Three siblings with congenital dilatation of the intrahepatic bile ducts (Caroli's disease) are presented. Bile duct pathology was associated with congenital hepatic fibrosis and polycystic renal disease in all three patients. On color Doppler imaging (CD imaingl, multiple small color Doppler signals were observed in or near the vascular radicles within the dilated bile ducts, besides other well-known sonographic findings such as bile duct dilatations, biliary calculi. Dopper frequency spectral analysis confirmed all these color Doppler signals as arterial origin in all patients, showing pulsatile wave pattern. Although portal venous radicles are well known in conventional sonograms or computed tomotraphy(CT), continuous wave patterns were not detected in all patients. In addition to previously reported sonographic findings about Caroli's disease, color Doppler signals showing arterial wave pattern in or around the portal venous radicles within dilated duets are another helpful diagnostic criteria and these findings are easily depicted on routine sonograms with color mapping.
Bile Ducts* ; Bile Ducts, Intrahepatic ; Bile* ; Caroli Disease* ; Dilatation ; Fibrosis ; Gallstones ; Humans ; Pathology ; Polycystic Kidney Diseases ; Siblings ; Ultrasonography

Biliary cystadenomas are very rare cystic neoplasms usually arising from the intrahepatic bile ducts. The majority of patients are middle-aged women who present with abdominal discomfort and/or a palpable mass. The diagnosis was performed using several methods such as abdominal ultrasonography, computed tomography, percutaneous cholangiography or endoscopic retrograde cholangiopancreatography, and showed multiloculated cystic tumors with multiple internal septation. But confirmatory diagnosis was done by surgical pathology. Because the prognosis is comparatively better than after complete resection and the cystadenoma is warranted to avoid malignant change, early detection and surgical resection must be needed. We have experienced two cases, 30 and 50 year old females, which presented with nonspecific abdominal pain and dyspepsia. They were diagnosed as having intrahepatic biliary cystadenoma histopathologically which was confirmed after surgical resection. We report two cases of intrahepatic biliary cystadenoma with a review of literature.
Abdominal Pain ; Bile Ducts, Intrahepatic ; Cholangiography ; Cholangiopancreatography, Endoscopic Retrograde ; Cystadenoma* ; Diagnosis ; Dyspepsia ; Female ; Humans ; Liver ; Middle Aged ; Pathology, Surgical ; Prognosis ; Ultrasonography

Hemangioma is one of the most frequently encountered benign hepatic neoplasm which can develop secondary degeneration. Sclerosed hemangioma is a rare disease histologically characterized by large amount of collagen and elastic fibril between sclerosed small vessels. Its differential diagnosis is very difficult. It should be included in the differential diagnosis of other hepatic lesions such as hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and metastatic hepatic tumor. A 77-year old male was admitted with upper abdominal discomfort. Abdominal ultrasonography revealed GB stone, dilated common bile duct with bile duct stone, and a 4.6 cm sized hyperechoic mass at segment 5 and 6 of the liver. Abdominal dynamic computed tomography demonstrated dilated intrahepatic bile ducts and a 5x5 cm sized mass which showed minimally delayed enhancement. Abdominal magnetic resonance imaging revealed the mass with low signal intensity in T1 weighted image, high signal intensity and focal low signal in T2 weighted image which showed minimal enhancement. We removed common bile duct stone with endoscopic retrograde cholangiopancreatography then decided to undergo right lower segmentectomy of liver due to possibility of cholangiocarcinoma. Histopathological examination of hepatic mass showed large amount of fibrous tissue with occasional residual vascular channels. We describe one case of sclerosed hemangioma mimicking cholangiocarcinoma.
Aged ; Bile Duct Neoplasms/diagnosis ; Bile Ducts, Intrahepatic ; Cholangiocarcinoma/diagnosis ; Diagnosis, Differential ; Hemangioma/*diagnosis/pathology/ultrasonography ; Humans ; Liver Neoplasms/*diagnosis/pathology/ultrasonography ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed

A 51-year-old male patient with chronic hepatitis B was referred to our hospital due to a 1-cm liver nodule on ultrasonography. Alpha-fetoprotein (AFP) was slightly elevated. The nodule showed prolonged enhancement on dynamic liver magnetic resonance imaging and appeared as a hyperintensity on both diffusion-weighted and T2-weighted imaging. The nodule was followed up because it was small and typical findings of hepatocellular carcinoma (HCC) were not observed in the dynamic imaging investigations. However, liver contrast-enhanced ultrasonography performed 1 month later showed enhancement during the arterial phase and definite washout during the delayed phase. Also, AFP had increased to over 200 ng/mL even though AST and ALT were decreased after administering an antiviral agent. He was presumptively diagnosed as HCC and underwent liver segmentectomy. Microscopy findings of the specimen indicated bile duct adenoma. After resection, the follow-up AFP had decreased to within the normal range. This patient represents a case of bile duct adenoma with AFP elevation mimicking HCC on contrast-enhanced ultrasonography.
Bile Duct Neoplasms/*complications/*diagnosis/pathology ; *Bile Ducts, Intrahepatic ; Hepatitis B, Chronic/*complications/*diagnosis/pathology ; Humans ; Liver/pathology/ultrasonography ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Tomography, X-Ray Computed ; alpha-Fetoproteins/*metabolism

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

This study describes an evaluation of the sonographic, cholangiographic, pathological, and immunological findings, and the protective effect shown by rats reinfected with Clonorchis sinensis. Eight experimental rat groups were, namely, a normal control, a primary infection control, a reinfection I (reinfection 7 week after treatment following 3-week infection), a reinfection II (reinfection 2 week after treatment following 8-week infection), a reinfection III (exploration of the intrahepatic bile ducts 1 week after reinfection 4 week after treatment following 4-week infection), a superinfection, a secondary infection control, and an infection following immunization group. Sonographic and cholangiographic findings showed moderate or marked dilatation of the bile duct confluence in the primary infection control, reinfection II, and secondary infection control groups. Juvenile worms survived in the intrahepatic bile ducts 1 week after reinfection following treatment in the reinfection III group. It was concluded that reinfecting juvenile worms found during the first week following reinfection failed to survive or grow further. Anatomical, pathophysiological, or immunological changes may induce protection from reinfection in rats.
Animals ; Anthelmintics/administration & dosage/therapeutic use ; Antibodies, Helminth/blood ; Antigens, Helminth/administration & dosage/immunology ; Bile Duct Diseases/parasitology/*pathology/ultrasonography ; Bile Ducts, Intrahepatic/parasitology/*pathology/ultrasonography ; Cholangiography ; Clonorchiasis/parasitology/*pathology/ultrasonography ; Clonorchis sinensis/*pathogenicity ; Immunization ; Praziquantel/administration & dosage/therapeutic use ; Rats ; Rats, Sprague-Dawley ; Sound Spectrography ; Support, Non-U.S. Gov't

IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Autoimmune Diseases/complications/diagnosis ; Bile Ducts, Intrahepatic/pathology/ultrasonography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing/complications/*diagnosis/drug therapy ; Common Bile Duct/pathology/ultrasonography ; Humans ; Immunoglobulin G/*blood ; Immunohistochemistry ; Male ; Pancreatitis/complications/diagnosis ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed