We reviewed the 4 cases of patients with carcinoma of the cystic duct based on the clinicopathological records. Histopathological findings (i. e., H. E. staining and cell proliferating potency assessed by MIB-1 staining) for both main and invasive regions of the carcinoma were compared to those of 12 patients with gallbladder carcinoma) and 6 patients with middle bile duct carcinoma, The US showed no swelling of the gallbladder in 3 cases, which were diagnosed later as atrophic gallbladder, adenomyomatosis of the gallbladder, and dystelectasis due to the carcinoma infiltration. The CT showed that in the 2 cases showing nodular-type, the tumors were enhanced by contrast medium. The direct cholangiography failed to demonstrate the right hepatic duct and gallbladder in a case of advanced carcinoma. The regions of biliary tree distal to the obstruction could be evaluated by MRCP. The angiography showed tumor stains in the 2 cases of advanced carcinomas. The pathology revealed that papillary adenocarcinoma predominated. The carcinoma of the cystic duct at advanced stages was observed to often invada into perineural space. The perineural invasion was more frequent in carcinoma of the cystic duct than in gallbladder carcinoma, the finding making cystic duct carcinoma rather similar to bile duct carcinoma. Thus, it is suggested that papillary carcinoma is transformed to tubular adenocarcinoma when extending out of the cystic duct. In this transformation, the carcinoma exhibited the high value of cell proliferating potency when assessed by MIB-1 staining. These results suggest that in carcinoma of the cystic duct the tumors extending out of the cystic duct become aggressive and therefore offer poorer prognosis.

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The purpose of the present study was to clarify the utility of chemoradiation therapy for patients with unresectable mass-forming intrahepatic cholangiocarcinoma (ICC). Fourteen patients with unresectable mass-forming ICC were studied. Six patients underwent external irradiation totalling 42-60 Gy and systemic chemotherapy (5 patients underwent low dose CDDP and 5-FU, and 1underwent EPIR and 5-FU) after 1999 (treated), while no radiation or chemotherapy was performed for 8 patients before 1999 (untreated). No significant differences existed between treated and untreated patients on imaging, TNM factor or stage. In the treated group, minor response was achieved in 3 patients (50%). Disease was stable in one patient, and progressed in the other two. The 50% survival period for the treated group was 17 months, while that for the untreated group was 5 months (p<0.05). Survival rates for the treated group were 66.7% at 1 year and 22.2% at 2 years. All untreated patients died within 8 months. Chemoradiation therapy seems to improve the prognosis of patients with unresectable mass-forming ICC.

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In patients with complete bile duct obstruction, the only pathway of the elimination of cholephilic compounds is through the urine. Although the changes of various transporters in the liver and kidney in cholestasis have been elucidated, little is known about how effectively the elimination of these compounds is compensated by the urinary excretion. Therefore, in the present study, the urinary excretion of cationic compounds was compared in bile duct-ligated rats for 3 days (BDL). Among organic cations, erythromycin (49±5.2% in BDL vs 19±3.6% in controls) was more effectively excreted into the urine than vinblastine (18.2±4.7% in BDL vs 7.4±2.2% in controls)in BDL. Although the elimination of the cholephilic compounds was compensated by the urinary excretion in BDL, the degree of the urinary excretion was different between cholephilic organic cations.

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We report a rare case of hilar bile duct cancer with constitutional ICG excretory defect, that revealed high retention of ICG test and normal retention of BSP test. The patient,60-year old man, was admitted with jaundice to our hospital. PTC and ERC showed the stenosis from hilar bile duct to right hepatic duct indicating hilar bile duct cancer. To reduce the total bililubin level, that was 12 mg/dl at admission, PTCD was performed into the hepatic bile duct of anterior and lateral segment. After the total bililubin level was normalized, ICG test, which was performed for the preoperative examination of hepatectomy routinely, revealed the markedly delay of ICG retention rate at 15 minutes (78%), while BSP retention rate at 30 and 45 minutes was the normal level (less than 1%). Since other liver function tests indicated no liver cirrhosis, we diagnosed this patient as constitutional ICG excretory defect and considered that the major hepatectomy may be feasible. Extended left hepatic lobectomy and hepatico-jejunostomy was performed and the post operative course was uneventful. Microscopic examination showed neither liver cirrhosis nor hepatitis, we supposed that the liver function of this case had been almost normal although the markedly delay of ICG retention rate. The constitutional ICG excretory defect was first reported by Namihisa et. al. in 1975, as a new type of dye excretory disorder of liver with heredity or construction. Our case is the first report of constitutional ICG excretory defect associated with hilar bile duct cancer that was required the major hepatectomy. In case of ICG excretory defect, the choice of adequate operation such as major hepatectomy is difficult, and more experience with this disorder might be required.

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A 35-year-old woman was admitted with a complaint of right hypochondralgia. Six months ago, she had had hemobilia due to liver biopsy for chronic hepatitis and undergone transcatheter arterial embolization for hemostasis. Computed tomography and ultrasonography on admission revealed swelling of the gallbladder and high density mass highly suggestive of blood clots in the gallbladder. Based on the diagnosis of acute cholecystitis caused by blood clots in the gallbladder, we performed cholecystectomy. In recent years, the incidence of hemobilia has increased because the technique of liver biopsy and percutaneous transhepatic biliary drainage has become widely used. Blood clots in the gallbladder are frequently observed associated with hemobilia (71.4%), and usually disappear within 2 months. However, in the case of poor gallbladder function, blood clots remain in the gallbladder and sometimes may cause acute cholecystitis. We therefore must take care to check the blood clots in the gallbladder after hemobilia.

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Bile duct carcinoma with multiple stenosis in the biliary system is hardly distinguished from primary sclerosing cholangitis.
A-70-year-old woman was referred to our department because of the dilatation of the biliary tree detected by a CT scan. Cholangiography demonstrated stenoses of the right hepatic duct and common bile duct. Because biopsy specimens were found to be adenocarcinoma, we performed a left hepatic trisegmentectomy with caudate lobectomy and pylorus-preserving pancreatoduodenectomy 4 weeks after percutaneous transhepatic portal embolization (PTPE).
Pathological examination of resected specimen disclosed that moderately differentiated adenocarcinoma was continuously spread from the left hepatic and anterior segmental ducts down to the intrapancraetic bile duct. The carcinoma had various degrees of invasion and desmoplastic reaction from site to site. We speculate that the multiple stenosis in biliary tree of this case is due to the differences in the cancer invasion and the desmoplastic reaction between the stenotic lesions and the others.

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