Hepatic Lymphoma Diagnosis

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Overview

Although systemic hematopoietic neoplasms commonly involve the liver, hepatopathologists may encounter rare cases of primary hepatic lymphoma (PHL), which can often mimic benign conditions. PHL is defined as a liver-confined lymphoma without extrahepatic involvement,1, 2 and it constitutes approximately 0.4% of all primary extranodal non-Hodgkin lymphomas (NHLs).3, 4, 5 It most commonly affects middle-aged men, with a mean age of 50 years to 62 years (range: 21–86 years), who present with

Non-Hodgkin B-cell lymphomas

Most lymphomas involving the liver are of B-cell type, and they include DLBCL, Burkitt lymphoma (BL), B-lymphoblastic leukemia/lymphoma (B-LBL), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), hairy cell leukemia (HCL), follicular lymphoma (FL), mantle cell lymphoma (MCL), and extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT).4, 5, 8 Although high-grade B-cell lymphomas (including DLBCL, BL, and B-LBL) are aggressive and can occur over a wide

Mature T-cell neoplasms

T-cell lymphomas are much less common than B-cell lymphomas and more commonly involve the liver as part of a systemic disease.4, 8, 13, 44 Primary hepatic T-cell lymphoma is exceedingly rare, representing only 5% to 10% of PHLs, and usually follows an aggressive clinical course.4, 6, 8, 44, 45, 46, 47 Nearly all cases are peripheral T-cell lymphoma (PTCL), not otherwise specified (NOS), or hepatosplenic T-cell lymphoma (HSTCL).4

Immunodeficiency-associated lymphoproliferative disorders: post-transplant lymphoproliferative disorders

Transplant patients may develop abnormal, mostly EBV-driven, B-cell (or rarely T/NK-cell) proliferations in the liver, known as post-transplant lymphoproliferative disorders (PTLDs).55, 56, 57 Non-destructive lesions of PTLD resemble reactive lymphoid hyperplasia or chronic inflammation (ie, infectious mononucleosis) and do not efface the hepatic parenchyma, whereas polymorphic and monomorphic forms can resemble malignant lymphomas and efface the normal architecture.56, 57 Polymorphic

Classic Hodgkin Lymphoma

Hepatic involvement by CHL is uncommon, but rare cases of primary CHL have been reported.58 CHL usually forms discrete nodules with variable numbers of Hodgkin/Reed-Sternberg (HRS) cells in a background of mixed inflammatory cells, often involving or surrounding portal tracts (Fig. 6A, B).16, 58 CHL can rarely present as acute liver failure with extensive hepatic involvement.59 Although CHL is of B-cell origin, most B-cell markers (including CD20) are negative. One B-cell antigen consistently

Lymphoma mimics: nodular lymphoid lesion

Hepatic nodular lymphoid lesion (NLL) (also known as reactive lymphoid hyperplasia) can mimic lymphoma by displacing the surrounding hepatic parenchyma and forming a discrete mass.61, 62 It is a benign lesion, however, consisting entirely of polyclonal B-cells and T-cells forming reactive follicles.61, 62 Unlike MALT lymphoma, no diffuse sheets of B-cells outside benign reactive follicles are seen on CD20 stain.61, 62 Rather, the interfollicular areas show a mixed population of CD3+ T-cells and

Epstein-barr virus-positive hepatic lymphoid infiltrates

EBV typically infects B cells, through the EBV receptor (CD21). In EBV hepatitis, a liver biopsy most commonly shows a mild sinusoidal lymphohistiocytic infiltrate without piling up of the cells, hemophagocytosis, or overt atypia (Fig. 7A, B). There is usually no hepatocyte injury or necrosis, but non-necrotizing granulomas (and rarely fibrin ring granulomas) can be present. Lineage markers are helpful in excluding an EBV-driven malignancy or chronic active EBV infection (which may involve

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      Citation Excerpt :

      The pathologic findings of PHL vary depending on the type of lymphoma. Most cases are B-cell lymphomas, with diffuse large B-cell lymphoma (DLBCL) making up the vast majority [42,46]. Cell size varies depending on lymphoma type, and cells may demonstrate portal and/or sinusoidal involvement [46].

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      Citation Excerpt :

      Nevertheless, a retrospective cohort of 24 patients with PHL treated by combination chemotherapy demonstrated a favorable outcome with the complete remission rate of 83%; the 5-year survival rate was 87%.10 A combination of rituximab with cytotoxic treatment (especially for DLBCL) and the utilization of antiviral therapy (for HCV+ patients) have been shown to improve the clinical outcome of PHL significantly.1,2,10 Hodgkin lymphoma (HL) is a common type of lymphoma that is characterized by the presence of Reed-Sternberg cells in lymph nodes and by spread in a contiguous fashion via the lymphatic system.5

    View all citing articles on Scopus

    Disclosure Statement: The authors have nothing to disclose.

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