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Approach to Mycosis Fungoides in children: Consensus-based recommendations
Alex Zvulunov1, Holly Neale2, Jonah Stern3
1Sheba Medical Center, Tel-Hashomer and Reichman University, Herzlia, Israel; Pediatric Dermatology Research Alliance, Portland, Oregon.
Insights
Pediatric Mycosis fungoides (MF) management now has new consensus recommendations. These guidelines incorporate clinical severity, quality of life, and psychological factors for early and advanced pediatric MF cases.
Area of Science:
- Dermatology
- Pediatric Oncology
Background:
- Current pediatric Mycosis fungoides (MF) management relies on adult guidelines, overlooking distinct clinical presentations in children.
- Development of specific recommendations is crucial for effective pediatric MF care.
Purpose of the Study:
- To establish consensus-based management recommendations tailored for pediatric Mycosis fungoides (MF).
Main Methods:
- An international panel of 83 experts in pediatric dermatology, dermatology, dermatopathology, and pediatric hematology-oncology participated.
- A three-round electronic Delphi process followed by a modified Nominal Group Technique meeting refined the recommendations.
Main Results:
- Consensus recommendations were established for managing early and advanced pediatric MF.
- Severity assessment now includes pruritus, functional/esthetic impact, quality of life, and psychological distress, alongside parental anxiety, exceeding traditional staging.
- Therapeutic choices for stages beyond MF Stage I showed some disagreement among experts.
Conclusions:
- A multinational effort successfully generated consensus recommendations to standardize pediatric MF management.
- Further research is required for structured, prospective protocols, particularly for advanced-stage pediatric MF.
- Systematic, prospective data collection is essential for advancing pediatric MF treatment.
Background:
Pediatric Mycosis fungoides (MF) management extrapolates from adult guidelines, despite differing clinical aspects. Recommendations are essential to address unique challenges in this distinct patient group.
Objective:
This project aims to derive consensus recommendations for pediatric MF management.
Methods:
Experts from pediatric dermatology, general dermatology, dermatopathology, and pediatric hematology-oncology (N = 83) were invited to contribute to consensus recommendations. The process involved 3 electronic Delphi rounds, concluding with a final consensus meeting using a modified Nominal Group Technique for unresolved items.
Results:
Consensus included more clinical severity measures than tumor-node-metastasis-blood staging: pruritus, functional or esthetic impairment (eg, palms, soles, genitalia), quality of life impact, and psychological aspects (eg, embarrassment, anxiety, depression), plus parental anxiety. Ten recommendations were made for managing early and advanced pediatric MF. Disagreement emerged in choosing therapies beyond stage I of the disease.
Discussion:
This multinational initiative aimed to standardize optimal pediatric MF management and successfully generated consensus recommendations. Additional work is needed for structured, prospective protocols in advanced-stage pediatric MF.
Limitations:
Lack of pediatric hematologists-oncologists and patients' representatives.
Conclusion:
Documentation of extended clinical severity and outcome measures is recommended. Addressing the need for structured protocols in advanced-stage pediatric MF and implementing systematic, prospective data collection is crucial.
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