Primary cutaneous B-cell lymphoma: subtypes, treatment and outcome of 10 cases.

Primary cutaneous B-cell lymphomas (CBCLs) are divided into three main types according to the WHO-EORTC classification: marginal zone B-cell lymphoma (MZL), follicle centre lymphoma (FCL), and diffuse large B-cell lymphoma, leg-type (DLBL) (1–4). A new tumour, lymph node, metastasis (TNM) staging system (Table SI (available from: other than mycosis fungoides and Sezary syndrome was recently proposed. The new system provides documentation of disease extent/distribution and can be used to select appropriate treatment (3, 5). The aims of our study on 10 CBCL cases were: (i) to characterize the clinicopathological features of the three subtypes of CBCL; (ii) to assess the value of the new TNM staging system; (iii) to evaluate response to the various treatment modalities and (iv) to assess the prognosis of CBCL with tumour staging. PATIENTS AND METHODS We reviewed the cases of 10 patients who received a diagnosis of CBCL between 1997 and 2007. These were classified according to the WHO-EORTC classification for CLs (Table SI). All patients had undergone complete staging, including complete blood cell count, chemistry panel, ultrasound examination of lymph nodes, and computed tomography scans of chest, abdomen , and pelvis, as well as bone marrow biopsy, as suggested by the ISCL-EORTC (5). Histological review was performed on haematoxylin-eosin stained sections, immunohistochemical staining including B-cell markers (CD20, CD79a), and at least one T-cell marker (usually CD3, CD5 or both), with additional staining for bcl2, bcl6, CD10, MUM-1 (in DLBCL). Positivity for bcl2, bcl6 on 1 MUM-1 was defined as expression of those markers by at least 50% of the tumour cells. Medical records were reviewed for demographic data, loca-lization, number, and extent of lesions at the time of diagnosis, according to the new TNM staging scheme (5) and for diagnosis according to the WHO-EORTC classification for CLs (1, 4). We reviewed serological tests for Borrelia infection, treatment modalities and response. RESULTS The CBCL patients were classified as MZL (n = 4), FCL (n = 3) and DLBL (n = 3) (Table I). The MZL patients TNM stages were T 1 a, T 2 a and T 2 b (mean age 49 years). The predilection sites for MZL were the head-neck (n = 2) and upper back (n = 2). Clinically, MZL began with pink, red, or red-brown nodules (Fig. 1a). The patients were treated with surgical excision or intralesional or systemic interferon (IFN)-α.Complete remission (CR) was achieved in all patients with MZL. Extracutaneous spread …