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

American Society of Clinical Oncology (ASCO) - Tập 28 Số 31 - Trang 4730-4739 - 2010
Nita Agar1, E. Wedgeworth1, Siobhan Crichton1, Tracey J. Mitchell1, Michael C. Cox1, Silvia Ferreira1, Alistair Robson1, Eduardo Calonje1, Catherine M. Stefanato1, E. Mary Wain1, Bridget S. Wilkins1, Paul Fields1, Alan Dean1, Katherine A. Webb1, Julia Scarisbrick1, Stephen Morris1, Sean Whittaker1
1From the Guy's and St Thomas' National Health Service (NHS) Foundation Trust; King's College London; Integrated Cancer Research (ICARSIS), London; and Norfolk and Norwich University Hospitals, Norwich, United Kingdom.

Tóm tắt

PurposeWe have analyzed the outcome of mycosis fungoides (MF) and Sézary 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 MethodsOverall 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.ResultsThe 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 Sézary 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.ConclusionThis study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.

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American Society of Clinical Oncology (ASCO)

Tập 28 Số 31

4730-4739

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