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Liver and intrahepatic bile ducts - tumor

Leukemia / lymphoma

Lymphoma-primary


Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.

General
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● Rare; ~ 100 cases reported; <1% of all extranodal lymphomas, 0.01% of non-Hodgkin lymphoma (Cancer 1972;29:252)
● Clinical disease due mainly to liver involvement, without systemic disease or distal lymphadenopathy
● 75% men, median age 55 years but all ages
● Symptoms: abdominal pain and hepatomegaly; may present with liver failure; associated with cirrhosis, immunocompromise
● Also associated with HBV (3% - 30%, Jpn J Cancer Res 2002;93:471), HCV (40%60%, Clin Lymphoma Myeloma 2006;6:273) and EBV
● Pathogenesis of HCV-associated lymphoma involves chronic antigen stimulation of B-cell populations (J Clin Pathol 2007;60:1378)
● Most common subtype is diffuse large B cell lymphoma
● Two year survival of 66%; poor survival if cirrhosis or immunocompromise

Gross description
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● Large mass or multiple masses (30-35%) resembling carcinoma
● Variable hemorrhagic and necrosis

Micro description
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● May have diffuse sinusoidal infiltration

Differential diagnosis
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● Carcinoma: primary or metastatic
● CMV or EBV: need serology or stains to confirm
● Granulomas
● Hepatitis: polymorphic infiltrates with minimal atypia
● Inflammatory myofibroblastic tumor
● Primary biliary cirrhosis
● Venous outflow obstruction

End of Liver and intrahepatic bile ducts - tumor > Leukemia / lymphoma > Lymphoma-primary


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