Published online Jan 28, 2017. doi: 10.4254/wjh.v9.i3.155
Peer-review started: September 14, 2016
First decision: November 14, 2016
Revised: November 28, 2016
Accepted: December 13, 2016
Article in press: December 14, 2016
Published online: January 28, 2017
Mucosa-associated lymphoid tissue (MALT) lymphoma of the liver is a very rare condition and thus the diagnosis may be challenging. The clinical presentation is usually variable, ranging from minimal clinical symptoms to severe end stage liver disease. In this paper, we describe the clinicopathologic findings 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 CD79a, but negative for CD5, CD43 and CD10. There were occasional lymphoepithelial lesions involving the hepatocytes or bile ducts. Polymerase chain reaction analysis showed monoclonal immunoglobulin heavy chain gene rearrangement in both cases. The first case was treated with surgery but developed pulmonary recurrence a year after complete resection but went into remission following treatment with rituximab. A second recurrence occurred in the right parotid gland 7 years later, which was treated with idelalisib. The second case was effectively treated with rituximab. To our knowledge, the second case is the first reported case linked to autoimmune hepatitis.
Core tip: The diagnosis and management of mucosa-associated lymphoid tissue lymphoma of the liver can be a clinical dilemma. Recognition of the clinic-pathologic pattern and its associations with underlying autoimmune disease can prevent misdiagnosis. This case report not only represents the first reported association with autoimmune hepatitis and the development of multiple recurrences of the lymphoma in the literature, but also it applies new successful treatment regimens as an alternative to current clinical practice.