Journal
JOURNAL OF CLINICAL ONCOLOGY
Volume 28, Issue 31, Pages 4730-4739Publisher
AMER SOC CLINICAL ONCOLOGY
DOI: 10.1200/JCO.2009.27.7665
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Funding
- Department of Health via the National Institute for Health Research Comprehensive Biomedical Research Centre
- Guy's and St Thomas' NHS Foundation Trust
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Purpose We have analyzed the outcome of mycosis fungoides (MF) and Sezary syndrome (SS) patients using the recent International Society for Cutaneous Lymphomas (ISCL)/European Organisation for Research and Treatment of Cancer (EORTC) revised staging proposal. Patients and Methods Overall survival (OS), disease-specific survival (DSS), and risk of disease progression (RDP) were calculated for a cohort of 1,502 patients using univariate and multivariate models. Results The mean age at diagnosis was 54 years, and 71% of patients presented with early-stage disease. Disease progression occurred in 34%, and 26% of patients died due to MF/SS. A significant difference in survival and progression was noted for patients with early-stage disease having patches alone (T1a/T2a) compared with those having patches and plaques (T1b/T2b). Univariate analysis established that (1) advanced skin and overall clinical stage, increased age, male sex, increased lactate dehydrogenase (LDH), and large-cell transformation were associated with reduced survival and increased RDP; (2) hypopigmented MF, MF with lymphomatoid papulosis, and poikilodermatous MF were associated with improved survival and reduced RDP; and (3) folliculotropic MF was associated with an increased RDP. Multivariate analysis established that (1) advanced skin (T) stage, the presence in peripheral blood of the tumor clone without Sezary cells (B0b), increased LDH, and folliculotropic MF were independent predictors of poor survival and increased RDP; (2) large-cell transformation and tumor distribution were independent predictors of increased RDP only; and (3) N, M, and B stages; age; male sex; and poikilodermatous MF were only significant for survival. Conclusion This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors. J Clin Oncol 28: 4730-4739. (C) 2010 by American Society of Clinical Oncology
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