A case of benign schwannoma in the porta hepatis]

Schwannomas or neurilemmomas are uncommon tumors arising from schwann cells of neural sheath, which most frequently affect the extremities, trunk, head and neck area. Moreover, benign schwannoma in the porta hepatis is extremely rare. Approximately, 2 cases of benign schwannoma in the porta hepatis have been reported in the literatures. We report a case of benign schwannoma in the porta hepatis occurring in a 53-year-old woman who was asymptomatic and incidentally found to have a mass. Abdominal CT scan showed a 4.5 cm-sized low attenuating mass with septum-like enhancing solid portion at porta hepatis. Abdominal exploration revealed a yellowish, cystic and encapsulated mass attached to portal vein, common bile duct, liver and duodenal wall above the hepatoduodenal ligament. There has been no evidence of recurrence during 11 months of follow-up.     

Mucosa-associated lymphoid tissue-type lymphoproliferative lesion of the common bile duct

Only a few cases of primary lymphoma of the common bile duct have been reported up to now. Here, we report a mucosa-associated lymphoid tissue (MALT)-type lymphoproliferative lesion of the common bile duct with some considerations about MALT lymphoma and benign lymphoproliferation. The patient, a 71-year-old woman, was admitted to our hospital because of progressive epigastric pain. Abdominal ultrasonography, magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography, and abdominal computed tomography showed a non-specific finding of stenosis of the common bile duct due to thickening of the wall. Angiography provided little diagnostic information. Bile duct carcinoma was diagnosed and pylorus-preserving pancreaticoduodenectomy was performed. Histological examination revealed that the common bile duct was occupied by full-thickness proliferation of lymphoplasma cells with many reactive lymph follicles. The histological features were somewhat consistent with those of MALT lymphoma. However, we could not determine the clonal proliferation of lymphoplasma cells in either immunohistochemical immunoglobulin light chain restriction of the plasma cells or in polymerase chain reaction based immunoglobulin heavy chain gene rearrangement. The present case may be categorized as a borderline lesion close to benign lymphoproliferation. To the best of our knowledge, there have been no reports of cases similar to the present case.     

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.     

Central pancreatectomy for pancreatic schwannoma: A case report and literature review

Schwannomas are mesenchymal tumors originating from Schwann cells in peripheral nerve sheaths. Although the tumor can be located in any part of the human body, the most common locations are the head, neck, trunk and extremities. Pancreaticschwannomas are rare. To our knowledge, only 64 cases of pancreatic schwannoma have been reported in the English literature over the past 40 years. In this paper, we present a pancreatic schwannoma in a 59-year-old female. Ultrasound, computed tomography and magnetic resonance imaging revealed the tumor located in the pancreatic body; however, accurate diagnosis was hard to obtain preoperatively and a pancreatic cystadenoma was preliminarily considered. During laparotomy, the mass was found in the body of the pancreas. An enlarged gallbladder with multiple stones was also observed. We performed central pancreatectomy, end-to-side pancreaticojejunostomy and cholecystectomy. Notably, central pancreatectomy has been reported in only one case prior to this report. The gross specimen showed a mass with a thin capsule, 1.6 cm × 1.1 cm × 1.1 cm in size. Microscopic examination showed that the tumor was mainly composed of spindle-shaped cells with palisading arrangement and no atypia, which is consistent with a benign tumor. Both hypercellular and hypocellular areas were visible. Immunohistochemical staining revealed strongly positive results for protein S-100. Finally, the tumor was diagnosed as a schwannoma of the pancreatic body. Postoperatively, the patient recovered well and left the hospital 6 d later. During the 53-mo follow-up period, the patient remained well and free of complications.     

Signet ring cell carcinoma of the bile duct: a case report

A 72-year-old man was admitted with obstructive jaundice. Computed tomography revealed a 4cm tumor with multiple cystic components obstructing the common bile duct. Endoscopic ultrasonography, endoscopic retrograde cholangiopancreatography and intraductal ultrasonography demonstrated the tumor, which derived from the lower bile duct, grew into the bile duct lumen. Peroral cholangioscopy revealed distended tumor vessels on the surface of the tumor. Signet ring cell carcinoma of the bile duct was diagnosed by biopsy. The patient died 3 months after the first hospital admission despite chemotherapy.     

CASE REPORT: Extrahepatic Biliary Schwannoma

Schwannoma (neurilemoma or neurinoma) rarely develops in the biliary tract. We report here a case of extrahepatic biliary schwannoma found in a 47-yr-old Japanese woman presenting with obstructive jaundice. The radiological imaging studies were suggestive of nonepithelial tumor involving the common bile duct. The patient underwent tumor resection. The tumor extended inward and outward from the wall of the common bile duct in the shape of a dumbbell. The extraductal tumor was solid with microcystic changes, while the intraductal lesion presented cystic changes. Microscopically, the tumor was predominantly composed of spindle-shaped cells with nuclear palisading, and it contained lymphoid aggregates. Immunohistochemically, the tumor cells were positive for S-100 protein. The final diagnosis was benign schwannoma of the common bile duct. The tumor differed from usual soft tissue schwannoma and closely resembled gastrointestinal schwannoma.     

Percutaneous transhepatic insertion of a permanent endoprosthesis in obstructive lesions of the extrahepatic bile ducts

A Teflon endoprosthesis for permanent bile drainage was inserted in 13 patients following percutaneous transhepatic puncture and catheterization of the bile duct system. Twelve patients had extrahepatic cholestasis because of a malignant tumor, whereas one patient had chronic inflammation involving the hepatoduodenal ligament (secondary to Crohn's disease) with obstruction of the extrahepatic bile ducts. The drainage periods varied from 1 week to 8 months. The endoprosthesis was regarded as partially effective in seven patients whereas in six cases the drainage through the endoprosthesis was insufficient and external bile drainage through a percutaneous transhepatic catheter was necessary. Infection of the bile duct system during the drainage period with a percutaneous transhepatic catheter and/or bile duct endoprosthesis occurred in 10 patients. Spontaneous dislocation of the endoprosthesis occurred in varying degrees in five patients. One patient developed an intrahepatic aneurysm adjacent to the puncture tract and died because of liver insufficiency following therapeutic embolization of the aneurysm and most of the hepatic arteries by injection of gelfoam particles into the common hepatic artery. Patients in whom palliative treatment by insertion of a permanent bile duct endoprosthesis may be suitable were defined.     

A CASE OF CHOLESTEROSIS OF THE COMMON BILE DUCT DIAGNOSED BY PERORAL CHOLANGIOSCOPY

We experienced a case of cholesterosis of the common bile duct in which peroral cholangioscopy (POCS) was very useful for making a diagnosis. The patient was a 66‐year‐old man. He was admitted for examination of an asymptomatic mass in the pancreas. The mass was diagnosed as a serous cystic tumor of the pancreas. While examining the tumor, endoscopic retrograde cholangiopancreatography (ERCP) incidentally revealed a 7 mm irregular filling defect in the distal common bile duct. EST was then performed, followed by POCS, which revealed a clump of yellowish‐white Ip or Isp polyps in the distal bile duct. Histological examination of biopsy specimens taken under direct vision gave a diagnosis of cholesterol polyps containing foamy cells. POCS was useful for allowing direct observation of the characteristic color of cholesterol polyps, and for obtaining biopsy specimens under direct vision.     

Absence of the common bile duct and junction of the cystic duct with the left hepatic duct in a patient with chronic portal vein thrombosis

A50-year-old male patient was admitted to the hospital because of fatigue and a palpable abdominal mass. The diagnoses of chronic renal failure and portal vein thrombosis were established by specific investigations. Incidentally, junction of the cystic duct with the left hepatic duct and absence of the common bile duct were found during endoscopic retrograde cholangiopancreatography study. To the authors' knowledge, this is the first reported case of an absence of the common bile duct and junction of the cystic duct with the left hepatic duct in a patient with chronic portal vein thrombosis. Such lesions may cause unexpected complications during biliary surgery.     

Should the bile duct be preserved or removed in radical surgery for gallbladder cancer?

BACKGROUND/AIMS: It is unclear whether resection of the extrahepatic bile duct in radical surgery for gallbladder cancer should be performed when direct infiltration into the hepatoduodenal ligament is absent. METHODOLOGY: The results of radical surgery with or without bile duct resection were compared in 55 patients with gallbladder cancer without direct extension to the hepatoduodenal ligament. Lymph node dissection and combined resection of involved organs were carried out according to the extent of the tumor. RESULTS: Nodal involvement was present in 43% of patients with tumors more advanced than pT1. Survival rates were similar between patients with or without bile duct resection in stages I-III, while significantly better survival was observed with bile duct resection in stage IV. CONCLUSIONS: Considering the adverse effect of bilioenteric anastomosis, preservation of the extrahepatic bile duct is recommended in radical surgery for gallbladder cancer when the tumor is less advanced than stage IV and does not extend to the hepatoduodenal ligament.     

Gallbladder carcinoma with choledochoduodenal fistula: a case report with surgical treatment.

A 79 year-old man was admitted to our hospital because of upper abdominal pain and nausea. A mobile tumor was palpable in the right upper abdomen. Abdominal ultrasonography, computed tomography and celiac angiography revealed a gallbladder tumor. Endoscopic retrograde cholangiopancreatography revealed a fistula 1.5 cm oral to the orifice of the papilla of Vater, dilatation of the common bile duct, and a filling defect in the gallbladder. Pancreatoduodenectomy associated with reconstruction using Imanaga's method was performed under a pre-operative diagnosis of gallbladder carcinoma with choledochoduodenal fistula. The gallbladder contained a tumor and two bilirubin stones impacted in the orifice of the duodenal papilla. Histological studies confirmed that the gallbladder tumor was a mucinous adenocarcinoma and had not infiltrated the bile duct. We speculated that choledochoduodenal fistula stimulated the development of cancer due to chronic irritation from pancreatic juice reflux.     

True left-sided gallbladder with variations of bile duct and cholecystic vein

A left-sided gallbladder without a right-sided round ligament, which is called a true left-sided gallbladder, is extremely rare. A 71-year-old woman was referred to our hospital due to a gallbladder polyp. Computed tomography (CT) revealed not only a gallbladder polyp but also the gallbladder located to the left of the round ligament connected to the left umbilical portion. CT portography revealed that the main portal vein diverged into the right posterior portal vein and the common trunk of the left portal vein and right anterior portal vein. CT cholangiography revealed that the infraportal bile duct of segment 2 joined the common bile duct. Laparoscopic cholecystectomy was performed for a gallbladder polyp, and the intraoperative finding showed that the cholecystic veins joined the round ligament. A true left-sided gallbladder is closely associated with several anomalies; therefore, surgeons encountering a true left-sided gallbladder should be aware of the potential for these anomalies.     

Extended right hepatic lobectomy with resection of inferior vena cava and portal vein for intrahepatic cholangiocarcinoma

We report a case of intrahepatic cholangiocarcinoma treated by extended right lobectomy and resection of the inferior vena cava (IVC) and portal vein. A 53-year-old man was referred with elevated serum alkaline phosphatase (ALP) and γ-glutamyl transpeptidase (γ-GTP) levels on April 23, 1999. He was not jaundiced and did not have any symptoms. Endoscopic retrograde cholangiopancreatography (ERCP) revealed irregular strictures in both the anterior and posterior segmental ducts. Contrast-enhanced computed tomography (CT) scan demonstrated a low-density tumor with an unclear margin in the right lobe of the liver. The patient underwent extended right hepatic lobectomy and total caudate lobectomy. Partial resection of the IVC (6 cm) was performed under total hepatic vascular exclusion. The main portal trunk and left portal vein were resected and reconstructed with an end-to-end anastomosis. Macroscopically, a 5.0 × 5.0 × 4.5-cm periductal infiltrating-type tumor occupied the right hepatic parenchyma along the posterior and anterior segmental ducts. Histological examination revealed moderately differentiated tubular adenocarcinoma with marked perineural invasion. Lymph node metastasis was observed in the hepatoduodenal ligament and posterior surface of the pancreatic head. The resected margins of the common bile duct and left hepatic duct were free of tumor. The patient's postoperative course was uneventful, and he was discharged from hospital on the 28th postoperative day. Nine months after the operation, he suddenly developed obstructive jaundice, and died with recurrent disease. This is the first reported case of intrahepatic cholangiocarcinoma treated with major hepatectomy and resection of the IVC and portal vein except ex situ procedure. This aggressive surgical approach may offer hope for patients with intrahepatic cholangiocarcinoma involving the IVC.     

Curative resection of a huge bile duct cancer without pancreatoduodenectomy

A 71-year-old woman was admitted to our hospital with obstructive jaundice. Magnetic resonance cholangiography revealed a 9-cm spindle-shaped tumor in the common bile duct. Laboratory examination noted a high concentration of total serum bilirubin. Although the serum concentration of carcinoembryonic antigen was within normal limits, the serum concentration of carbohydrate antigen 19-9 was elevated. We diagnosed the lesion as an extrahepatic bile duct cancer and performed a laparotomy. The anterior wall of the common bile duct was incised, and the tumor was found to have a small base in only the posterior wall of the duct, and spread within the duct was from the common hepatic duct to the intrapancreatic bile duct. Bile duct resection, cholecystectomy with lymphadenectomy and hepaticojejunostomy were performed. Frozen sections of the proximal and distal surgical margins of the bile duct were free of cancer. Macroscopically, the lesion was an expansive polypoid tumor measuring 9x3cm in diameter arising from a 5-mm base. Histologic examination revealed that the tumor was a well-differentiated tubular adenocarcinoma that had infiltrated the fibromuscular layer at its base. Longitudinal spread was more extensive hepatopetally than hepatofugally. The patient is alive and well without any complaints 8 months after surgery.     

Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst

A 56-year-old woman who had undergone excision of the gallbladder because of a choledochal cyst had a tumorous lesion of the pancreas identified by upper abdominal ultrasonography, but an operation was not carried out, because there was no apparent increase in the cystic mass and no elevation of serum tumor markers. In October 2001, she was admitted to our hospital to check for malignancy because of elevated levels of the tumor marker Dupan-2. Abdominal enhanced computed tomography and upper abdominal ultrasonography revealed a large multilocular cystic mass in the body to tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed elongation of the common duct that communicates with the common bile duct and the main pancreatic duct, indicating an anomalous arrangement of the biliary and pancreatic duct system. No apparent communications between the cystic mass and the main pancreatic duct were observed. In January 2002, the patient underwent a spleen-preserving distal pancreatectomy, and histopathological and immunohistochemical examinations led to the diagnosis of pancreatic mucinous cystadenoma with ovarian-like stroma. The mucinous cystadenoma was detected 17 years after the operation for the choledochal cyst. To the best of our knowledge, no documented case reports of mucinous cystadenoma of the pancreas associated with a choledocal cyst have been reported to date. We present here the first case report of pancreatic mucinous cystadenoma occurring in the body to tail of the pancreas, associated with a choledocal cyst.     

Lymphatic spread differs according to tumor location in extrahepatic bile duct cancer

BACKGROUND/AIMS: Identification of nodal involvement according to primary tumor location in extrahepatic bile duct carcinoma may guide surgical therapy. METHODOLOGY: Pathologic data of 81 patients who underwent curative operation for bile duct carcinoma were studied to clarify the differences in lymphatic spread from distal bile duct carcinoma, middle bile duct carcinoma, and proximal bile duct carcinoma. RESULTS: Lymph node metastases were present in 25 of 41 patients (61%) with distal bile duct carcinoma, 9 of 19 (47%) with middle bile duct carcinoma, and 11 of 21 (52%) with proximal bile duct carcinoma. The number of positive nodes per node-positive patient was greater in patients with middle bile duct carcinoma than in those with distal- or proximal bile duct carcinoma (mean 5.33 vs. 3.56 or 2.64, p < 0.05). Lymph nodes in the hepatoduodenal ligament were most frequently involved regardless of the primary tumor location. The frequency of distal- and middle bile duct carcinoma patients with metastasis to the superior mesenteric or para-aortic nodes was significantly higher than that of proximal bile duct carcinoma patients (p < 0.05 and p < 0.05). CONCLUSIONS: Patterns of lymphatic spread were different according to primary tumor location in bile duct carcinoma. Metastatic nodes were spread widely, from the hepatoduodenal ligament or posterior pancreaticoduodenal region to the nodes around the superior mesenteric artery and abdominal aorta, in distal- and middle bile duct carcinoma.     

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.     

THE ROLE OF PERORAL CHOLANGIOSCOPY FOR BILE DUCT LESIONS

We reviewed the current status of peroral cholangioscopy under duodenoscopic assistance, which allows direct visualization of the bile duct. Direct visual assessment may be a useful adjunct to endoscopic retrograde cholangiopancreatography (ERCP) for distinguishing malignant from benign bile duct lesions. Several clinical studies suggest the efficacy of peroral cholangioscopy for various bile duct lesions. However, solely cholangioscopic examination may be insufficient to confirm accurate differential diagnosis between benign and malignant strictures or tumor extension of bile duct carcinoma. Directed tissue acquisition in biliary strictures by using peroral cholangioscopy is another application that has not been properly studied because of the limited maneuverability of the long babyscope. Further improvement of suitable instruments and cholangioscopes are needed.     

A case of abscess caused by a penetrating duodenal ulcer.

A case of abscess caused by a penetrating duodenal ulcer in a 34 year-old female patient is presented. She had a past history of duodenal ulcer and presented with a low grade fever which had persisted for 1 month. Abdominal ultrasound confirmed a hypoechoic mass and computed tomography revealed a low density area in the posterior side of the hepatoduodenal ligament. The common bile duct and portal vein were compressed. Mild peripheral enhancement was detected. Laparotomy was performed and an abscess in the posterior side of the hepatoduodenal ligament was confirmed. The abscess was firmly adhered to the lesser curvature side of the bulbus and a penetrating duodenal ulcer scar was noted. In conclusion, this report describes a rare event where penetrating duodenal ulcer formed an abscess with only mild complaints.     

Biliary tuberculosis mimicking cholangiocarcinoma: treatment with metallic biliary endoprothesis

A 58-yr-old patient who presented with obstructive jaundice was evaluated with ultrasonography (US), computed tomography (CT), and percutaneous transhepatic cholangiography (PTC). Diffuse irregular stenosis of the extrahepatic bile ducts and periductal ill-defined soft tissue density along the hepatoduodenal ligament was determined. The patient was originally misdiagnosed with cholangiocarcinoma and, because the extent of disease process made surgical bypass impossible, was treated with a percutaneously inserted metallic stent. Histopathological examination of the endoluminal biopsy revealed ductal tuberculosis (TB). Most of the previous reports in the literature indicated that biliary obstruction was due to enlarged tuberculous lymph nodes compressing the bile duct. To our knowledge, only three cases of biliary stricture due to tuberculous involvement of the bile ducts were reported previously. This case illustrates the importance of tissue diagnosis in all cases of obstructive jaundice to avoid missing rare but curable diseases.