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WHO Classification of Tumours
Diffuse large B-cell lymphoma (DLBCL), NOS
Liver and intrahepatic bile ducts


Definition

Diffuse large B-cell lymphoma (DLBCL) is the most frequent type of non-Hodgkin lymphoma, representing 30%-40% of cases. It consists of a diffuse proliferation of large neoplastic B-cells without significant admixture of centrocytes. It affects mainly adults, with a mean age of manifestation in the 7th decade. The majority of DLBCLs originates primarily in lymph nodes, but up to 40% are initially confined to extranodal sites
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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



. DLBCLs are aggressive but potentially curable with multiagent chemotherapy.

Primary hepatic lymphoma is defined as an extranodal lymphoma arising in the liver, with the bulk of the disease localized to this site. More often, the liver is a secondary site of involvement by lymphoma. Primary lymphoma of the liver is rare

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Isaacson PG (1999)
Gastrointestinal lymphomas of T- and B-cell types.
Mod Pathol 12: 151-8



and mainly a disease of middleaged men
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Memeo L, Pecorello I, Ciardi A, Aiello E, De Quarto A, Di Tondo U (1999)
Primary non-Hodgkin's lymphoma of the liver.
Acta Oncol 38: 655-8



, although occasional paediatric cases are reported.

DLBCLs
The majority of primary hepatic lymphomas are DLBCLs and are composed of a monotonous proliferation of large cells expressing CD20 and other B-cell associated antigens, with formation of a destructive mass lesion.

Other hepatic B-cell lymphomas:
> T-cell/histiocyte rich large B-cell lymphomas involve the liver more diffusely, usually with infiltration of portal triads.
> Occasional cases of Burkitt lymphoma have been described

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Huang CB, Eng HL, Chuang JH, Cheng YF, Chen WJ (1997)
Primary Burkitt's lymphoma of the liver: report of a case with long-term survival after surgical resection and combination chemotherapy.
J Pediatr Hematol Oncol 19: 135-8



, usually presenting as a solitary mass.
> Low-grade B-cell lymphomas of MALT type are characterized by a dense lymphoid infiltrate within the portal tracts. The atypical lymphoid cells have centrocyte- like cell morphology and surround reactive germinal centres. Lymphoepithelial lesions may involve the bile-duct epithelium, and these may be highlighted by staining with antibodies to keratins

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Kirk CM, Lewin D, Lazarchick J (1999)
Primary hepatic B-cell lymphoma of mucosa-associated lymphoid tissue.
Arch Pathol Lab Med 123: 716-9




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Maes M, Depardieu C, Dargent JL, Hermans M, Verhaeghe JL, Delabie J, Pittaluga S, Troufléau P, Verhest A, De Wolf-Peeters C (1997)
Primary low-grade B-cell lymphoma of MALT-type occurring in the liver: a study of two cases.
J Hepatol 27: 922-7



. IgG4-associated sclerosing cholangitis should be considered in the differential diagnosis of hepatic MALT lymphoma and is usually associated with autoimmune pancreatitis
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Deshpande V, Sainani NI, Chung RT, Pratt DS, Mentha G, Rubbia-Brandt L, Lauwers GY (2009)
IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material.
Mod Pathol 22: 1287-95



.
> Secondary involvement of the liver by low grade B-cell malignancies shows preferential involvement of the portal triads, with the morphology and phenotype related to the specific entity.

The prognosis for patients with primary hepatic lymphoma relates to the specific disease entity. DLBCL is an aggressive disease, with the international prognostic index providing guidance for expected treatment response and outcome

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Memeo L, Pecorello I, Ciardi A, Aiello E, De Quarto A, Di Tondo U (1999)
Primary non-Hodgkin's lymphoma of the liver.
Acta Oncol 38: 655-8




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Shipp MA (1994)
Prognostic factors in aggressive non-Hodgkin's lymphoma: who has "high-risk" disease?
Blood 83: 1165-73




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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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