Primary hepatic large B-cell lymphoma

Primary hepatic large B-cell lymphoma (PHLBCL) is a non-Hodgkin large B-cell lymphoma, arising in the liver, without apparent evidence of extrahepatic involvement at diagnosis. Due to its rarity, and, in many cases, a lack of prior history of lymphoma, PHLBCL is not usually included in the initial differential diagnoses for large cell liver neoplasms with a poorly-differentiated appearance. If diagnosed, many patients with PHLBCL show favorable responses to chemotherapy. Although clinical features and oncologic outcome of primary hepatic large B-cell lymphoma are relatively well-described in the literature, histopathologic features are not well-documented. This review summarizes the clinical features, morphology, immunophenotype and available genetic data of PHLBCL. Significant features of PHLBCL from the literature include: female predilection, hepatomegaly, abdominal pain, elevated liver enzymes, a variable number of liver lesions, ki-67 proliferation index of at least 60% and no predominance of either the germinal center or non-germinal center subtypes of diffuse large B-cell lymphoma.