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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; 28(31):4730-9JC

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.

Authors+Show Affiliations

Guy's and St Thomas' National Health Service (NHS) Foundation Trust, London, United Kingdom.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

20855822

Citation

Agar, Nita Sally, et al. "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." Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology, vol. 28, no. 31, 2010, pp. 4730-9.
Agar NS, Wedgeworth E, Crichton S, et al. 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;28(31):4730-9.
Agar, N. S., Wedgeworth, E., Crichton, S., Mitchell, T. J., Cox, M., Ferreira, S., ... Whittaker, S. J. (2010). 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. Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology, 28(31), pp. 4730-9. doi:10.1200/JCO.2009.27.7665.
Agar NS, et al. 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. PubMed PMID: 20855822.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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. 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 A, AU - Dean,Alan, AU - Webb,Katherine, AU - Scarisbrick,Julia, AU - Morris,Stephen, AU - Whittaker,Sean J, Y1 - 2010/09/20/ PY - 2010/9/22/entrez PY - 2010/9/22/pubmed PY - 2010/11/10/medline SP - 4730 EP - 9 JF - Journal of clinical oncology : official journal of the American Society of Clinical Oncology JO - J. Clin. Oncol. VL - 28 IS - 31 N2 - 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. SN - 1527-7755 UR - https://www.unboundmedicine.com/medline/citation/20855822/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_ L2 - http://ascopubs.org/doi/full/10.1200/JCO.2009.27.7665?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -