Lymphoma and plasma cell neoplasms
B cell lymphoma subtypes
Primary cutaneous diffuse large B cell lymphoma

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 3 April 2017, last major update March 2011

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PubMed Search: primary cutaneous diffuse large B cell lymphoma

Cite this page: Primary cutaneous diffuse large B cell lymphoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomaprimarycutdiffuse.html. Accessed April 24th, 2017.
Definition / General
  • 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
  • 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)
Case Reports
  • t(14;18)(q32;q21) involving IgH and MALT1 (not BCL2, distinguish MALT1 from BCL2 by FISH, Hum Pathol 2003;34:1212)
Treatment
  • Surgery and radiotherapy; less aggressive than noncutaneous tumors
Gross Description
  • Solitary or grouped papules, plaques or tumors, usually in head, neck and trunk of older patients
Micro Description
  • Infiltrative sheets of nonepidermotropic 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
Negative Stains
  • CD3 (positive in background T cells), CD10 (usually)
Flow Cytometry Description