Primary cutaneous B-cell lymphomas : Part II. Therapy and future directions - 17/08/13

Doi : 10.1016/j.jaad.2013.06.011

Andrea Luísa Suárez, MD, PhD ^a,^e, Christiane Querfeld, MD, PhD ^b,^e, Steven Horwitz, MD ^c,^e, Melissa Pulitzer, MD ^d,^e, Alison Moskowitz, MD ^c,^e, Patricia L. Myskowski, MD ^a,^b,^e,^⁎
^a Department of Dermatology, New York–Presbyterian Hospital/Weill Cornell Medical Center, New York, New York
^b Dermatology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York
^c Lymphoma Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York
^d Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York
^e Weill Cornell Medical College, New York, New York

^∗Correspondence to: Patricia L. Myskowski, MD, Dermatology Service, Department of Medicine, Rockefeller Outpatient Pavilion, Memorial Sloan-Kettering Cancer Center, 160 E 53rd St, New York, NY 10022.

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.

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Key words : lymphoma, primary cutaneous B-cell lymphoma, primary cutaneous diffuse large B-cell lymphoma, primary cutaneous follicle center lymphoma, primary cutaneous marginal zone lymphoma, rituximab, therapy

Abbreviations used : CBCL, CHOP, DLBCL, EORTC-CLG, FISH, MALT, NCCN, PCBCL, PCDLBCL-LT, PCFCL, PCMZL, R-CHOP