Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides

Santen, Suzanne van; Roach, Rachel E J; Doorn, Remco van; Hórvath, Barbara; Bruijn, M. S.; Sanders, C. J. G.; Pooter, Jacco C. de; Rossum, Michelle M. van; Haas, E; Veraart, J. C. J. M.; Bekkenk, Marcel W.; Vermeer, Maarten H.; Willemze, Rein

doi:10.1001/jamadermatol.2016.1597

Cited by 129 publications

(215 citation statements)

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“…Predictors of poor survival were advanced age, male sex, and stage >IB. Our study supports the previous literature, which suggests that advanced age and male sex are associated with poor survival [2,5,[13][14][15][16] . This is in keeping with the cutaneous lymphoma international prognostic index (CLIPi) for MF and SS, which lists male gender and age >60 as significant adverse prognostic factors [17] .…”

Section: Discussionsupporting

confidence: 92%

“…We used PUVA as the initial treatment for most patients with widespread MF. Sixty-five percent of the patients in the study were treated with PUVA, and PUVA was used more frequently compared to other studies [2,5] . One of the possible causes of poorer survival for FMF patients in other cohorts could be the early use of aggressive chemotherapy and associated morbidity and mortality.…”

Section: Discussionmentioning

confidence: 96%

“…sive to skin-directed therapies compared to classic MF, and aggressive treatments have been advocated in early stages of FMF [2,3,5,12] . However, recent data revealed a more complex relationship between folliculotropism and clinical outcomes.…”

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confidence: 99%

“…It has been suggested that there is a group of FMF patients for whom the disease is more indolent, and the prognosis is like that of classic MF [4,9] . A distinction can also be made between patients with advanced FMF and early FMF, with the latter having a favorable treatment response and prognosis [5] . The heterogeneity of this disease is supported by the wide range of reported survival data for FMF.…”

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confidence: 99%

“…No study has reported an association between treatment response and OS. Also, until very recently there were no specific treatment guide- [5] . The aim of the current study is to investigate the impact of treatment, demographic, and clinical factors on survival for 218 patients with MF, FMF, and Sézary syndrome (SS) at a single center, and to perform a meta-analysis of existing data to elucidate the importance of folliculotropism as a prognostic factor in MF.…”

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confidence: 99%

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Folliculotropism Does Not Affect Overall Survival in Mycosis Fungoides: Results from a Single-Center Cohort and Meta-Analysis

Giberson

Mourad²,

Gniadecki³

2017

Dermatology

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Background: Folliculotropic mycosis fungoides (FMF) is a distinct subtype of mycosis fungoides (MF) with unique clinicopathological features. The medical literature suggests that FMF has a more aggressive course and worse survival than classic MF. Previous studies do not use standardized treatment, and no studies have reported an association between treatment response and overall survival (OS). Objective: To compare OS for MF, FMF, and Sézary syndrome (SS) patients. Methods: Data were collected retrospectively from 218 patients (171 MF, 15 SS, 32 FMF) treated in a single academic center between 1970 and 2016. Results: Negative predictors of OS were age (OR = 1.07), male sex (OR = 1.63), and stage IIB, III, and IV (OR = 4.10, 5.42, and 7.54, respectively, vs. stage IA). Lack of initial PUVA response was strongly associated with negative OS (OR = 3.08). Kaplan-Meier analysis of age-, sex-, and stage-matched MF and FMF patients found similar OS between the 2 groups. The 5-year OS was 91% for FMF and 74% for MF. Meta-analysis of current data and 2 published studies where survival of FMF patients was compared to MF did not reveal statistically significant differences between these 2 diseases. Conclusions: When patients were matched for age, sex, and disease stage, folliculotropism did not affect OS in MF.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 96%

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confidence: 99%

mentioning

confidence: 99%

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confidence: 99%

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Folliculotropism Does Not Affect Overall Survival in Mycosis Fungoides: Results from a Single-Center Cohort and Meta-Analysis

Giberson

Mourad²,

Gniadecki³

2017

Dermatology

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An Indurated Plaque on the Eyelid

Chen

Yao

2020

JAMA Dermatol

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A man in his 60s presented with a 1-year history of a pruritic, dark red plaque on his right upper eyelid. No remarkable medical history was noted. Physical examination findings revealed a 4 × 2-cm erythematous, violaceous, indurated, scaly plaque with some papules on his right upper eyelid (Figure , A). Results of routine blood tests and biochemistry analysis were within normal limits. A biopsy sample was obtained for histopathologic examination (Figure , B).Histopathologic analysis revealed perifollicular lymphocytic infiltrates around cystically dilated follicular infundibula and exocytosis of atypical lymphocytes into the epithelium, with admixed plasma cells, eosinophils, and multinucleate giant cells (Figure , C). Alcian blue staining showed focal mild follicular mucin deposition. Immunohistochemical analysis showed lymphocytic infiltrates positive for CD3, CD4, CD5, and CD7 and negative for CD8, CD20, CD30, CD79a, and CD56 (Figure , D). The ratio of CD4 to CD8 cells within hair follicles was greater than 7:1. Monoclonal T-cell gene rearrangement was demonstrated.

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Association of a Proposed New Staging System for Folliculotropic Mycosis Fungoides With Prognostic Variables in a US Cohort

et al. 2021

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IMPORTANCE A new cutaneous staging system for folliculotropic mycosis fungoides (FMF) has been purported to better estimate survival compared with the staging system for conventional mycosis fungoides.OBJECTIVE To analyze predictive variables associated with survival and evaluate the effectiveness of the newly proposed staging system for estimating overall survival and disease-specific survival (DSS) in a US cohort. DESIGN, SETTING, AND PARTICIPANTSThis cohort study assessed 195 patients with FMF in the dermatopathology database of the University of California, San Francisco from January 1, 1990, to April 31, 2012, for eligibility. A total of 153 patients were excluded for the following reasons: (1) alternative diagnoses were favored ranging from benign dermatitides to other forms of cutaneous lymphoma; (2) technical problems with slides; and (3) lack of follow-up information. Data were analyzed from January 1, 2018, to August 31, 2020.MAIN OUTCOMES AND MEASURES Kaplan-Meier curves were used to estimate overall survival and DSS for the entire cohort. Possible predictive variables associated with survival were evaluated using Cox proportional hazards regression modeling. Each variable was examined separately, followed by a multiple-variable model. Kaplan-Meier curves were used to estimate overall survival and DSS by subdividing the cohort into early-and advanced-stage cutaneous disease.RESULTS Forty-two patients were included in the analysis (mean age at diagnosis, 55 [range, 8-89] years; 31 men [74%]). For the entire cohort, the estimated 5-year overall survival rate was 89% (95% CI, 79%-99%); 10-year rate, 78% (95% CI, 63%-92%); and 15-year rate, 58% (95% CI, 31%-85%). Estimated 5-and 10-year DSS rates were 89% (95% CI, 79%-99%); 15-year rate, 80% (95% CI, 61%-99%). For overall survival in the multiple-variable Cox proportional hazards regression model, only age was statistically significant (hazard ratio [HR] per 10-year age increase, 3.1; 95% CI, 1.4-7.2; P = .008), whereas for DSS, only cutaneous disease was statistically significant (HR, 11.4; 95% CI, 1.3-103.0; P = .03). When stage stratified, the 5-year estimated overall survival rate for early-stage disease was 96% (95% CI, 89%-103%); 10-year rate, 82% (95% CI, 65%-98%); and 15-year rate, 65% (95% CI, 33%-97%). For advanced-stage disease, the estimated 5-and 10-year overall survival rates were 70% (95% CI, 41%-98%); the 15-year rate, 53% (95% CI, 16%-89%). For early-stage cutaneous disease, the estimated 5-, 10-and 15-year DSS rates were all 96% (95% CI, 89%-103%). For advanced-stage cutaneous disease, the estimated 5-and 10-year DSS rates were 70% (95% CI, 41%-98%); the 15-year rate, 53% (95% CI, 16%-89%). CONCLUSIONS AND RELEVANCECox proportional hazards regression modeling demonstrated cutaneous stage to be the only statistically significant predictive variable associated with DSS. Subdividing FMF into early and advanced cutaneous stages was associated with effective estimation of survival in this cohort. Thus, findings suggest that FMF is a heterogene...

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Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides

Cited by 129 publications

References 71 publications

Folliculotropism Does Not Affect Overall Survival in Mycosis Fungoides: Results from a Single-Center Cohort and Meta-Analysis

Folliculotropism Does Not Affect Overall Survival in Mycosis Fungoides: Results from a Single-Center Cohort and Meta-Analysis

An Indurated Plaque on the Eyelid

Association of a Proposed New Staging System for Folliculotropic Mycosis Fungoides With Prognostic Variables in a US Cohort

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