Hepatolithiasis and the syndrome of recurrent pyogenic cholangitis: clinical, radiologic, and pathologic features.

Primary hepatothiasis (HL) and recurrent pyogenic cholangitis (RPC) are two terms describing the different aspects of the same disease, with HL emphasizing the pathologic changes and RPC emphasizing the clinical presentation and suppurative inflammation. It is predominantly a disease of the Far East. In the 1960s, it was the third most common cause of emergency abdominal surgery at a university hospital in Hong Kong. Thereafter, its incidence has decreased considerably, possibly due to improved standards of living and Westernized diet. Clinically, patients may present acutely with recurrent bacterial cholangitis and its possible complications, such as liver abscess and septicemic shock, or with chronic complications, such as cholangiocarcinoma. Pathologically, it is characterized by pigmented calcium bilirubinate stones within dilated intrahepatic bile ducts featuring chronic inflammation, mural fibrosis, and proliferation of peribiliary glands, without extrahepatic biliary obstruction. Episodes of suppurative inflammation cumulate in sclerosing cholangitis of peripheral ducts and parenchymal fibrosis resulting from collapse and scarring. Mass-forming inflammatory pseudotumor and neoplasms like intraductal papillary neoplasms and cholangiocarcinoma are increasingly recognized complications. Modern imaging techniques allow definitive diagnosis, accurate assessment for treatment planning, and detection of complications. A multidisciplinary team approach (interventional endoscopist, interventional radiologist, hepatobiliary surgeon, and intensivists) is important for optimal patient outcome.

Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease.

IgG4-related sclerosing disease is a recently recognized inflammatory lesion frequently involving pancreas, submandibular gland, lacrimal gland, and lymph node. We report 3 cases of ocular adnexal lymphoma arising in IgG4-related chronic sclerosing dacryoadenitis, a phenomenon that has not been previously reported. The patients presented with bilateral or unilateral ocular adnexal mass usually present for many years. One patient also had asymptomatic diffuse lymphadenopathy. Two patients had biopsy-proven IgG4-related chronic sclerosing dacryoadenitis before the current presentation, and 1 had systemic involvement by IgG4-related sclerosing disease as evidenced by increased IgG4+ cells in a prior nasopharyngeal biopsy. Two cases showed features of extranodal marginal zone lymphoma of mucosa-associated lymphoid-tissue type (1 with large cell transformation) and 1 follicular lymphoma. Thus, the lymphoid hyperplasia of IgG4-related sclerosing disease can provide a substrate for the emergence of lymphoma. In addition, we report 3 cases of ocular adnexal extranodal marginal zone B-cell lymphoma that show sclerosing inflammation in the background and numerous IgG4+ monotypic plasma cells. In the absence of prior biopsies or information on serum IgG4 titer, it is unclear whether these cases represent lymphoma complicating IgG4-related sclerosing disease or de novo lymphoma. Nonetheless, these cases are distinctive in that the neoplastic cells express IgG4 (light chain restricted), whereas unselected cases of ocular adnexal lymphomas do not show IgG4 expression.