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.
OBJECTIVE
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.


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.


CONCLUSIONS
This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.
Agar
Nita Sally
NS
Guy's and St Thomas' National Health Service (NHS) Foundation Trust, London, United Kingdom.
Wedgeworth
Emma
E
Crichton
Siobhan
S
Mitchell
Tracey J
TJ
Cox
Michael
M
Ferreira
Silvia
S
Robson
Alistair
A
Calonje
Eduardo
E
Stefanato
Catherine M
CM
Wain
Elizabeth Mary
EM
Wilkins
Bridget
B
Fields
Paul A
PA
Dean
Alan
A
Webb
Katherine
K
Scarisbrick
Julia
J
Morris
Stephen
S
Whittaker
Sean J
SJ
D000328:Adult; D000367:Age Factors; D000368:Aged; D000704:Analysis of Variance; D014408:Biomarkers, Tumor / Q000097:blood; D001706:Biopsy; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D018450:Disease Progression; D018572:Disease-Free Survival; D005060:Europe; D005260:Female; D018859:Hair Follicle / Q000473:pathology; D006801:Humans; D007391:International Cooperation; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008198:Lymph Nodes / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000401:mortality / Q000473:pathology / Q000628:therapy; D009367:Neoplasm Staging / Q000379:methods* / Q000592:standards; D011379:Prognosis; D012044:Regression Analysis; D018570:Risk Assessment; D012307:Risk Factors; D012737:Sex Factors; D012751:Sezary Syndrome / Q000401:mortality / Q000473:pathology / Q000628:therapy; D012878:Skin Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D012955:Societies, Medical; D016019:Survival Analysis; D016896:Treatment Outcome
D014408:Biomarkers, Tumor; D007770:L-Lactate Dehydrogenase