1/9. Villous adenoma of the bile ducts: a case report and a review of the reported cases in korea.Villous adenomas are benign epithelial lesions with malignant potential which can occur at any site in the gastrointestinal tract. They are usually encountered in the rectum and colon, less frequently in the small bowel and very rarely in the biliary trees. Nine cases of bile duct villous adenomas have been reported in the literature. However, 4 cases of bile duct villous adenomas have been reported in the Korean literature. Recently, we experienced a case of villous adenoma in the common hepatic duct in a 77-year-old man presenting with obstructive jaundice in which preoperative histologic diagnosis of villous adenoma played a critical role in managing this patient. Herein, we present a case report of bile duct villous adenoma and a review of the reported cases in korea to help define and manage this rare disease entity in the bile ducts. In addition, confusing nomenclature of bile duct adenomas is discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/9. Undifferentiated spindle cell carcinoma of the extrahepatic bile ducts: a case report.Spindle cell carcinoma is a rare tumor that generally occurs in the upper digestive tract. We report an 81-year-old man with spindle cell carcinoma located in the extrahepatic bile ducts, resulting in obstructive jaundice. The patient died 10 months after operation due to local recurrence. The literature on this rare disease is reviewed and discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/9. Clinical features and imaging diagnosis of biliary cystadenocarcinoma of the liver.Biliary cystadenocarcinoma of the liver is a relatively rare disease. Herein, we reported a case of biliary cystadenocarcinoma with a review of the literature. A 71-year-old female was admitted with the chief complaint of epigastralgia. The imaging studies revealed a biliary cystadenocarcinoma in the left hepatic lobe with suspicion of direct invasion to the left and middle hepatic veins and inferior vena cava. However, there was no direct invasion of the tumor to these veins in operation findings, and an extended left hepatic resection was performed without resection of inferior vena cava. The tumor was histologically diagnosed as biliary cystadenocarcinoma of the liver. diagnosis of biliary cystadenocarcinoma is usually difficult preoperatively, however, a diagnosis was possible with the use of imaging studies. It was suggested that this tumor originated from a benign cystadenoma because of the existence of a transitional zone between normal cells and atypical cells in the cystic wall. Systematic hepatectomy was recommended as the initial treatment in consideration of the features of cystadenocarcinoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/9. Embolization of proper hepatic artery pseudoaneurysm complicating choledochoscopic laser therapy.Biliary papillomatosis is a rare disease characterized by multiple papillary proliferation of the epithelial cell of the bile duct. Because it has a tendency to be recurrent, the treatment strategy is choledochoscopic laser therapy. A patient with biliary papillomatosis treated by choledochoscopic laser therapy, which was complicated by massive haemobilia and shock, is presented. An intrahepatic artery pseudoaneurysm was diagnosed on angiography. A coexisting occlusion necessitated a superselective embolization of the pseudoaneurysm in order to avoid devascularization of the left lobe of the liver.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/9. Papillomatosis confined to the distal biliary tract--a rare cause of obstructive jaundice: report of a case.Based on the findings of a patient with recurrent obstructive jaundice due to papillomatosis of the distal bile duct, we herein describe the diagnostic difficulties and therapeutic options in this very rare disease. Endoscopic retrograde cholangiopancreatography and, in particular, cholangioscopy are the imaging procedures of choice if biliary papillomatosis is suspected. Due to the tendency of such patients to demonstrate malignant transformation and develop biliary cirrhosis with septic complications, an early and radical surgical resection is recommended in rare cases of localized papillomatosis. This approach may offer the only chance of a cure although the potential risk of multifocal recurrence cannot be ruled out. When considering a radical resection, intraoperative cholangioscopy is strongly recommended to confirm any localized papillomatosis and rule out any diffuse papillomatosis of the entire biliary tract.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/9. Intrahepatic sarcomatoid cholangiocarcinoma.A 69-year-old woman was admitted to our hospital with fever and abdominal pain in the epigastric region. Abdominal ultrasonography demonstrated a well-defined hypoechoic mass in the epigastric region with encasement of the left hepatic lobe and stomach. Computed tomography confirmed a low-density mass, 20 cm in diameter, with enhancing peripheral areas. Angiography revealed the tumor to be hypovascular. After admission, the patient had a persistent fever and anemia that required transfusions of concentrated red blood cells. On the twelfth day after admission, she suffered disseminated intravascular coagulation and underwent an emergency operation. A lateral segmentectomy with dissection of lymph nodes, cholecystectomy, and hemigastrectomy were carried out. The size of the tumor was 22 x 17 x 15 cm. Macroscopically, a cross-section revealed massive necrosis with hemorrhage. Histological examination of the tumor showed a malignant neoplasm with a carcinomatous component and a sarcomatous component, which were partly intermingled. The former consisted of moderately differentiated adenocarcinoma, while the latter consisted of pleomorphic spindle cells. Immunohistochemical examination of the sarcomatous component showed positive staining for vimentin, epithelial membrane antigen, and cytokeratin. The tumor was diagnosed as cholangiocarcinoma with extensive sarcomatous changes, based on these histological and immunohistochemical findings. The patient had an uneventful postoperative course. However, she died 3 months after surgery from dissemination of the carcinoma. The literature on this rare disease is reviewed and discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/9. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/9. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/9. A case of spindle cell sarcomatous change of hepatic ducts manifesting as obstructive jaundice.Spindle cell carcinoma is a rare tumor commonly occurring in the upper aerodigestive tract. We report a 62-year-old male with spindle cell sarcomatous change located at the hepatic hilum, resulting in obstructive jaundice. The patient died after an extended resective operation. The rare disease and its histogenesis is discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |