Tackling Tough Questions

Background: Coexistence of splenic marginal zone lymphoma with hepatocellular carcinoma is rare. Although some reports have suggested the possible pathogenic role of HBV, HCV, chronic and persistent antigenic stimulation in lymphoma, their role in causing lymphomas is still unclear. Case presentation: We describe a hepatocellular carcinoma with concomitant splenic marginal zone lymphoma in a 64-year-old Chinese man with cirrhosis. Serum hepatitis B virus surface antigen was positive and antihepatitis C virus antibody was negative. The resected liver mass measuring 4 × 3 × 3 cm was grey and soft with a small area of bleeding, necrosis and intact capsule. Cut surface of the spleen was red-purple and had a diffuse reticulonodular appearance indicative of prominent white pulp. On histologic sections, the liver mass was well and moderately differentiated hepatocellular carcinoma, and the splenic tumor was a specific low-grade small B-cell lymphoma. Immunohistochemical staining and gene rearrangement studies supported that the splenic tumor represents a clonal B-cell lymphoma. Therefore, the diagnosis of SMZL was made from the splenic specimen. Conclusion: To our knowledge, this is the second case report describing coexistence of hepatocellular carcinoma and splenic marginal zone lymphoma in the course of chronic HBV infection. However, we cannot assert at present that hepatitis B virus is directly involved in splenic lymphomagenesis until more information is collected from more cases in the future. Background The frequency of primary lymphoma of the spleen is relatively rare, although malignant lymphoma commonly involves the spleen [1]. Splenic marginal zone lymphoma (SMZL) is a distinctive and well-characterized B-cell neoplasm that involves the spleen and various organs [2]. Although SMZL is accepted as an entity in the World Health Organization (WHO) classification [3], its histogenesis remains unclear. There are several reports of simultaneous occurrence of additional cancers and SMZL [4-6]. To our knowledge, only one reported patient who had coexistent SMZL with hepatocellular carcinoma (HCC) [4]. Here, we present a case report of SMZL with coexistent HCC. Published: 6 February 2007 Diagnostic Pathology 2007, 2:5 doi:10.1186/1746-1596-2-5 Received: 16 September 2006 Accepted: 6 February 2007 This article is available from: http://www.diagnosticpathology.org/content/2/1/5 © 2007 Zhang et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.