Cutaneous B‐cell lymphomas: 2013 update on diagnosis, risk‐stratification, and management

Abstract

Disease overview: Approximately one‐fourth of cutaneous lymphomas are B‐cell derived and are generally classified into three distinct subgroups: primary cutaneous follicle‐center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL), and primary cutaneous diffuse large B‐cell lymphoma, leg type (PCLBCL, LT). Diagnosis: Diagnosis and disease classification is based on histologic review and immunohistochemical staining of an appropriate skin biopsy. Pathologic review and an appropriate staging evaluation are necessary to distinguish primary cutaneous B‐cell lymphomas from systemic B‐cell lymphomas with secondary skin involvement. Risk‐stratification: Disease histology remains the most important prognostic determinant. Both PCFCL and PCMZL are indolent lymphomas that infrequently disseminate to extracutaneous sites and are associated with an excellent long‐term prognosis. In contrast, PCLBCL, LT is an aggressive lymphoma with an inferior prognosis. Risk‐adapted therapy: PCFCL and PCMZL patients with solitary or relatively few skin lesions may be affectively managed with local radiation therapy. Although single‐agent rituximab may be employed for patients with more widespread skin involvement, multiagent chemotherapy is rarely appropriate. In contrast, management of patients with PCLBCL, LT is comparable to the management of patients with systemic DLBCL. © Am. J. Hematol. 2013. © 2012 Wiley Periodicals, Inc.

Breast Cancer Mimic: Cutaneous B-Cell Lymphoma Presenting as an Isolated Breast Mass

“…The two former subtypes are recognized as indolent lymphomas, while the latter has a more intermediate behavior [10, 11]. The following discussion will focus on pCMZL, which is the subtype of pCBCL in our patient.…”

Primary cutaneous B-cell lymphoma and chronic leg ulcers in a patient with type 2 diabetes

“…Classically, the identification of a primary cutaneous lymphoma necessitates that there is no extracutaneous disease involved at the moment of the diagnosis (4). Although the cutaneous T-cell lymphomas (CTCLs) are the more common subtype, the cutaneous B-cell lymphomas (CBCLs) range from 25% to 30% of all primary cutaneous lymphomas (5, 6, 7). To note, the primary cutaneous diffuse large B-cell lymphoma (PCDLBCL), leg type, is a rare and aggressive lymphoma, which represents approximately 4% of all cutaneous lymphomas and 20% of all primary CBCLs (5, 6, 7).…”

“…Although the cutaneous T-cell lymphomas (CTCLs) are the more common subtype, the cutaneous B-cell lymphomas (CBCLs) range from 25% to 30% of all primary cutaneous lymphomas (5, 6, 7). To note, the primary cutaneous diffuse large B-cell lymphoma (PCDLBCL), leg type, is a rare and aggressive lymphoma, which represents approximately 4% of all cutaneous lymphomas and 20% of all primary CBCLs (5, 6, 7). PCDLBCL often affects elderly individuals and females seem to be more affected than male (5, 6, 7).…”

“…To note, the primary cutaneous diffuse large B-cell lymphoma (PCDLBCL), leg type, is a rare and aggressive lymphoma, which represents approximately 4% of all cutaneous lymphomas and 20% of all primary CBCLs (5, 6, 7). PCDLBCL often affects elderly individuals and females seem to be more affected than male (5, 6, 7). Traditionally, the cutaneous manifestation of this lymphoma is a red or violaceous tumors that becomes apparent on a leg (4).…”

Primary Cutaneous B-Cell Lymphoma: Management and Patterns of Recurrence at the Multimodality Cutaneous Lymphoma Clinic of The Ohio State University

“…The skin is the second most common extranodal site at presentation after the gastrointestinal tract [1]. In contrast to indolent PCBCL, diffuse large B-cell lymphoma with secondary cutaneous involvement carries a worse prognosis with a 5-year overall survival comparable to that of PCBCL-LT [7]. To date, no molecular, histologic, or clinical criteria are available that can consistently distinguish PCBCL from sBCL with cutaneous involvement.…”