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Giornale Italiano di Dermatologia e Venereologia 2006 April;141(2):139-54

Copyright © 2006 EDIZIONI MINERVA MEDICA

language: English

Management of cutaneous B-cell lymphomas

Cerroni L.

Department of Dermatology Medical University of Graz, Graz, Austria

Primary cutaneous B-cell lymphomas (PCBCLs) represent an heterogeneous group of lymphoid malignancies with various clinicopathologic presentation and prognosis. Primary cutaneous lymphomas are defined as malignant lymphomas confined to the skin at presentation after complete staging procedures. Thus, staging investigations are mandatory for all patients. The classification of cutaneous lymphomas published by the World Health Organization (WHO) and the European Organization for Research and Treatment of Cancer (EORTC) – Cutaneous Lymphoma Project Group recognizes four main types of PCBCL: follicle center lymphoma and marginal zone B-cell lymphoma are in a group characterized by an indolent clinical behavior (five-year survival >90%), whereas diffuse large B-cell lymphoma, leg type, and diffuse large B-cell lymphoma, other (this last including several rare variants) are in a group with intermediate clinical behavior (five-year survival 50-60%). Management of patients depend on precise classification as well as on clinical presentation (e.g., solitary or multiple tumors, age, general conditions, etc.). The main therapeutic strategies for PCBCLs include local radiotherapy, surgical excision, anti-CD20 antibody (rituximab), interferon-alfa, and systemic chemotherapy (usually CHOP). It must be stressed that systemic chemotherapy is needed only rarely in cases of PCBCL with indolent behaviour. Cases associated with infection by Borrelia burgdorferi may be managed with antibiotic treatment. Selected patients may be followed-up at regular intervals according to a so-called “watchful waiting” strategy.