Primary cutaneous B-cell lymphomas: part II. Therapy and future directions

Abstract

The choice of therapy for primary cutaneous B-cell lymphoma (PCBCL) relies on correct histopathologic classification and the exclusion of systemic disease. In part II of this continuing medical education article, we will review the available therapies for the different types of PCBCL. Primary cutaneous follicle center lymphoma (PCFCL) and primary cutaneous marginal zone lymphoma (PCMZL) are indolent tumors with an excellent prognosis. They are managed similarly with local therapy, such as radiotherapy or surgical excision, for isolated disease and observation for asymptomatic multifocal presentations. Relapses are common in both PCFCL and PCMZL, but overall survival remains excellent. Primary cutaneous diffuse large B-cell lymphoma (both leg type and other) has a much poorer prognosis than indolent PCBCL, and it often requires an aggressive approach with radiation therapy and/or multiagent chemotherapy. Investigational approaches hold promise for the treatment of these malignancies, particularly primary cutaneous diffuse large B-cell lymphoma.

Keywords: CBCL; CHOP; DLBCL; EORTC-CLG; European Organisation for Research and Treatment of Cancer Cutaneous Lymphoma Group; FISH; MALT; NCCN; National Comprehensive Cancer Network; PCBCL; PCDLBCL-LT; PCFCL; PCMZL; R-CHOP; cutaneous B-cell lymphoma; cyclophosphamide, doxorubicin, onvocin/vincristine, and prednisone; cyclophosphamide, doxorubicin, onvocin/vincristine, and prednisone with rituximab; diffuse large B-cell lymphoma; fluorescence in situ hybridization; lymphoma; mucosa-associated lymphoid tissue; 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; rituximab; therapy.