Endoscopic retrograde cholangiopancreatography (ERCP) has played an important role in diagnosis and management of biliary disease. Traditionally, duodenal perforations, rare complication of ERCP, have been managed surgically. However, in the past decade, there were arguments for surgical and nonsurgical management of ERCP-related duodenal perforations and reports of successful conservative treatment were increasing especially in patients with old age and poor medical conditions. We experienced a case of type I duodenal perforation following ERCP treated with covered metal stent in 74 years old female with duodenal invasion by Klatskin tumor.
Aged ; Cholangiopancreatography, Endoscopic Retrograde ; Diagnosis ; Female ; Humans ; Klatskin's Tumor ; Stents*

Agenesis of the right lobe of the liver is a rare anomaly which may be associated with stone diseases, portal hypertension, and other congenital anomalies. The radiological differential diagnosis includes lobar atrophy due to cirrhosis or hilar cholangiocarcinoma. We present a case of agenesis of the right lobe of the liver with characteristic clinical and radiological findings.
Atrophy ; Diagnosis, Differential ; Fibrosis ; Hypertension, Portal ; Klatskin Tumor ; Liver*

Agenesis of the right lobe of the liver is a rare anomaly which may be associated with stone diseases, portal hypertension, and other congenital anomalies. The radiological differential diagnosis includes lobar atrophy due to cirrhosis or hilar cholangiocarcinoma. We present a case of agenesis of the right lobe of the liver with characteristic clinical and radiological findings.
Atrophy ; Diagnosis, Differential ; Fibrosis ; Hypertension, Portal ; Klatskin Tumor ; Liver*

Emphysematous gastritis is a rare disease characterized by air in the wall of the stomach due to gas-forming microorganisms. This lethal entity was first described by Fraenkel in 1889. Forty-six cases have been reported so far in the literature. The predisposing factors include corrosive ingestion, immunocompromized state, alcohol abuse, cancer, diabetes and so on. Computed tomography is the procedure of choice for early diagnosis. Only prompt diagnosis and treatment can avoid mortality. Initial antibiotic therapy should cover gram-negative organisms and anaerobes. We report a case of emphysematous gastritis in a 41-year-old man with Klatskin's tumor.
Adult ; Alcoholism ; Causality ; Diagnosis ; Early Diagnosis ; Eating ; Gastritis* ; Humans ; Klatskin's Tumor* ; Mortality ; Rare Diseases ; Stomach

IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection.
Aged ; Bile Ducts/pathology/surgery ; Cholangitis/blood/*diagnosis ; Diagnosis, Differential ; Humans ; Immunoglobulin G/*blood ; Klatskin Tumor/blood/*diagnosis ; Liver/pathology/surgery ; Male

Primary biliary mucosa-associated lymphoid tissue (MALT) lymphoma is extremely rare. We report a case of primary biliary MALT lymphoma with obstructive jaundice diagnosed by endoscopic biopsy, without surgical intervention. Obstructive jaundice was relieved by endoscopic drainage and endoscopic biopsy was done simultaneously during endoscopic retrograde cholangiopancreatography. Unnecessary surgical intervention can be avoided after pathological confirmation of lymphoma. The patient received radiotherapy, and is alive without any evidence of recurrence or biliary obstruction. Diagnosis of primary biliary lymphoma is very difficult because of its low prevalence. However, it should always be considered as a differential diagnosis, since when an accurate diagnosis is made, unnecessary surgical intervention can be avoided.
Biopsy ; Cholangiocarcinoma ; Cholangiopancreatography, Endoscopic Retrograde ; Diagnosis ; Diagnosis, Differential ; Drainage ; Humans ; Jaundice, Obstructive ; Klatskin Tumor* ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Prevalence ; Radiotherapy ; Recurrence

The results of surgery on Klatskin tumors have improved during the era of the combined liver resection. However, some surgeons still have a negative point of view on liver resection because of the locally infiltrative characteristics of cancer and the high mortality and morbidity. We treated 98 patients with a Klatskin tumor between 1990 and 1996. The mean age was 57 year, and the sex ratio (M : F) was 2.2 : 1. Among them, 27 patients (27.6%) received a combined liver resection, 11 patients (11.2%) received a segmental resection of the extrahepatic bile duct, and the other 60 patients (61.2%) had non resective procedures (a bypass operation, an intubational operation, or a percutaneous drainage procedure). The mean survival of the combined liver resection group was 30.0 months-higher than those of the other groups (17.1 months for the segmental-resection group, 14.0 months for the non resective procedures group)(p<0.05). The cumulative 1-, 2-, and 3-year survival rates in patients undergoing a combined liver resection were 95.5%, 83.9%, and 55.9% respectively (significantly higher than the 72.7%, 11.4%, and 0% in the patients undergoing a resection and the 41.1%, 11.4%, and 5.7% in the patients undergoing a non resective procedures). There was no difference in the survival rates between the segmental-resection and non resective-procedure group. In the segmental-resection group, 5 patients had a negative resection margin, and 6 patients had a positive resection margin. The mean survivals were 18.4 and 16.0 months, respectively (p>0.05). The morbidity rate for the combined liver resection was 74%--higher than that for the segmental resection (45%) and for the non resective-procedure group (30%)(p<0.05). There was one operative mortality (1.0%), the patient died due to postoperative sepsis after a U-tube intubational operation. Based on a univariate analysis, significant prognostic factors after liver resection were lymph node involvement, and bilirubin (>5 mg/dl) at admission. A multivariate analysis showed no prognostic significance for either lymph node involvement or bilirubin at admission. These results indicate that segmental resection is not a curable treatment modality and that only aggressive radical surgery, including a hepatectomy, gives any chance of a cure for a Klatskin tumor. We conclude that combined radical liver resection is the treatment of choice for a Klatskin tumor and that accurate preoperative diagnosis and cautious perioperative care decrease the mortality and morbidity of a hepatectomy.
Bile Ducts, Extrahepatic ; Bilirubin ; Diagnosis ; Drainage ; Hepatectomy ; Humans ; Klatskin's Tumor* ; Liver ; Lymph Nodes ; Mortality ; Multivariate Analysis ; Perioperative Care ; Sepsis ; Sex Ratio ; Survival Rate

PURPOSE: The purpose of this study was 1) to describe the thin section helical CT findings of hilar cholangiocarcinoma and of benign stricture, and to discuss the differential points between the two disease entities and 2) using cholangiographic correlation, to evaluate the diagnostic accuracy of helical CT in determining the extent of hilar cholangiocarcinoma. MATERIALS AND METHODS: Twenty-seven patients with hilar cholangiocarcinoma and eight with benign biliary dilatation were studied. All except four with hilar cholangiocarcinoma, who underwent CT using a conventional scanner, were studied with two-phase helical CT. In all patients, cholangiographs were obtained by digital fluoroscopy after the injection of contrast materials into PTBD catheters. The level of obstruction was classified according to Bismuth, and 35 CT scans were studied blindly and retrospectively by two radiologists. The findings were analyzed for the presence of tumor, and then divided into two groups (cholangiocarcinomas and benign strictures), and the positive predictive value was calculated. The CT images of klatskin tumor were analyzed with special emphasis on the level and shape of the hilar obstruction. The level of biliary obstruction and extent of the tumor were carefully correlated with the results of cholangiography. RESULTS: Thin-section spiral CT correctly identified all tumor mass as a focal wall thickening obliterating the lumen. On arterial/portal phase CT scanning, 81% of infilterative tumors showed high attenuation. In all patients, differentiation between benign stricture and klatskin tumor was possible ; correct identification of the level of obstruction and extent of tumor, according to Bismuth's classification, was possible in 63% of cases. CONCLUSION: For correct diagnosis of hilar cholangiocarcinoma and differentiation of benign stricture, helical CT was highly accurate and effective. Because of limital Z-axis resolution, however, the exact intraductalextent of the tumor was less accorately diagnosed.
Bismuth ; Catheters ; Cholangiocarcinoma ; Cholangiography ; Classification ; Constriction, Pathologic* ; Contrast Media ; Diagnosis ; Dilatation ; Fluoroscopy ; Humans ; Klatskin's Tumor* ; Retrospective Studies ; Tomography, Spiral Computed* ; Tomography, X-Ray Computed

Undifferentiated sarcoma is an uncommon primary malignant tumor of the liver typically occurring in older children. It is also referred to as malignant mesenchymoma, fibromyxosarcoma, or mesenchymal sarcoma. We experienced a case of undifferentiated sarcoma in 72-year-old male. Contrast enhanced liver CT scan revealed a 3.4 cm heterogeneously enhancing, ill-defined, and low attenuated mass in the left liver and subtle intrahepatic duct dilatation. And, in tubogram, there were segmental stenosis and occlusion from the hilum to the proximal common bile duct. We did ultrasonography guided liver biopsy. The pathologic finding revealed infiltrative growth of atypical cells with rhabdoid features. Some atypical cells showed clear cytoplasm, but no organoid pattern was identified. The stroma around atypical cells was filled with eosinophilic hyaline material. These tumor cells were positive for vimentin only, and the tumor was consistent with undifferentiated sarcoma of the liver.
Aged ; Bile Ducts, Intrahepatic/pathology ; Diagnosis, Differential ; Dilatation, Pathologic ; Humans ; Klatskin's Tumor/diagnosis ; Liver Neoplasms/*diagnosis/pathology/ultrasonography ; Male ; Positron-Emission Tomography ; Sarcoma/*diagnosis/pathology/ultrasonography ; Tomography, X-Ray Computed ; Tuberculosis/drug therapy/radiography ; Vimentin/metabolism

Primary non-Hodgkin's lymphoma of the extrahepatic bile duct presenting as obstructive jaundice is extremely rare. A 60-year-old man was admitted due to suddenly developed jaundice. Computerized tomography and endoscopic retrograde cholangiopancreatography showed a tumor at the proximal common hepatic duct. These clinical and radiologic findings resembled those of Klatskin tumor. The resection of the common hepatic duct tumor, lymph node dissection, and Roux-en-Y hepaticojejunostomy were carried out. There was no regional lymph node metastasis and no residual tumor at the resection margins. Histology and immunohistochemistry of the resected specimen confirmed a diffuse large B-cell malignant lymphoma involving the common hepatic duct. The patient is scheduled to receive adjuvant chemotherapy. In summary, primary non-Hodgkin's lymphoma of the extrahepatic bile duct, despite its rarity, should be considered in the differential diagnosis of causes for obstructive jaundice. An accurate histopathologic diagnosis and surgical resection combined with chemotherapy may be the approach to offer a chance for cure.
Antigens, CD20/metabolism ; Bile Duct Neoplasms/*diagnosis/pathology/surgery ; Cholangiopancreatography, Magnetic Resonance ; Diagnosis, Differential ; Humans ; Klatskin's Tumor/diagnosis ; Lymphoma, Large B-Cell, Diffuse/*diagnosis/pathology/surgery ; Male ; Middle Aged ; Tomography, X-Ray Computed