosis was observed in the lower bile duct, and circumferential wall thickening was apparent in some parts of the upper and middle bile duct by intraductal ultrasonography and peroral cholangioscopy. With the diagnosis of choledochocele associated with superficial spreading cancer and cholesterolosis of the bile duct, pylorus-preserving pancreaticoduodenectomy was carried out. Microscopically, the tumor had spread extensively from the lower to the upper bile duct. Its invasion was limited to the mucosa for the most part, with microinvasion of the subserosa in the upper bile duct. In the lower bile duct, foamy cells were located beneath the neoplastic epithelium of the elevation. The number of cases of choledochocele associated with biliary cancer is becoming higher than previously reported. This anomaly may play some role in the development of biliary malignancy. Histological examination seems to be mandatory before making a diagnosis of cholesterolosis of the bile duct, since this condition may occasionally accompany cancer.     

Double common bile duct： A case report

Double common bile duct （DCBD） is a rare congenital anomaly in which two common bile ducts exist. One usually has normal drainage into the papilla duodeni major and the other usually named accessory common bile duct （ACBD） opens in different parts of upper gastrointestinal tract （stomach, duodenum, ductus pancreaticus or septum）. This anomaly is of great importance since it is often associated with biliary lithiasis, choledochal cyst, anomalous pancreaticobiliary junction （APBJ） and upper gastrointestinal tract malignancies. We recently recognized a rare case of DCBD associated with APB3 with lithiasis in better developed common bile duct. The opening site of ACBD was in the pancreatic duct. The anomaly was suspected by transabdominal ultrasonography and finally confirmed by endoscopic retrograde cholangiopancreatography （ERCP） followed by endoscopic sphincterotomy and stone extraction. According to the literature, the existence of DCBD with the opening of ACBD in the pancreatic duct is most frequently associated with APB3 and gallbladder carcinoma. In case of DCBD, the opening site of ACBD is of greatest clinical importance because of its close implications with concomitant pathology. The adequate diagnosis of this rare anomaly is significant since the operative complications may occur in cases with DCBD which is not recognized prior to surgical treatment.     

Synchronous double cancers of the common bile duct

We report an extremely rare case of synchronous double cancers of the common bile duct without pancreaticobiliary maljunction. Only two similar cases have been reported in the English literature. Endoscopic re-trograde cholangiopancreatography showed a tuberous filling defect in the middle and superior parts of the common bile duct, and mild stenosis in the inferior duct. Computed tomography (CT) showed a well enhanced mass in the middle and superior parts of the common bile duct. A single cancer of the middle and superior bile duct was suspected and extra-hepatic bile duct resection was performed. CT eleven months after surgery revealed enhanced inferior bile duct wall and a slightly enhanced tumor within it. Retrospective review of the CT images taken before first surgery showed enhanced inferior bile duct wall without intrabiliary tumor only on the delayed phase. The inferior bile duct tumor was suspected to have originally co-existed with the middle and superior bile duct tumor. Pancreaticoduodenectomy was performed subsequently. Histopathological examination revealed that the middle and superior bile duct tumor was a moderately differentiated tubular adenocarcinoma while the inferior bile duct tumor was a papillary adenocarcinoma. The two tumors were separated and had different histological findings and growth patterns, further suggesting that they were two primary cancers.     

多种内镜检查方法联合应用对胆管狭窄性疾病的诊断价值

目的评估多种内镜检查方法联合应用对胆管狭窄性疾病的诊疗价值。方法回顾性分析36例胆管狭窄性疾病患者的诊断情况。36例患者均进行了超声内镜检查术（EUS）、经内镜逆行胰胆管造影术（ERCP）、胆管内超声检查术（IDUS）,胆道靶向刷检行细胞学涂片、液基薄层细胞学检查,并结合临床资料及组织学病理检查,综合诊断。结果最终诊断胆管恶性病变21例,其中胆管细胞癌9例、十二指肠乳头癌4例、胰腺癌侵犯胆总管4例、肝癌侵犯胆总管4例;胆管良性病变15例,其中胆总管结石9例、肝吸虫感染所致胆管狭窄4例、单纯胆管炎性狭窄1例、外部压迫所致胆管狭窄1例。EUS、ERCP、IDUS及ERCP＋IDUS对胆管狭窄性疾病鉴别诊断的准确率分别为77．8％、88．9％、91．7％、94．4％,ERCP、IDUS及ERCP＋IDUS均明显高于EUS（P均〈0．05）;ERCP＋IDUS对胆管狭窄性疾病鉴别诊断的敏感度、特异度、阳性预测值与阴性预测值分别为95．2％、93．3％、95．2％、93．3％,均高于EUS、ERCP及IDUS单独检查。胆道刷检细胞学、液基薄层细胞学或组织病理学检查,19例诊断为恶性狭窄,17例诊断为良性狭窄,对鉴别胆管狭窄性质诊断的敏感度为90．5％、特异度为100．0％、准确率为94．4％。结论对于胆管狭窄性病变,ERCP＋IDUS可使诊断准确率得到明显提高;联合应用ERCP＋IDUS＋病变胆管的靶向刷检等多种内镜检查方法,诊断准确率更高。