There are many autoimmune liver diseases in which diagnosis is difficult so that overlap is accepted, and this negatively affects treatment. The initial diagnosis is therefore important for later treatment and convalescence. We distinguished autoimmune cholangitis, autoimmune hepatitis and primary biliary cirrhosis by the Mahalanobis·Taguchi Adjoint (MTA) method in the Mahalanobis·Taguchi system and analyzed the pattern of factor effects by the MTA method. As a result, the characteristic factor effect pattern of each disease was classified, enabling the qualitative evaluation of cases including overlapping cases which were difficult to diagnose.
We report a case of a 64-year-old Japanese man with adenoid cystic carcinoma. An elevated lesion covered by intact epithelium in the thoracic esophagus was found in September, 2007 and been followed. After dysphagia appeared follow-up endoscopy was performed in January, 2010, and morphological change into a protruding tumor was recognized. Since adenoid cystic carcinoma was detected by endoscopic biopsy, the patient underwent esophageal resection. The resected specimen showed a cribriform pattern and a certain amount of mucous substance which was positive for Alcian blue, within a solid nest. The tumor cells were positive for S-100 protein and negative for αSMA, so the tumor was diagnosed as an adenoid cystic carcinoma.
A 98-year-old woman with aspiration pneumonia caused by vomiting was admitted to our hospital. Abdominal computed tomography and ultrasonography showed common bile duct dilation and occlusion of the gastrointestinal tract due to a large tumor in the duodenum. Aspiration pneumonia and hepatic failure led to the patient's death. Pathological autopsy showed an 11×6×2.5-cm polypoid tumor originating from the descending part of the duodenum and involving the common bile duct. Histological examination of the tumor showed mainly spindle cells but also some adenocarcinoma cells. Tumor cells, including spindle cells, were strongly positive for cytokeratin 7; therefore, sarcomatoid carcinoma originating from the duodenum was diagnosed. To the best of our knowledge, only 5 cases of sarcomatoid carcinoma originating from the duodenum have been reported, and they often show polypoid growth. The possibility of sarcomatoid carcinoma should be considered if such findings are noted.
Thiopurine drugs have been widely used in the treatment of inflammatory bowel disease. However, their use is limited by adverse effect that can lead to cessation of therapy. We report 2 cases of thiopurine-induced acute pancreatitis in patients with inflammatory bowel disease. Both patients complained of abdominal pain, showed elevated pancreatic enzymes, and swollen pancreases on computed tomography. The patients' signs and symptoms resolved uneventfully after withdrawal of the thiopurine drugs. Although the mechanism of thiopurine-induced pancreatitis remains unclear, close monitoring and early recognition of acute pancreatitis is important in the management of new thiopurine users.
We report a rare case which had been followed up for hepatic hemangioma and in whom was surgical resection revealed with cholangiolocellular carcinoma (CoCC) combined with intrahepatic cholangiocarcinoma (ICC). A 69-year-old man who was an HBV carrier had been regularly followed up with hepatic hemangioma from November, 2005. Because the arterial phase of dynamic CT scan exhibited an enhanced lesion in the dorsal portion of the hemangioma on November, 2009, the patient was admitted for intensive examination of the liver tumor. After surgical resection of the tumor, histological examination revealed small irregular tubules in the outer part and scattered small duct structures in the inner part of the tumor. In addition, immunohistochemical analysis demonstrated that cytokeratin (CK) 7, CK19 and epithelial membrane antigen (EMA) were all positive in the outer part, and EMA was only negative in the inner part of the tumor. From these findings, this case was diagnosed as CoCC combined with ICC.
We describe a case of autoimmune hepatitis diagnosed after acute hepatitis B. The patient was a 65-year-old man admitted because of markedly elevated transaminase level. Laboratory tests showed positive IgM-HBc antibody and a short prothorombin time. He was diagnosed as severe acute hepatitis B due to sexual transmission. He received lamivudine and steroid pulse therapy. Transaminase level increased again after steroid pulse therapy and liver atrophy progressed, so cyclosporine was induced. Liver biopsy was done because of prolonged liver function disorder. Biopsy specimens showed not only centrizonal inflammation but also interface hepatitis and bridging fibrosis, which were characteristic of autoimmune hepatitis. We diagnosed autoimmune hepatitis which became clinically evident after acute hepatitis B. Lamivudine was discontinued 7 months after diagnosis and he is now receiving 3mg prednisolone.
A 41-year-old man was admitted to our hospital with gastrointestinal bleeding. Esophagogastroduodenoscopy revealed a submucosal protrusion with erosion in the duodenal bulb which was thought to be the bleeding source. Dynamic CT scan, ultrasonography and angiography of the abdomen revealed a hepatic artery aneurysm and a dilated celiac artery that dissected from its origin. Although we considered percutaneous transcatheter arterial embolization with metallic coils, we chose surgical resection and vascular reconstruction to prevent hepatic ischemia resulting from interruption of collateral circulation. On the 8th day, hepatic artery aneurysmectomy and revascularization with a great saphenous vein was carried out without any severe complication. The pathological specimen demonstrated segmental arterial mediolysis.
A 57-year-old man presented with jaundice. Abdominal computed tomography showed a 10-cm left hepatic lobe heterogeneous solid mass with low attenuated areas in the mass, multiple liver metastases and lung metastasis. Serology for hepatitis B and C were negative. Serum alpha-fetoprotein, CEA and CA19-9 were normal. The patient died a few weeks later of progressive liver failure and an autopsy was performed. Histologically, the tumor consisted of sarcomatoid mononuclear cells and osteoclast-like giant cells. The liver tissue surrounding the tumor showed no cirrhotic pattern. The osteoclast-like giant cells were uniformly and strongly immunoreactive with CD68. The mononuclear cells demonstrated expression of vimentin but were negative for CAM5.2. The MIB-1 index was 20% for the mononuclear cells. In conclusion, the histopathological diagnosis revealed an osteoclast-like giant cell tumor of the liver.