Primary Cutaneous B-Cell Lymphoma: Review and Current Concepts

Abstract

PCBCL is defined as a B-cell lymphoma originating in the skin. There is no evidence of extracutaneous disease at presentation and for 6 months after diagnosis, as assessed by adequate staging procedures. Currently, the European Organization for Research and Treatment of Cancer classification is the most concise disease classification scheme, dividing the subtypes of PCBCL by clinical behavior and histopathologic findings. Based on this classification, the most common subtype of PCBCL is follicular center cell lymphoma. PCBCL is generally an indolent form of lymphoma with a good prognosis. Although local cutaneous recurrences are observed in 25% to 68% of patients, dissemination to internal organs is rare. Five-year survival rates typically range from 89% to 96%. A specific subtype, large B-cell lymphoma of the leg, is noted to have a poorer prognosis, with a 5-year survival rate of 58%. Overly aggressive treatment of PCBCL has not been shown to improve survival or prevent relapse. The treatment of choice usually varies depending on the type of PCBCL, the body surface area, and the location of the involvement, as well as the age and general health condition of the patient. The majority of studies indicate that PCBCL is highly responsive to radiation therapy. Polychemotherapy should be reserved for involvement of noncontiguous anatomic sites or those with extracutaneous spread.

Cutaneous B‐cell lymphoma: Pathological spectrum and clinical outcome in 51 consecutive patients

“…The EORTC (European Organisation for the Research and Treatment of Cancer) classification of cutaneous lymphoma [5] differs considerably from the WHO in some major areas. One such entity is that of ''large B-cell lymphoma of the leg,'' which is reported as having an unfavorable prognosis compared to other types of PCBCL [4,6,13,15]. In this series, the majority of patients with primary DLBCL and MZL had lesions on the head and neck.…”

“…Primary cutaneous B-cell lymphomas (PCBCL) constitute approximately 20-25% of all cutaneous lymphomas and are defined as B-cell lymphomas originating in the skin with no evidence of extracutaneous disease at presentation and for at least 6 months after diagnosis [1][2][3][4][5][6].…”

EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome

“…Approximately two-thirds of these lymphomas are of T-cell origin, and the pathogenesis and management of both T-and B-cell cutaneous lymphomas have been recently reviewed. [1][2][3][4][5][6] The most common form of cutaneous T-cell lymphoma (CTCL) is mycosis fungoides (MF), accounting for around 60% of new cases. Sé zary syndrome (SS) is much rarer and accounts for only 5% of CTCL cases.…”

Primary Cutaneous Diffuse Large B-Cell Lymphoma – a Case Report

“…PCBCL belongs to a distinct group of lymphoproliferative disorders defined by its presentation in the skin without evidence of extracutaneous spread at the time of diagnosis (8). Extranodal involvement occurs in approximately 25% of non-Hodgkin's lymphomas, with the gastrointestinal tract being the most common site of extranodal involvement, followed by the skin (9).…”