Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome: Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal

TY - JOUR

T1 - Survival Outcomes and Prognostic Factors in Mycosis Fungoides/Sezary Syndrome

T2 - Validation of the Revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal

AU - Agar, Nita Sally

AU - Wedgeworth, Emma

AU - Crichton, Siobhan

AU - Mitchell, Tracey J.

AU - Cox, Michael

AU - Ferreira, Silvia

AU - Robson, Alistair

AU - Calonje, Eduardo

AU - Stefanato, Catherine M.

AU - Wain, Elizabeth Mary

AU - Wilkins, Bridget

AU - Fields, Paul

AU - Dean, Alan

AU - Webb, Katherine

AU - Scarisbrick, Julia

AU - Morris, Stephen

AU - Whittaker, Sean J.

PY - 2010/11/1

Y1 - 2010/11/1

N2 - 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.

AB - 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.

U2 - 10.1200/JCO.2009.27.7665

DO - 10.1200/JCO.2009.27.7665

M3 - Article

SN - 1527-7755

VL - 28

SP - 4730

EP - 4739

JO - Journal of Clinical Oncology

JF - Journal of Clinical Oncology

IS - 31

ER -