Cutaneous staging system may predict survival in folliculotropic mycosis fungoides
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Cutaneous staging was the only significant predictive variable of disease-specific survival in patients with folliculotropic mycosis fungoides, according to a study published in JAMA Dermatology.
“Folliculotropic mycosis fungoides (FMF) was recognized as a distinct entity and distinguished from conventional mycosis fungoides by its clinicopathologic features and purportedly worse prognosis,” Yann Charli-Joseph, MD, of the Instituto Nacional de Ciencia Médicas y Nutrición Salvador Zubirán in Mexico City, and colleagues wrote. “Differing survival data for FMF have been reported. ... Therefore, a new FMF-specific staging system, which divides patients between early and advanced cutaneous stages, has been proposed. This system, currently lacking full validation, is considered potentially better associated with survival in FMF.”
To investigate the effectiveness of the staging system for estimating overall survival and disease-specific survival (DSS), 42 patients with FMF (mean age at diagnosis 55 years; 74% men) underwent analysis. Researchers analyzed predictive variables associated with survival using Cox proportional hazards regression modeling and estimated overall survival by subdividing the cohort into early and advanced stage cutaneous disease using Kaplan-Meier curves.
For the entire cohort, estimated 5-year overall survival rate was 89% (95% CI, 79%-99%), estimated 10-year overall survival rate was 78% (95% CI, 63%-92%) and estimated 15-year overall survival rate was 58% (95% CI, 31%-85%). Estimated DSS rates for 5 years and 10 years were 89% (95% CI, 79%-99%), and the estimated 15-year DSS rate was 80% (95% CI, 61%-99%).
Multiple-variable Cox proportional hazards regression models signified age as having a significant association with overall survival (HR per 10-year age increase = 3.1; 95% CI, 1.4-7.2; P = .008) and cutaneous disease being statistically significant for DSS (HR = 11.4; 95% CI, 1.3-103.0; P = .03).
When researchers stratified patients by stage, estimated overall 5- , 10- and 15-year survival rates for early-stage disease were 96% (95% CI, 89%-103%), 82% (95% CI, 65%-98%) and 65% (95% CI, 33%-97%), respectively. For advanced disease, estimated overall survival rates for 5 years and 10 years were 70% (95% CI, 41%-98%), and the estimated 15-year overall survival rate was 53% (95% CI, 16%-89%).
Estimated DSS rates for early-stage cutaneous disease at all timepoints was 96% (95% CI, 89%-103%). Advanced-stage DSS estimates at 5 years and 10 years were 70% (95% CI, 41%-98%) and 53% (95% CI, 16%-89%) at 15 years.
“Cox proportional hazards regression modeling demonstrated cutaneous stage to be the only statistically significant predictive variable associated with DSS,” Charli-Joseph and colleagues wrote. “Subdividing FMF into early and advanced cutaneous stages was associated with effective estimation of survival in this cohort.”