Prognostic factors in primary cutaneous large B-cell lymphomas: A Europeanmulticenter study

Purpose: Most primary cutaneous B-cell lymphomas have an excellent prognosi s. However, primary cutaneous large B-cell lymphomas (PCLBCLs) of the leg h ave been recognized as a distinct entity with a poorer prognosis in the Eur opean Organization for Research and Treatment of Cancer (EORTC) classificat ion. This distinction on the basis of site has been debated. Our aim was to identify independent prognostic factors in a large European multicenter se ries of PCLBCL. Patients and Methods: The clinical and histologic data of 145 patients with PCLBCL were evaluated. According to the EORTC classification, 48 patients had a PCLBCL of the leg and 97 had a primary cutaneous follicle center-cell lymphoma (PCFCCL). Data from both groups were compared. Univariate and mul tivariate analyses of specific survival were performed using a Cox proporti onal hazards model. Results: Compared with PCFCCL, PCLBCL-leg were characterized by an older ag e of onset, a more recent history of skin lesions, a more frequent predomin ance of tumor cells with round nuclei and positive bcl-2 staining, and a po orer 5-year disease-specific survival rate (52% v 94%; P < .0001). Univaria te survival analysis in the entire study group showed that older age, a mor e recent onset of skin lesions, the location on the leg, multiple skin lesi ons, and the round-cell morphology were significantly related to death. in multivariate analysis, the round-cell morphology (P < .0001), the location on the leg (P = .002), and multiple skin lesions (P = .01) remained indepen dent prognostic factors. The round-cell morphology was an adverse prognosti c factor both in PCLBCL-leg and in PCFCCL, whereas multiple skin lesions we re associated with a poor prognosis only in patients with PCLBCL-leg. Conclusion: With site, morphology, and number of tumors taken into account, guidelines for the management of PCLBCL are presented. J Clin Oncol 19:3602-3610. (C) 2001 by American Society of Clinical Oncolog y.