ISCL/EORTC-CLTG/USCLC recommendations for the diagnosis, staging and treatment of early-stage paediatric mycosis fungoides: a modified Delphi consensus : part one: diagnosis and staging : guidelines
Mycosis fungoides (MF) is considered rare in children and adolescents. Accumulating experience indicates that compared with adult patients, almost all paediatric patients with MF are diagnosed at an early stage of disease, exhibit higher rates of atypical presentations and follow a notably indolent...
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| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
September 2025
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| In: |
British journal of dermatology
Year: 2025, Volume: 193, Issue: 3, Pages: 405-414 |
| ISSN: | 1365-2133 |
| DOI: | 10.1093/bjd/ljaf199 |
| Online Access: | Verlag, kostenfrei, Volltext: https://doi.org/10.1093/bjd/ljaf199
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| Author Notes: | Emmilia Hodak, Iris Amitay-Laish, Martine Bagot, Maxime Battistella, Dan Ben Amitai, Antonio Cozzio, Madeleine Duvic, Katalin Ferenczi, Francine Foss, Larisa Geskin, Emmanuella Guenova, Youn H Kim, Mark Jean-Aan Koh, Montserrat Molgó, Arti Nanda, Jan P Nicolay, Tim Niehues, Pablo Ortiz-Romero, Amra Osmancevic, Evangelia Papadavid, Jong Bin Park, Pietro Quaglino, José A Sanches, Julia Scarisbrick, Maria Luisa Sulis, Rudolf Stadler, Franz Trautinger, Maarten Vermeer, Iris Wohlmuth, John A Zic and Chalid Assaf |
| Summary: | Mycosis fungoides (MF) is considered rare in children and adolescents. Accumulating experience indicates that compared with adult patients, almost all paediatric patients with MF are diagnosed at an early stage of disease, exhibit higher rates of atypical presentations and follow a notably indolent course. Despite the need for special staging/investigations specific for children and adolescents, the staging of paediatric MF is currently based primarily on standard practice in adults. The aim of this work was to develop staging recommendations specifically designed for MF in children and adolescents, on behalf of the three largest societies for cutaneous lymphomas, the International Society for Cutaneous Lymphomas, the European Organisation for Research and Treatment of Cancer - Cutaneous Lymphoma Tumor Group, and the United States Cutaneous Lymphoma Consortium. We developed this guideline through an international expert consensus process and in accordance with the EQUATOR Network’s recommendations for guideline development. A modified Delphi process was conducted, using questionnaires covering topics including the definition, characteristics and staging of paediatric MF. Two rounds of expert feedback were conducted, with an additional hybrid consensus meeting. We reached a consensus that the term ‘paediatric MF’ should be reserved for MF diagnosed at ≤ 18 years of age. Panellists specified the unique clinical characteristics of paediatric MF, including a high prevalence of hypopigmented and folliculotropic variants, and reached a consensus on the indolent nature of the disease during childhood and adolescence with rare progression to advanced stage. The consensus reached recommends that while the staging approach is largely similar to that in adults, imaging for early-stage paediatric MF should rely on ultrasound of the lymph nodes if indicated, rather than computed tomography with contrast or integrated with positron emission tomography. Along with this, clinicians should be aware that in children, palpable lymph nodes are common owing to the increased incidence of infectious diseases. In summary, this guideline addresses the major clinical characteristics of paediatric MF, which differ from those in adults, and provides practical staging recommendations considering the safety implications specific for this age group.Mycosis fungoides (MF) is one of a group of skin cancers called skin lymphomas. Cases of MF in younger children (‘paediatric MF’) are rare, and the disease is mostly managed as for adults with MF. We have learned that paediatric MF is different from adult MF. Firstly, most children are diagnosed with MF at an early disease stage. Secondly, MF in children shows higher rates of more unusual symptoms. Thirdly, paediatric MF is characterized by a slow progression if at all, so that the scans typically used in adults may be unnecessary and carry risks of harm in children.Because of these differences, we have developed recommendations specially for younger people. We assembled a panel of different medical specialists from 17 countries. They all manage patients with skin lymphomas. The panellists used a special agreement process to decide which recommendations to include. Panellists also agreed on the characteristics of paediatric MF that make it distinct from adult MF. These include an MF type where skin lesions are paler, and a type involving hair follicles. Panellists agreed that paediatric MF rarely progresses to advanced stages.There are safety concerns surrounding tests done on adults to classify the disease stage (‘staging’). Therefore, for staging paediatric MF, we recommended tests that do not carry the risks of scans done on adults. We recommended a lower-risk scan called an ultrasound, done on the lymph nodes, to see if they are enlarged. Children are already more likely to have infectious diseases, which can cause lymph nodes to enlarge. Doctors should keep this in mind.In summary, this guideline describes the main clinical characteristics of paediatric MF that make it different from adult MF. It also recommends safer scans for the staging process in children. |
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| Item Description: | Online veröffentlicht: 27. Mai 2025 Gesehen am 04.09.2025 |
| Physical Description: | Online Resource |
| ISSN: | 1365-2133 |
| DOI: | 10.1093/bjd/ljaf199 |