Hilar cholangiocarcinoma associated with sarcoid reaction in the regional lymph nodes

A 74-year-old woman was admitted to our hospital with a 2-week history of jaundice. Percutaneous transhepatic cholangioscopy revealed a nodular tumor originating in the upper part of the common hepatic duct, which was invading the confluence of the right and left hepatic ducts. Microscopic examination of biopsy specimens revealed adenocarcinoma. Abdominal ultrasonography and computed tomography demonstrated multiple enlarged lymph nodes around the extrahepatic bile duct and the common hepatic artery. Laparotomy revealed lymph node enlargement in the hepatoduodenal ligament, behind the pancreatic head, and along the common hepatic and left gastric arteries. Extended left hepatic lobectomy, caudate lobectomy, and resection of extrahepatic bile duct with extended lymph node dissection were performed. The histology of permanent specimen revealed no tumor metastasis but a sarcoid reaction in the lymph nodes. The patient is in good health 21 months after the operation, without any evidence of recurrence. This is the first successfully resected case of hilar cholangiocarcinoma associated with sarcoid reaction in the regional lymph nodes.     

Mucosal bile duct carcinoma with superficial spread

We describe a case of mucosal bile duct carcinoma with superficial spread in a 69-year-old man with gallstone pancreatitis. The patient was seen at the hospital because of abdominal pain, fever, and jaundice. Endoscopic retrograde cholangiography (ERC) demonstrated a protruding lesion in the lower third of the common bile duct (CBD) showing wall irregularity suggestive of malignancy. Percutaneous transhepatic cholangioscopy (PTCS) disclosed a papillary tumor with granular mucosa extending continuously to the middle third of the CBD. Cholangioscopic biopsy specimens taken from both the papillary tumor and surrounding granular mucosa revealed papillary adenocarcinoma. After this assessment of extent of cancer by PTCS, we performed pancreatoduodenectomy with extrahepatic bile duct resection and regional lymph node dissection. Pathology examination revealed papillary adenocarcinoma limited to the mucosal layer. The resected margin of the bile duct was free of tumor. We also reviewed 25 cases of early mucosal bile duct carcinoma described in detail in the Japanese literature, and we discuss the diagnostic advantages of PTCS.     

Advanced gallbladder carcinoma with biliobiliary fistula, resected by hepatopancreatoduodenectomy, in an aged patient

We report a 78-year-old man with a gallbladder carcinoma and biliobiliary fistula, diagnosed by percutaneous transhepatic cholangioscopic biopsy through the fistula. The impacted stones in the common hepatic duct were crushed, and then selective cholangiography under percutaneous transhepatic cholangioscopy (PTCS) revealed a biliobiliary fistula. Cholangioscopic biopsy tissues taken from the gallbladder revealed adenocarcinoma, but biopsies taken from the fistula revealed no evidence of malignancy. Further investigations indicated that the gallbladder carcinoma involved the duodenum and the distal common bile duct. A hepatopancreatoduodenectomy, including both an extended right hepatic lobectomy with resection of the caudate lobe and a pancreatoduodenectomy, was performed. Despite the patient's advanced age, he made an unremarkable postoperative recovery and was able to enjoy an active social life for 8 months after the surgery. We discuss biliobiliary fistula associated with gallbladder carcinomas and the use of hepatopancreatoduodenectomy for advanced biliary cancer in aged patients.     

Recurrent liver tumor with intrabiliary ductal growth after hepatectomy for metastatic rectal cancer: Case report

A case of recurrent tumor with intrabiliary ductal growth after hepatic resection for liver metastasis from rectal cancer is presented. The patient, a 55-year-old female, underwent subsegmentectomy of the anteroinferior and posteroinferior areas of the liver for metastatic liver cancer on August 29, 1988. Computed tomography in February 1990 showed dilatation of the intrahepatic bile duct in the right anterosuperior subsegment (B8), in which a filling defect was detected by cholangiography through a percutaneous transhepatic biliary drainage (PTBD) catheter. Percutaneous transhepatic cholangioscopy (PTCS) revealed a protruding lesion without tumor vessels. Cholangioscopic biopsy revealed dysplasia, but not adenocarcinoma. However, recurrent tumor originating in the resected margin of the remnant liver was suspected, and resection of the right lobe of the liver and partial resection of the duodenum were therefore performed. The resected specimen showed a tumor, 4 cm in diameter, in the previous resected margin, forming a protruding lesion with a rough surface (measuring 10×20 mm) in the B8 bile duct. This case suggested the possibility of cancer recurrence in the resected margin of the liver after hepatectomy for metastatic colorectal cancer, with intrabiliary ductal tumor growth showing segmental biliary dilatation.     

Large-cell neuroendocrine carcinoma of the distal bile duct

Large-cell neuroendocrine carcinoma (LCNEC) in the distal bile duct is very rare and different from common distal bile duct adenocarcinoma. A 77-year-old man was admitted with obstructive jaundice. Severe stenosis of the distal bile duct was revealed by percutaneous transhepatic cholangiography. Subtotal stomach-preserving pancreaticoduodenectomy was performed. A tumor measuring 1.8 cm in diameter was located in the distal bile duct. Both histopathological and immunohistochemical examination of the resected specimen revealed features of LCNEC of the bile duct. The patient developed multiple liver metastases, lung metastases, and local recurrence and died of disease 3 months after the operation. The clinical behavior of LCNEC in the distal bile duct appears to be highly aggressive with early metastases and a fatal outcome.     

Recurrent carcinoma of cystic duct remnant with subcutaneous implantation in abdominal wall

A case of recurrent carcinoma of the cystic duct remnant invading the common bile duct and portal vein with subcutaneous implantation of the abdominal wall is presented. The patient was a 55-year-old woman with an abdominal wall tumor at the site of the surgical scar of a cholecystectomy, performed at a local hospital 5 years ago for symptomatic cholelithiasis. The diagnosis was made by incisional biopsy of the tumor, computed tomography, percutaneous transhepatic cholangiography, and angiography. She underwent extended right hepatic lobectomy with en bloc resection of the caudate lobe, extrahepatic bile duct, and portal vein. The abdominal wall tumor was resected concomitantly. Histological examination showed that both the recurrent carcinoma of the cystic duct remnant and the abdominal wall implantation were moderately differentiated adenocarcinoma. This recurrence probably could have been prevented if both the macroscopic and microscopic examinations of the resected specimen had been precisely carried out after the previous cholecystectomy and the primary carcinoma identified and treated at that time.     

Villous tumor of the papilla of Vater presenting with hypopotassemia

We report a case of villous tumor of the papilla of Vater associated with hypopotassemia. The patient was a 73-year-old woman who presented with jaundice and fever. She had a history of diabetes mellitus and liver dysfunction. Laboratory studies revealed that levels of total bilirubin, alkaline phosphatase, and C-reactive protein, and the white blood cell count were elevated (suggestive of cholangitis) and that the serum potassium level was markedly reduced, to 1.9 mEq/l (normal value 3.5–5.0 mEq/l). Duodenoscopy showed a villous tumor arising in the papilla of Vater. Percutaneous transhepatic biliary drainage was performed. Approximately 700–1500 ml of bile with viscous mucoid fluid was drained daily. Percutaneous transhepatic cholangioscopy showed a papillary lesion in the distal common bile duct. Biopsied specimens from both percutaneous transhepatic cholangioscopy and duodenoscopy disclosed tubulovillous adenoma. Endoscopic ultrasonography showed that the tumor had spread to the main pancreatic duct as well as to the common bile duct. The patient underwent pylorus-preserving pancreaticoduodenectomy. Pathology examination disclosed well differentiated adenocarcinoma, carcinoma in situ, in tubulovillous adenoma. The cancer cells were observed at the bottom of the tumor spreading in the common bile duct. This is a rare case of a patient presenting with hypopotassemia associated with a tubulovillous tumor of the papilla of Vater that secreted mucoid material.     

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).     

Staging cholangiocarcinoma by cholangioscopy

Peroral cholangioscopy (POCS) and percutaneous transhepatic cholangioscopy (PTCS) were first developed in the 1970s, and technical developments and clinical applications have taken place gradually ever since. POCS is used to diagnose small mucosal biliary lesions in non-icteric patients and early malignant changes in patients with persistent primary sclerosing cholangitis (PSC). Although PTCS is a more invasive diagnostic procedure than POCS, it has the advantage of precise diagnosis with mapping biopsy in defining the proximal and distal extension of superficially spreading cholangiocarcinoma (CCA) or mucin-producing CCA, which is predominantly found in papillary type CCA. POCS is significantly superior to ERCP in distinguishing between malignant and benign dominant bile duct stenoses in patients with PSC. The positive rate of PTCS biopsy for CCA is 96%, while morbidity and mortality of PTCS are 9% and 0%, respectively. Although magnetic resonance (MR) cholangiography may replace PTCS in determining the longitudinal spread of infiltrating type hilar CCA, the accuracy of MR cholangiography in papillary type hilar CCA is significantly lower than that of PTCS.     

Implantation metastasis along the percutaneous transhepatic biliary drainage sinus tract

We describe herein the case of a 75-year-old man with metastatic tumor seeding at the percutaneous transhepatic biliary drainage tract that occurred following a pylorus-preserving pancreatoduodenectomy for carcinoma of the distal common bile duct. On postoperative day 30, the catheter was removed and ethanol was injected into the percutaneous transhepatic biliary drainage sinus tract to prevent cancer implantation. One year and 3 months after the initial operation, abdominal computed tomography showed dilation of the left lateral segmental bile ducts and a 2-cm mass. The location of this mass corresponded to the puncture point from the previously performed percutaneous transhepatic biliary drainage. Implantation of the bile duct carcinoma at the percutaneous transhepatic biliary drainage sinus tract was diagnosed, and the recurrent tumor was successfully resected by performing a left hepatic lobectomy. Currently, 1 year after the second operation, the patient is in good health without any signs of recurrence. This case report demonstrates the importance of resecting the percutaneous transhepatic biliary drainage sinus tract during the initial surgery. If left in place, careful follow-up and awareness of this mode of tumor recurrence may lead to a timely resection, with preservation of a good quality of life and long-term survival.     

An unusual growth pattern of a mucin-producing intrahepatic cholangiocellular carcinoma involving the hepatic hilus.

The case of a mucin-producing intrahepatic cholangiocellular carcinoma in a 73 year-old-man is presented. A tumor originating in the right posterior inferior segment of the liver was found to be invading the right posterior and anterior bile ducts, and the hepatic hilus. Extensive superficial spread was observed in the entire posterior segmental bile duct extending to the hepatic hilus. Mucin produced and excreted by the tumor was retained in the common hepatic and common bile duct. The diagnosis in this case was suggested by percutaneous transhepatic aspiration of mucinous bile, and was confirmed by utilizing the techniques of ultrasonography, percutaneous transhepatic cholangiography, computed tomography and angiography. Curative surgery, which included right hepatic lobectomy with total caudate lobectomy and bile duct resection, was performed. Biliary continuity was maintained by left hepaticojejunostomy using a Roux-en-Y jejunal loop. The histological diagnosis was mucin-producing papillary adenocarcinoma originating in the right posterior inferior segment of the liver. Postoperative recovery was very good and the patient has now been enjoying a good active social life for the last 20 months with no signs of tumor recurrence. This case report discusses the unusual growth pattern of a mucin-producing intrahepatic cholangiocellular carcinoma involving the hepatic hilus, and suggests rational surgical treatment.     

Anomalous junction of the pancreaticobiliary duct accompanied by gallbladder cancer and obstructive jaundice in a patient with high serum and bile cytokine levels

We report a case of anomalous junction of the pan creaticobiliary duct (AJPBD) associated with gallbladder cancer and obstructive jaundice in a patient with high serum and bile cytokine levels. The patient was a 63-year-old woman who complained of right hypochondralgia. Ultrasound, computed tomography, percutaneous transhepatic cholangiography, and endoscopic retrograde cholangio-pancreatatography revealed dilation of the bile ducts, an elevated lesion of the gallbladder, and AJPBD. She underwent percutaneous transhepatic cholangio-drainage (PTCD) for obstructive jaundice. However, the total bilirubin concentration remained high 7 days after PTCD. Her serum interleukin 6 level was 57 359 pg/ml before PTCD, and gradually decreased to 10 pg/ml after PTCD. Bile interleukin 6 level was 10 pg/ml before PTCD, 8997 pg/ml 3 h after PTCD and gradually decreased there after. Serum and bile levels of tumor necrosis factor α and hepatocyte growth factor were high before and after PTCD. The patient underwent an extended cholecystectomy and resection of the extrahepatic bile duct. The resected specimen showed two elevated lesions of the gallbladder which, microscopically, revealed moderately differentiated tubular adenocarcinoma. These findings suggest that pre-existing inconspicuous inflammation of the biliary tract due to reflux of pancreatic juice is involved in elevation of serum and bile cytokines, and that cytokines may participate in gallbladder carcinogenesis associated with AJPBD. (Received July 24, 1997; accepted Jan. 23, 1998)     

Rupture of the hepatic artery after left hemihepatectomy for hilar bile duct carcinoma

Hilar bile duct carcinoma has a poor prognosis, but this has been improved in recent years by an aggressive surgical approach. We treated a 73-year-old woman who had obstructive jaundice due to bile duct carcinoma at the hepatic hilum. The jaundice decreased after percutaneous transhepatic biliary drainage. The tumor was resected with the left and caudate lobe of the liver and a part of portal vein. The right hepatic artery was located behind the common hepatic duct, and was suspected to be invaded by the tumor. We dissected the tumor from the arterial wall without carrying out combined resection of the hepatic artery. On the 6th postoperative day, the hepatic artery ruptured and the patient suffered hypovolemic shock. Resection of the hepatic artery and reconstruction were done, but the patient died 2 days later. Histological examination of the resected artery showed that the tumor had been curatively removed by dissection and that no tumor remained at the arterial wall. The rupture of the right hepatic artery was thought to have been caused by damage to the wall during the dissection procedure.     

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.     

Pancreatoduodenectomy after hepato-biliary resection for recurrent metastatic rectal carcinoma

Intrapancreatic bile duct metastasis from rectal carcinoma is rare. A 48-year-old man underwent extended left hepatic lobectomy and caudate lobectomy with extrahepatic bile duct resection for liver metastasis from a rectal carcinoma presenting with intrabiliary growth. A second recurrent tumor was successfully resected by pancreatoduodenectomy without injury to the jejunal loop for biliary reconstruction. Preservation of the previous bilio-enteric anastomosis was critical. Placing the jejunal limb of the hepaticojejunostomy through the retrogastric route was superior to placement through the common retrocolic and anteduodenal route, because the mesentery of the Roux-en Y jejunal limb did not obscure the pancreatic head. Histologic examination revealed a recurrent tumor growing into the remnant intrapancreatic bile duct. This suggested two possibilities: spontaneous shedding of cancer cells from the proximal metastasis, and implantation as a complication of percutaneous transhepatic biliary drainage. In both these circumstances, the metastatic lesion is not systemic, but is a local disease. An aggressive surgical approach for localized recurrence of this type may improve survival.     

Resection of intrahepatic bile duct carcinoma with hilar bile duct and portal vein invasion--a case report

A case of resected intrahepatic bile duct cancer with hilar bile duct and portal vein invasion is presented. Percutaneous transhepatic biliary drainage was performed to alleviate jaundice and evaluate the biliary system. Intraductal tumor extension was determined, and an accurate histological diagnosis was made in biopsy material obtained under percutaneous transhepatic cholangioscopy. Preoperative surgical planning was carried out on the basis of an evaluation of the findings of ultrasonography, computed tomography, arteriography, portography and percutaneous transhepatic cholangioscopy. Curative surgery, which included right hepatic lobectomy with total caudate lobectomy and combined resection and reconstruction of the portal vein, was performed. Bilioenteric continuity was re-established by a Roux-en-Y jejunal loop. The histological diagnosis was moderately differentiated tubular adenocarcinoma originated in the right posterior branch of the intrahepatic bile duct. Postoperative recovery was very good, and the patient has now been enjoying a good active social life for the past three years with no signs of tumor recurrence. This case report discusses the accurate diagnosis and rational surgical treatment for intrahepatic bile duct carcinoma with hilar invasion.     

Carcinoma of the hepatic hilus with superficial spreading to the intrahepatic segmental bile ducts: Report of a case

A case of superficially-spreading carcinoma of the hepatic hilus is presented. Percutaneous transhepatic biliary drainage was performed to alleviate jaundice and to evaluate the biliary system. A nodular tumor originating in the upper part of the common hepatic duct was found to be invading the confluence of the right and left hepatic ducts. Extensive superficial spread was observed in the proximal portion of the right anterior superior, right anterior inferior, right posterior superior, right posterior inferior, and caudate bile duct branches. Preoperative surgical planning was carried out on the basis of an evaluation of the findings of ultrasonography, computed tomography, percutaneous transhepatic cholangiography, and percutaneous transhepatic cholangioscopy. Absolute curative surgery, which included right hepatic lobectomy with total caudate lobectomy and bile duct resection, was performed. Bilioenteric continuity was reestablished with a Roux-en-Y jejunal loop. The histological diagnosis was well-differentiated tubular adenocarcinoma of the common hepatic duct. Postoperative recovery was very good; the patient has now enjoyed a good active social life for the past 4 years and 10 months, with no signs of recurrence. In this case report, we discuss the precise preoperative diagnosis and rational surgical treatment for carcinoma of the hepatic hilus with superficial spread.     

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.     

Synchronous cancer of the biliary tract and pancreas associated with anomalous arrangement of the pancreaticobiliary ductal system.

A 58-year-old man on abdominal ultrasonography and CT had an irregularly elevated lesion at the neck of the gallbladder and a cyst of approximately 6.5 cm in diameter at the pancreatic tail. Percutaneous transhepatic cholangiography revealed a 2-cm shadow defect at the neck of the gallbladder and an irregular, translucent 30 x 12 mm lesion in the intrapancreatic bile duct. Total pancreatectomy and extended cholecystectomy with regional lymph node dissection was performed. An anomalous arrangement of the pancreaticobiliary ductal system (AAPBD) was demonstrated by postoperative contrast radiography of resected specimen. The lesions of the gallbladder and common bile duct were papillary adenocarcinoma. In addition, papillary adenocarcinoma was limited almost entirely to the mucosal layer of the main pancreatic duct and its branches, from the junction of the common bile duct and pancreatic duct to the pancreatic tail. The three tumors were not continuous. The cyst at the pancreatic tail was a pseudocyst. This case represents synchronous cancer of the gallbladder, common bile duct, and pancreas associated with AAPBD.     

A case of adenocarcinoma in situ of the distal common bile duct diagnosed by percutaneous transhepatic cholangioscopy

Extrahepatic cholangiocarcinoma is often clinically challenging to diagnose. Even multidisciplinary approaches which include computed tomography, magnetic resonance imaging, and endoscopic retrograde cholangiography are unsatisfactory in some cases, especially with biliary stricture. Percutaneous transhepatic cholangioscopy (PTCS) with its direct visualization for biopsy appears to be a promising technique for detecting cholangiocarcinoma at an early stage. We report a case of adenocarcinoma in situ of the distal common bile duct (CBD) that was confirmed by PTCS. This case suggests the useful role of PTCS in the differential diagnosis of a distal CBD obstruction, particularly when other diagnostic modalities do not provide definitive information.