Case Report

Diffuse Large B-Cell Lymphoma, Not Otherwise Specified (DLBCL NOS) Presenting as Multiple Subcutaneous Nodules: An Unusual Cutaneous Presentation of Systemic Disease

Table 1

Example differential diagnosis of a primarily cutaneous high-grade B-cell neoplasm with CD5 expression.

High-grade CD5+ B-cell lymphoma with primary cutaneous involvement (select diagnostic considerations and WHO 5th edition criteria)
Primary cutaneous follicle center lymphoma
 Essential
  Follicular and/or diffuse proliferation of centrocytes and admixed centroblasts
  B-cells with coexpression of germinal center markers (e.g., CD10 and BCL6)
  No extracutaneous involvement by lymphoma
 Desirable
  Localization to head or trunk
  Evidence of B-cell monoclonality
  Absent or weak BCL2 expression (usually)
  Lack of MUM1 expression
  Absence of BCL2 rearrangement (usually)
Primary cutaneous diffuse large B-cell lymphoma, leg type
 Essential
  Morphology and phenotype consistent with aggressive B-cell lymphoma
  Mature B-cell phenotype
  Diffuse growth with absence of follicular dendritic cell meshworks
  Skin-confined disease at presentation
 Desirable
  Strong BCL2 expression
  Expression of IgM and MUM1, non-GCB phenotype
High-grade B-cell lymphoma with MYC and BCL2 rearrangements
 Essential
  Morphology and phenotype consistent with aggressive B-cell lymphoma
  Evidence of concurrent MYC and BCL2 rearrangements
 Desirable
  GCB Phenotype
  TdT protein expression status (negative)
  Determination of MYC fusion partner
Mantle cell lymphoma (CD5+)
 Essential
  B-cell immunophenotype with CD5 expression
  Classic or variant (e.g., blastoid or pleomorphic) morphology
  CyclinD1 positive or detection of CCND1 rearrangement
 Desirable
  SOX11 positive
B-lymphoblastic leukemia/lymphoma (lymphoma-type presentation)
 Essential
  Diffuse involvement by a monomorphic population of blasts with a B-cell phenotype (CD19, CD22, cCD79a, and/or PAX5) and markers of immaturity (TdT, CD34, and/or CD10)
 Desirable
  Immunophenotype associated with specific recurrent genetic abnormalities
  Identification of specific recurrent genetic abnormalities