Long-term Outcomes of 1,263 Patients with Mycosis Fungoides and Sézary Syndrome from 1982 to 2009
- 15 September 2012
- journal article
- Published by American Association for Cancer Research (AACR) in Clinical Cancer Research
- Vol. 18 (18), 5051-5060
- https://doi.org/10.1158/1078-0432.ccr-12-0604
Abstract
Purpose: The purpose of this prospectively collected single center study cohort of 1,263 patients with mycosis fungoides (MF)/Sézary syndrome (SS) is to evaluate the significance of stage and risk of disease progression from initial presentation and to examine other prognostic factors. Patients and Methods: The prognostic variables effecting overall survival (OS) were examined in a unique prospective cohort of 1,263 patients with MF and SS seen by one investigator at MD Anderson Cancer Center (Houston, TX) from 1982 to 2009. Kaplan–Meier estimates were used to determine median OS, progression-free survival (PFS), and disease-specific survival (DSS). Cox proportional hazards regression model assessed prognostic factors. Results: Mean age at diagnosis was 55.33 years. Early mycosis fungoides (stage IA–IIA) represented 71.5% (903 of 1,263) and advanced (stage IIB–IVB) 28.5% (360 of 1,263) patients. Progression to a higher stage occurred in 147 patients (11.6%) of whom 112 (12%) were early and 35 (9.7%) advanced. Death from disease occurred in 102 of 1,263 (8.1%) patients. Median OS was 24.44 years, PFS was 16 years, and median DSS was not reached. OS and PFS were significantly better for early-stage patients with patches (T1a/T2a) than with patches/plaques (T1b/T2b). The PFS analyzed in 1,241 patients found that only 337 (27.2%) had disease progression or had died from disease. Risk factors associated with progression or deaths were advanced age, plaque stage, lactate dehydrogenase (LDH) level, and tumor area. Conclusions: Improved outcome of MF/SS, reflected by OS and PFS for all stages, may result from earlier diagnosis, new therapies, and aggressive treatment of infections. Clin Cancer Res; 18(18); 5051–60. ©2012 AACR.Keywords
This publication has 47 references indexed in Scilit:
- Sézary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviorsBlood, 2010
- Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 casesBlood, 2009
- Poor prognosis in non-Caucasian patients with early-onset mycosis fungoidesJournal of the American Academy of Dermatology, 2009
- Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)Blood, 2007
- Phase 2 trial of oral vorinostat (suberoylanilide hydroxamic acid, SAHA) for refractory cutaneous T-cell lymphoma (CTCL)Blood, 2006
- Prognostic factor analysis in mycosis fungoides/Sézary syndromeJournal of the American Academy of Dermatology, 1999
- Combined modality therapy for cutaneous T-cell lymphomaJournal of the American Academy of Dermatology, 1996
- Lymph node classification systems in cutaneous T-cell lymphoma. Evidence for the utility of the working formulation of non-Hodgkin's lymphomas for clinical usageCancer, 1994
- Nonparametric Estimation from Incomplete ObservationsJournal of the American Statistical Association, 1958
- Nonparametric Estimation from Incomplete ObservationsJournal of the American Statistical Association, 1958