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

J Clin Oncol. 2010 Nov 1;28(31):4730-9. doi: 10.1200/JCO.2009.27.7665. Epub 2010 Sep 20.

Abstract

Purpose: We 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 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 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.

Conclusion: This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.

Publication types

  • Research Support, Non-U.S. Gov't
  • Validation Study

MeSH terms

  • Adult
  • Age Factors
  • Aged
  • Analysis of Variance
  • Biomarkers, Tumor / blood
  • Biopsy
  • Cell Transformation, Neoplastic / pathology
  • Disease Progression
  • Disease-Free Survival
  • Europe
  • Female
  • Hair Follicle / pathology
  • Humans
  • International Cooperation
  • L-Lactate Dehydrogenase / blood
  • Lymph Nodes / pathology*
  • Male
  • Middle Aged
  • Mycosis Fungoides* / mortality
  • Mycosis Fungoides* / pathology
  • Mycosis Fungoides* / therapy
  • Neoplasm Staging / methods*
  • Neoplasm Staging / standards
  • Prognosis
  • Regression Analysis
  • Risk Assessment
  • Risk Factors
  • Sex Factors
  • Sezary Syndrome* / mortality
  • Sezary Syndrome* / pathology
  • Sezary Syndrome* / therapy
  • Skin Neoplasms* / mortality
  • Skin Neoplasms* / pathology
  • Skin Neoplasms* / therapy
  • Societies, Medical
  • Survival Analysis
  • Treatment Outcome

Substances

  • Biomarkers, Tumor
  • L-Lactate Dehydrogenase