Primary cutaneous B-cell lymphomas: part I. Clinical features, diagnosis, and classification

Abstract

Primary cutaneous B-cell lymphomas (PCBCLs) are defined as lymphomas with a B-cell phenotype that present in the skin without evidence of systemic or extracutaneous disease at initial presentation, after adequate staging. In non-Hodgkin lymphomas, the skin is the second most common site of extranodal involvement after the gastrointestinal tract. PCBCLs are histologically very similar to their nodal counterparts, and these histologic similarities can lead to confusion about both therapy and prognosis. This article will summarize the clinical, pathologic, and diagnostic features of the 3 main types of PCBCL: primary cutaneous follicle center lymphoma, primary cutaneous marginal zone lymphoma, and primary cutaneous diffuse large B-cell lymphoma, leg-type, and the appropriate evaluation and staging procedures for each of these entities.

Keywords: DLBCL; FISH; FOX-P1; Forkhead box P1; MALT; MUM1/INF4; PCBCL; PCDLBCL-LT; PCFCL; PCMZL; SALT; diffuse large B-cell lymphoma; fluorescence in situ hybridization; histopathology; mucosa-associated lymphoid tissue; multiple myeloma-1/interferon regulatory factor-4; primary cutaneous B-cell lymphoma; primary cutaneous diffuse large B-cell lymphoma; primary cutaneous diffuse large B-cell lymphoma, leg type; primary cutaneous follicle center lymphoma; primary cutaneous marginal zone lymphoma; skin-associated lymphoid tissue; staging.