Hemorrhage from bile duct varices treated with fully covered expandable metallic stent placement in pancreatic carcinoma

A 64-year-old woman with unresectable pancreatic body carcinoma was admitted with epigastralgia with a sudden onset 6 h earlier. She had received chemotherapy for her cancer for 2 months. Physical examination showed mild anemia. Contrast-enhanced computed tomography showed dilated vessels in the bile duct walls connecting with dilated and tortuous vessels around the extrahepatic bile duct and portal vein obstruction due to invasion by a pancreatic body tumor. Endoscopic examination showed transpapillary hemorrhage suggesting bile duct hemorrhage. On endoscopic retrograde cholangiopancreatography, the lower bile duct was filled with a mass and the middle bile duct had filling defects with compression of the wall. To stop the bleeding, we placed a fully covered expandable metallic stent (EMS) at the middle to lower portion of the bile duct, and the hemorrhage stopped. Bile duct hemorrhage is not a common disorder. This report shows bile duct hemorrhage from bile duct varices can occur in patients with pancreatic carcinoma with portal obstruction and that fully covered EMS placement can stop the hemorrhage.     

Curative resection for a pancreatic endocrine carcinoma involving the portal vein

A 68-year-old male was admitted to our hospital for worsening of diabetes mellitus. Abdominal computed tomography showed a well-enhanced tumor in the head of the pancreas. Endoscopic retrograde cholangiopancreatography revealed tapering obstruction of the main pancreatic duct and stricture of the common bile duct. Celiac angiography demonstrated a vague tumor stain. Percutaneous transhepatic portography disclosed stricture of the portal vein. Pylorus-preserving pancreatoduodenectomy with segmental resection and reconstruction of the portal vein was performed under a diagnosis of nonfunctioning endocrine carcinoma of the pancreas. Histological examination demonstrated that the tumor was composed of small nests and cords of cuboidal cells with nodal invasion, and was proven to be nonfunctioning endocrine carcinoma of the pancreas. Microscopically, the tumor invaded the portal vein, common bile duct and main pancreatic duct. The postoperative course was uneventful, and he is now well without any evidence of recurrent disease 14 months after surgery.     

Palliative treatment with metallic stents for unresectable gallbladder carcinoma involving the portal vein and bile duct

We report a case of gallbladder carcinoma associated with biliary obstruction and portal vein stenosis caused by massive lymph node metastases. The patient, a 59‐year‐old woman, was treated with self‐expandable metallic stents — a spiral Z‐stent in the portal vein, and a Wallstent in the bile duct — and intra‐arterial infusion chemotherapy. She returned to work immediately after leaving the hospital, and has been treated with intra‐arterial infusion chemotherapy once a week at our outpatient department. At present, she has good quality of life, with patency of both endoprostheses, 8 months after the placement of the metallic stents in the portal vein and the common bile duct. This case shows that portal vein and biliary stenting, together with intra‐arterial infusion chemotherapy, can be an effective modality for the palliative treatment of advanced gallbladder carcinoma involving the portal vein and bile duct, to improve quality of life.     

Portal vein thrombosis associated with hilar bile duct carcinoma and liver abscess

As most portal vein occlusion in hilar bile duct carcinoma is caused by tumor invasion to the portal vein, other mechanisms of its occlusion are very rare. We report the case of a 69-year-old man who underwent surgical resection for an advanced hilar bile duct carcinoma associated with unusual portal vein occlusion. Preoperative diagnosis was advanced hilar bile duct carcinoma with liver abscess and right portal vein occlusion due to tumor invasion. Extended right hepatectomy combined with resection of caudate lobe was performed. Intraoperatively, tumor invasion to the portal vein was not evident and resected margin of the right portal vein showed thrombosis and no evidence of malignancy histologically. To our knowledge, this is the first reported case of a patient with a combination of portal vein thrombosis and liver abscess in hilar bile duct carcinoma. Although portal vein occlusion due to thrombosis is an unusual complication in hilar bile duct carcinoma, the presence of liver abscess may be a useful diagnostic implication of this occlusion.     

A case of small cell carcinoma of the common bile duct

Small cell carcinoma occasionally occurs in the gastrointestinal tract, but rarely in the biliary tract. We report a case of small cell carcinoma which occurred in the common bile duct. A 66-year-old female complained of epigastralgia and weight loss. Computed tomography and ultrasonography showed a mass near the pancreas head and dilatation of the intrahepatic bile ducts. Endoscopic nasobiliary drainage was undertaken, and it revealed obstruction of the common bile duct. The patient was diagnosed preoperatively as having extrahepatic bile duct cancer. Upon laparotomy, a tumor was found to be located in the middle common bile duct. Pylorus-preserving pancreaticoduodenectomy was performed. The main trunk of the portal vein and the right hepatic artery were resected concomitantly because of tumor involvement. Postoperative pathological examination revealed well-differentiated papillary adenocarcinoma on the surface of the bile duct lumen, but a large part of the extraductal component was small cell carcinoma. Upon immunohistochemical examination, synaptophysin and chromogranin A were found to be focally positive in small cell carcinoma, but negative for L-26 and CEA. The patient then underwent two postoperative courses of systemic chemotherapy. Nevertheless, she died of cancer recurrence eight months after the operation, which showed that the tumor had a highly lethal nature, with rapid and widespread dissemination. Further therapeutic trials are needed to improve survival in such cases.     

Preoperative assessment of extrahepatic bile duct carcinoma using three-dimensional intraductal US.

BACKGROUND: We investigated the utility of a new imaging modality, three-dimensional intraductal ultrasonography (US), for staging bile duct cancer. METHODS: In eight patients with extrahepatic bile duct carcinoma, two- and three-dimensional intraductal US was used to assess tumor invasion of the right hepatic artery, portal vein, and pancreatic parenchyma before resection. The findings were correlated with histologic information from the resected specimen. RESULTS: Three-dimensional intraductal US enabled accurate assessment of tumor invasion of the right hepatic artery in 88% of cases, the portal vein in 100%, and pancreatic parenchyma in 100%. Two-dimensional intraductal US enabled accurate assessment of invasion of these structures in 88%, 88%, and 88% of cases. CONCLUSIONS: Three-dimensional intraductal US is useful in assessing tumor stage in bile duct carcinoma.     

Small duct cholangitis induced byn-formyll-methioninel-leucinel-tyrosine in rats

Primary sclerosing cholangitis (PSC) frequently accompanies inflammatory bowel diseases. In an attempt to increase our understanding of the pathogenesis of PSC, we studied bile duct changes in rats with colitis which had been givenn-formyll-methioninel-leucinel-tyrosine (fMLT) rectally; fMLT is one of the chemotactic peptides produced byEscherichia coli, and is secreted into the bile by hepatocytes after it enters the portal blood. Transrectal administration of fMLT induced a marked inflammation in the portal triad and mild hepatocyte necrosis on the 4th day. The infiltrating leukocytes in the portal tract were mostly mononuclear cells, which densely infiltrated around the bile ducts. These mononuclear cells appeared to attach to bile duct epithelial cells, and they were more numerous in the smaller bile ducts. Electron microscopy revealed that lymphocytes were in direct contact with bile duct lining cells and that some epithelial cells had degenerated or collapsed. These results suggest that thisE. coli-derived peptide may induce cholangitis in the small bile duct through cell-mediated mechanisms. Since these pathologic changes resemble those of the bile duct observed in the early stage of PSC, it can be concluded that bacterial chemotactic peptides may play a role in the pathogenesis of small-duct PSC.     

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.     

A case of retroperitoneal lymphoma presenting as pancreatic tumor

We describe a case of non-Hodgkin's lymphoma arising in the peripancreatic retroperitoneal lymph node and extending into the pancreatic parenchyma. Abdominal ultrasonogram and computed tomography of the abdomen showed an mass in the head of pancreas with absence of extrapancreatic disease and no direct tumor extension to the portal vein or superior mesenteric artery. Laparotomy demonstrated a large pancreatic mass with no obstruction of common bile duct or encasement of portal vein and superior mesenteric artery and a pancreaticoduodenectomy was performed. He received chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone. The patient is alive and in remission with a follow-up time of 24 months.     

Mucin-producing intrahepatic cholangiocarcinoma with portal vein thrombosis

Mucin-producing intrahepatic cholangiocarcinoma is rare. Computed tomographic scan and magnetic resonance imaging showed a well-defined tumor with marked dilatation of the left intrahepatic bile duct and portal vein thrombosis. We performed extended left hepatectomy and portal vein thrombectomy. A massive amount of mucin was observed in the left intrahepatic bile duct. Histological examination revealed noninvasive and well differentiated tubular adenocarcinoma.     

Biliopancreatic fistula with portal vein thrombosis caused by a pancreatic pseudocyst

We encountered a very rare case of biliopancreatic fistula with portal vein thrombosis caused by pancreatic pseudocyst. A 57-year-old man was referred to our hospital because of abdominal pain, obstructive jaundice, and portal vein thrombosis due to acute pancreatitis. Computed tomography showed a 7-cm-diameter pseudocyst around the superior mesenteric vein extending towards the pancreatic head, dilatation of the intrahepatic bile duct, and portal vein thrombosis. Endoscopic retrograde pancreatography revealed a main pancreatic duct with a pseudocyst communicating with the common bile duct. After pancreatic sphincterotomy, a 7-F tube stent was endoscopically placed into the pseudocyst. However, a 6-F nasobiliary tube could not be inserted into the bile duct because the fistula had a tight stenosis. Subsequently, the patient’s abdominal pain improved, the pancreatic cyst disappeared, and the serum amylase level normalized. Two months after the endoscopic retrograde cholangiopancreatography, percutaneous transhepatic biliary drainage was required because the patient’s jaundice became aggravated. Two weeks after the choledochojejunostomy, the patient left the hospital in good condition. A follow-up computed tomography showed cavernous transformation of the portal vein and no pancreatic pseudocyst. The patient remains asymptomatic for 2 years and 7 months after surgery. Biliary drainage may be necessary for biliopancreatic fistula with obstructive jaundice in addition to pancreatic cyst drainage. Biliopancreatic fistula can be treated by endoscopic procedure in some cases; however, surgical treatment should be required in cases that are impossible to insert a biliary stent because of hard stricture.     

Cholestasis as presenting symptom of portal cavernoma

A 51-year-old man with a history of portal vein thrombosis, was examined because of elevated liver tests and a tumoral mass in the liver hilus. Computed tomography (CT) scan and magnetic resonance imaging confirmed the portal vein thrombosis and an infiltrating mass in the porta hepatis with compression on the common bile duct. Endoscopic retrograde cholangiography showed an irregular narrowing of the mid-part of the common bile duct. The patient was referred for explorative laparotomy, which revealed a hypervascular mass in the liver hilus surrounded by many blood vessels. The diagnosis of portal cavernoma was made. Further haematological examination for the cause of portal vein thrombosis revealed an anti-phospholipid syndrome as well as myeloproliferative disease. Oral anticoagulant treatment is started. In conclusion, we report a case of biliary stricture due to portal vein thrombosis and cavernoma (portal biliopathy) which was not diagnosed preoperatively. Biliary strictures associated with portal vein thrombosis are due to extrinsic compression by collaterals and can also be induced by ischemic injury secondary to venous and arterial thrombosis of the choledochal vascular plexus.     

Medical treatment of primary sclerosing cholangitis

Until 1970, primary sclerosing cholangitis (PSC) was considered to be a medical curiosity. With the development of endoscopic cholangiography, PSC is now recognized more frequently and is a common indication for liver transplantation. PSC is usually progressive, leading to cirrhosis, portal hypertension, and liver failure. The manifestations of disease may be clinically similar to those of other causes of bile duct obstruction and must be distinguished from gallstone disease, bile duct carcinoma, primary biliary cirrhosis, and secondary biliary cirrhosis due to bile duct stricture. Medical management of PSC must take into account the likelihood that destroyed bile ducts do not regenerate as hepatocytes do. Hence, PSC should be treated early in its course. The goal of therapy is to prevent further damage and destruction of bile ducts. In this article, we will present relevant data concerning the medical management of primary sclerosing cholangitis.     

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.     

Pancreatoduodenectomy for bile duct and ampullary cancer

Pylorus-preserving pancreatoduodenectomy has become a standard operation for distal and middle bile duct cancers. Bile duct cancer typically extends longitudinally and invades vertically. It frequently metastasizes to the lymph nodes and infiltrates the perineural spaces. The presence of residual cancer in the bile duct stump and lymph node metastases are significant prognostic factors. Negative surgical margins and D2 lymph node dissection are necessary for curative resection. The clinical course after portal vein resection for bile duct cancer with portal vein invasion is better than that of non-resectable bile duct cancer. Portal vein resection can therefore be useful. The efficacy of prophylactic portal vein resection is unclear. We describe here our methods for performing pylorus-preserving pancreatoduodenectomy for bile duct cancer.     

Ventral dissection of replaced right hepatic artery during pancreatoduodenectomy

A novel technique for dissecting a replaced right hepatic artery during pancreatoduodenectomy in patients with middle or lower bile duct carcinoma is presented. After skeletonizing the left, proper, common hepatic arteries and the portal vein, the replaced right hepatic artery is dissected from the ventro-medial side of the hepatoduodenal ligament by severing the thin connective tissue behind the portal vein. Thus the hepatic arteries and the portal vein are completely isolated without the Kocher maneuver, leaving the cancer and the bile duct untouched en bloc with the surrounding lymph nodes and the pancreas head. The periaortic lymph nodes can also be resected en bloc with the main lesion.     

Histological confirmation of periductal fibrosis from ultrasound diagnosis in cholangiocarcinoma patients

Cholangiocarcinoma (CCA) has no specific clinical signs and symptoms and non‐specific bio‐ and tumor‐markers in the early disease stage. Usually patients present to tertiary care with advanced disease stage. In order to detect early cases of CCA that may present as a mass, dilatation of intrahepatic duct or combination, ultrasonography is accepted as a powerful imaging tool. A smaller mass or bile duct segmental dilatation requires further imaging for characterization, including computerized tomography (CT) or magnetic resonance imaging (MRI). We examined whether liver echo pattern was correlated with high risk for CCA in an endemic area of Opisthorchis viverrini (Ov). Ov infestation caused chronic inflammation of the biliary tree by periductal fibrosis (PDF), which may subsequently lead to CCA development. In our study, a World Health Organization classification of pattern of increased periportal echo (IPE) for schistosomiasis was applied. Two CCA patients gave consent for operation. Histopathological diagnosis showed both had cholangiocarcinoma with periductal fibrosis of the non‐tumorous area of the liver. Ultrasonography was used to compare the non‐tumorous area with parenchymal echo pattern and was shown to have an early CCA detection role and a surveillance role in an endemic area of Ov by detection of PDF.     

Primary sclerosing cholangitis in which differential diagnosis from gallbladder carcinoma was difficult

We report a case of localized primary sclerosing cholangitis (PSC) which was difficult to distinguish from gallbladder carcinoma. A 75-year-old woman with elevated serum bilirubin was hospitalized and underwent endoscopic nasobiliary drainage (ENBD). There was no history of diseases such as gallbladder stone, pancreatitis, or ulcerative colitis. Cholangiography through the ENBD tube showed localized stenosis of the common bile duct; the gallbladder could not be seen. Angiography showed no encasement of the hepatic artery. Ultrasonography showed a tumor in the cystic duct, and the tumor had invaded the gallbladder and common bile duct. We diagnosed gallbladder carcinoma on radioimaging, and performed an S4aS5 subsegmentectomy of the liver and resection of the extrahepatic biliary tree. Pathologically, no malignant cells were detected, and fibrosis around bile ducts and infiltration of inflammatory cells into hepatic tissue were found. It is well known that PSC is sometimes difficult to differentially diagnose from cholangiocarcinoma. Our case is of high interest because ultrasonography showed findings suggestive of gallbladder carcinoma. It is therefore necessary to keep the possibility of PSC in mind for the diagnosis and treatment of such localized biliary stenosis.     

A role of mast cells for hepatic fibrosis in primary sclerosing cholangitis.

We encountered four patients with overt primary sclerosing cholangitis (PSC) which were histologically classified into stage 2 or 3. We examined the expression of stem cell factor (SCF), a ligand of c-kit, in injured bile ducts by immunohistochemistry, and mast cells were identified by immunohistochemistry using anti-HMCT (human mast cell tryptase) and anti-c-kit antibodies to clarify their relation with portal fibrosis coincident with destroyed bile ducts. SCF was detected in the epithelia of most bile ducts in PSC, and many HMCT- and c-kit-positive mast cells were found in portal tracts. Image analysis showed more significant numbers of c-kit-positive mast cells per area of portal tract in PSC than in chronic hepatitis C, and they might increase from stage 2 to 3. c-Kit-positive cells infiltrated into the portal tracts with SCF-positive destroyed bile ducts, and c-kit mast cells should be investigated in detail to make a role for portal fibrosis in PSC.     

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.