| Abstract: | Mucosa-associated lymphoid tissue(MALT) lymphoma of the liver is a very rare condition and thus the diag-nosis may be challeng-ing-. The clinical presentation is usually variable, rang-ing- from minimal clinical symptoms to severe end stag-e liver disease. In this paper, we describe the clinicopatholog-ic finding-s in two cases of primary hepatic MALT lymphoma. One case is an 80-year-old female with no underlying- chronic liver disease and the second case is a 30-year-old female with autoimmune hepatitis complicated by MALT lymphoma. In both specimens, there was diffuse infiltration of atypical B-lymphocytes that were positive for CD20 and CD79 a, but neg-ative for CD5, CD43 and CD10. There were occasional lymphoepithelial lesions involving- the hepatocytes or bile ducts. Polymerase chain reaction analysis showed monoclonal immunog-lobulin heavy chain g-ene rearrang-ement in both cases. The first case was treated with surg-ery but developed pulmonary recurrence a year after complete resection but went into remission following- treatment with rituximab. A second recurrence occurred in the rig-ht parotid g-land 7 years later, which was treated with idelalisib. The second case was effectively treated with rituximab. To our knowledg-e, the second case is the first reported case linked to autoimmune hepatitis. |
