Biliary cystadenocarcinoma with oncocytic differentiation was first reported in 1992. This is a report of a second case. The patient (a 71-year-old man) was admitted to our hospital complaining of abdominal fullness. Multicystic lesions were identified in the left hepatic lobe radiologically. The patient died of peritoneal dissemination of carcinoma 20 months later. At autopsy, the tumor of the left hepatic lobe was found to be composed of adjoining multiple cystic lesions and a solid lesion with infiltration of the hepatic hilus and peritoneal dissemination. Histologically, the multicystic lesions were covered by papillary neoplastic epithelial cells with an eosinophilic granular cytoplasm resembling that of oncocytes and a fine fibrovascular core. The cyst wall was fibrous, but there was no mesenchymal stroma. In the solid lesion and infiltrated areas, acidophilic and granular carcinoma cells formed small glandular or solid cord patterns with much mucin secretion (mucinous carcinoma). Immunohistochemically, carcinoma cells of both components were found to contain many mitochondria and showed the phenotypes of hepatocytes and cholangiocytes. Interestingly, the intrahepatic biliary tree also was invaded by carcinoma cells. This may be a case of intraductal oncocytic papillary neoplasm of the left hepatic lobe followed by secondary cystic dilatation of the affected bile duct.
Aims: In this study, we attempted to define a new histological staging and grading system to provide more information reflecting the clinical laboratory data and prognosis to hepatologists. Methods and Results: First, 17 histological lesions of primary biliary cirrhosis (PBC) were scored in 188 needle liver biopsy specimens. Factor analysis yielded three independent groups of factors: Factor 1 (fibrosis, fibrous piecemeal necrosis, orcein positive granules, bile plugs, Mallory bodies, feathery degeneration, bile duct loss and atypical ductular proliferation); Factor 2 (portal inflammation, eosinophilic infiltration, lymphoid follicle, epithelioid granuloma, interface hepatitis and chronic cholangitis); and Factor 3 (interface hepatitis, lobular hepatitis, acidophilic bodies and pigmented macrophages). Eight findings of Factor 1, but not Factors 2 and 3, were significantly correlated with the clinical laboratory data and scores of Mayo's prognostic model. Factor 1 lesions may reflect the histological progression (staging), while Factor 2 and 3 lesions may relate to necroinflammatory activities (grading). Then, we devised a staging and grading system using three lesions (bile duct loss, fibrosis and orcein positive granules) from Factor 1 and three from Factors 2 and 3 (chronic cholangitis, interface hepatitis and lobular hepatitis). This new system might provide more pathological information of PBC patients to hepatologists.
Hiramatsu -4-
Serum antinuclear antibodies (ANA) are occasionally noted in the patients with non-alcoholic steatohepatitis (NASH). We examined the significance of ANA in NASH by comparing the clinicopathological features in the patients with ANA-positive NASH (n=35) and ANA-negative NASH (n=36). Inflammatory cell profiles and the distribution of oxidative stress markers were also examined immunohistochemically. The ANA-positive NASH was significantly associated with female gender (p= .005), high degree of portal inflammation (p= .039), interface activity (p= .036) and hepatocellular ballooning (p= .0008). In addition, The ANA of high-titer (320-fold or more) was significantly associated with the histological grade and stage of NASH (p= .02). The degree of steatosis is rather mild in high-titer ANA group (p= .01). The analysis of inflammatory cell profiles revealed that CD3-positive T cells were predominant and plasma cells were rather few in portal area and hepatic lobules in both ANA-positive and ANA-negative groups. There was no difference in the distribution of oxidative stress markers between ANA-positive and ANA-negative groups. These findings suggest that the presence of ANA may be related to the progression of NASH and that a different type of autoimmune mechanisms may be involved in the pathogenesis of NASH with ANA, compared to the pathogenesis of autoimmune hepatitis.
Idiopathic retroperitoneal fibrosis (IRPF) is an inflammatory fibrosclerosing condition, leading to renal failure by obstruction of the ureters. Idiopathic chronic pancreatitis associated with marked inflammatory infiltrates has recently been referred to as autoimmune pancreatitis (AIP), and infiltrating plasmacytes carrying immunoglobulin-gamma type 4 (IgG4) are relevant to its pathogenesis. The case is described herein of IRPF associated with subclinical pancreatitis that was most probably AIP in a 70-year-old man. Biopsy specimens of the retroperitoneal pseudotumor revealed a marked lymphoplasmacytic infiltration with dense fibrosis. Infiltrating plasma cells were immunoreactive for anti-IgG4 antibodies. Subsequent systemic examinations showed an extremely elevated serum IgG4 level and pancreatitis concordant with AIP. Following oral steroid administration, the serum IgG4 level normalized, although the appearance of the pseudotumor did not alter. Some AIP cases have been associated with idiopathic fibrosclerosing disorders including IRPF, but histological evidence of IgG4-related IRPF has rarely been provided.
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