Childhood hepatocellular carcinoma: a clinicopathological study of 12 cases with special reference to EpCAM.

Aims: To elucidate the characteristics of hepatocellular carcinoma (HCC) in children.

Methods And Results: A retrospective search of our database identified 12 children with HCC (aged 10 months to 11 years; male/female ratio of 5:7). Their pathological features were compared with those of adult HCCs (n = 20), fibrolamellar HCCs (n = 14), and hepatoblastomas (n = 15).

Possible involvement of CCL1-CCR8 interaction in lymphocytic recruitment in IgG4-related sclerosing cholangitis.

Background & Aims: IgG4-related sclerosing cholangitis and type 1 autoimmune pancreatitis (IgG4-SC/AIP) are characterized by massive lymphoplasmacytic infiltration including Th2 and regulatory T cells (Tregs). This study was conducted to address which chemotactic factors are involved in this condition.

Methods: Chemokine expression profiles in tissue were examined in IgG4-SC/AIP (n=17), classical primary sclerosing cholangitis (IgG4(low) PSC, n=17), PSC with elevated serum/tissue IgG4 levels (IgG4(high) PSC, n=5), and primary biliary cirrhosis (n=7).

Immunoglobulin G4-related sclerosing cholangitis: pathologic features and histologic mimics.

Since immunoglobulin G4 (IgG4)-related sclerosing cholangitis was first described in 2004, this condition has been recognized as a distinct entity characterized by unique pathologic features. Affected bile ducts show diffuse wall thickening, which represents transmural sclerosing inflammation composed of a dense lymphoplasmacytic infiltrate and fibrosis arranged at least focally in a storiform pattern. A diffuse IgG4-positive plasma cell infiltrate is demonstrable by immunohistochemistry.

Sclerosing cholangitis with granulocytic epithelial lesion: a benign form of sclerosing cholangiopathy.

The association between autoimmune pancreatitis and sclerosing cholangitis has attracted considerable attention. In contrast to type 1 (IgG4-related) autoimmune pancreatitis, bile duct involvement is uncommon in type 2 autoimmune pancreatitis, a more benign condition characterized histologically by granulocytic epithelial lesions (GELs). Following our recent report on a child with GEL-positive sclerosing cholangitis and excellent response to steroids, we retrospectively reviewed the liver histology of a large number of patients with sclerosing cholangitis to investigate the possible role of type 2 autoimmune pancreatitis in this pathology.

Solid organ transplantation for non-TTR hereditary amyloidosis: report from the 1st International Workshop on the Hereditary Renal Amyloidoses.

Fibrinogen A α-chain (AFib) and apolipoprotein AI (AApoAI) amyloidosis due to variants in the AFib and ApoAI genes are the most common types of hereditary amyloidosis in Europe and the United States. Liver is the exclusive source of the aberrant amyloidogenic protein in AFib and responsible for supplying approximately half of the circulating variant ApoAI. Nephrotic syndrome and renal impairment due to renal amyloidosis are common disease manifestations; however, recent research provides evidence to support a more diverse and systemic disease phenotype, which in turn has implications in the management of the hereditary amyloidoses with solid organ transplantation and, in particular, liver transplantation.