Lymphoma - B cell neoplasms - Primary cutaneous diffuse large B-cell lymphoma

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Lymphoma - B cell neoplasms

B cell lymphoma subtypes

Primary cutaneous diffuse large B-cell lymphoma

Reviewer: Nikhil Sangle, M.D., University of Utah and ARUP Laboratories (see Reviewers page)
Revised: 13 March 2011, last major update March 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● By definition, arises in skin with no known extracutaneous disease within 6 months of initial diagnosis
● Other common primary cutaneous B cell lymphomas are follicular lymphoma and marginal zone lymphoma (Mod Pathol 2004;17:623)

Clinical features
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● Represents 20% of primary cutaneous B-cell lymphomas and 4% of all primary cutaneous lymphomas
● Usually men (85%), mean age 64 years (Am J Clin Path 2002;117:574)
● Better outcome than other large B cell lymphomas - relapse common but death rare within 48 months
● CD10+ tumors in head and neck have increased risk of relapse
● Can divide into germinal center type (CD10+, bcl6+, CD138- but without bcl2 translocations) and those with post-germinal center or activated B cell phenotype (more common)

Treatment
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● Surgery and radiotherapy; less aggressive than non-cutaneous tumors

Case reports
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● t(14;18)(q32;q21) involving IgH and MALT1 (not bcl2, distinguish MALT1 from bcl2 by FISH, Hum Pathol 2003; 34:1212)

Gross description
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● Solitary or grouped papules, plaques or tumors, usually in head, neck and trunk of older patients

Micro
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● Infiltrative sheets of non-epidermotropic centroblasts and immunoblasts
● Grenz zone (zone of relatively normal collagen separating epidermis and a dermal lesion) is present
● Occasional smaller B-cells
● May have prominent T cell infiltrate (Mod Pathol 2001;14:10)
● Can divide large B cell lymphomas into 3 groups: (a) monomorphous diffuse large B cell lymphoma, (b) diffuse large B cell lymphoma with small B cells, (c) grade 3 follicular lymphoma (Am J Surg Path 2001;25:307)

Micro images
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Various images

Positive stains (tumor cells)
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● CD20, CD79a, bcl2 and IRF4/MUM1 in 90%, variable bcl6

Negative stains (tumor cells)
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● CD3 (positive in background T-cells), CD10 (usually)

Flow cytometry
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● T cells are 65-90% of cells (Arch Pathol Lab Med 1999;123:1236)

End of Lymphoma - B cell neoplasms > B cell lymphoma subtypes > Primary cutaneous diffuse large B-cell lymphoma

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