EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2023.

Pablo L Ortiz-Romero, Franz Trautinger, Rein Willemze, Sean Whittaker, Ulrike Wehkamp, Maarten H Vermeer, Liisa Väkevä, Rudolf Stadler, Julia Scarisbrick, Annamari Ranki, Pietro Quaglino, Nicola Pimpinelli, Evangelia Papadavid, Johanna Latzka, Jan P Nicolay, Stephen Morrris, Robert Knobler, Claus-Detlev Klemke, Constanze Jonak, Emmilia Hodak, Robert Gniadecki, Emmanuella Guenova, Reinhard Dummer, Antonio Cozzio, Martine Bagot, Chalid Assaf

Journal: European journal of cancer (Oxford, England : 1990) 2023;195():113343

PMID: 37890355

Free Full Text on Elsevier Science View on PubMed

Abstract

On behalf of the EORTC Cutaneous Lymphoma Tumours Group (EORTC-CLTG) and following up on earlier versions published in 2006 and 2017 this document provides an updated standard for the treatment of mycosis fungoides and Sézary syndrome (MF/SS). It considers recent relevant publications and treatment options introduced into clinical practice after 2017. Consensus was established among the authors through a series of consecutive consultations in writing and a round of discussion. Treatment options are assigned to each disease stage and, whenever possible and clinically useful, separated into first- and second line options annotated with levels of evidence. Major changes to the previous version include the incorporation of chlormethine, brentuximab vedotin, and mogamulizumab, recommendations on the use of pegylated interferon α (after withdrawal of recombinant unpegylated interferons), and the addition of paragraphs on supportive therapy and on the care of older patients. Still, skin-directed therapies are the most appropriate option for early-stage MF and most patients have a normal life expectancy but may suffer morbidity and impaired quality of life. In advanced disease treatment options have expanded recently. Most patients receive multiple consecutive therapies with treatments often having a relatively short duration of response. For those patients prognosis is still poor and only for a highly selected subset long term remission can be achieved with allogeneic stem cell transplantation. Understanding of the disease, its epidemiology and clinical course, and its most appropriate management are gradually advancing, and there is well-founded hope that this will lead to further improvements in the care of patients with MF/SS.

Address: Department of Dermatology and Venereology, University Hospital of St. Pölten, Karl Landsteiner University of Health Sciences, St. Pölten, Austria; Karl Landsteiner Institute of Dermatological Research, Department of Dermatology and Venereology, University Hospital of St. Pölten, St. Pölten, Austria. Electronic address: [email protected].; Department of Dermatology, HELIOS Klinikum Krefeld, Krefeld, Germany; Institute for Molecular Medicine, Medical School Hamburg, University of Applied Sciences and Medical University, Hamburg, Germany; Department of Dermatology, HELIOS Klinikum Schwerin, University Campus of The Medical School Hamburg, Schwerin, Germany.; Department of Dermatology, Hopital Saint Louis, Université Paris Cité, INSERM U976, Paris, France.; Department of Dermatology and Allergology, Kantonspital St. Gallen, St. Gallen, Switzerland.; Department of Dermatology, University of Zurich, Zurich, Switzerland.; Department of Dermatology, University Hospital of Lausanne and Faculty of Biology and Medicine, University of Lausanne, Lausanne, Switzerland.; Department of Dermatology, University of Copenhagen, Copenhagen, Denmark; Division of Dermatology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.; Cutaneous Lymphoma Unit, Davidoff Cancer Center, Rabin Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.; Department of Dermatology, Medical University of Vienna, Vienna, Austria.; Hautklinik, Städtisches Klinikum Karlsruhe, Karlsruhe, Germany.; Guy's and St Thomas' NHS Foundation Trust, Guy's Hospital, London, UK.; Department of Dermatology, Venereology and Allergology, University Medical Center Mannheim, Mannheim, Germany.; Department of Dermatology, Hospital Universitario 12 de Octubre, Institute i+12, CIBERONC, Medical School, University Complutense, Madrid, Spain.; National and Kapodistrian University of Athens, 2nd Department of Dermatology and Venereology, Attikon General Hospital, University of Athens, Chaidari, Greece.; Department of Health Sciences, Division of Dermatology, University of Florence, Florence, Italy.; Department of Medical Sciences, Section of Dermatology, University of Turin, Turin, Italy.; Department of Dermatology and Allergology, Inflammation Center, Helsinki University Central Hospital, Helsinki, Finland.; Department of Dermatology, University Hospital Birmingham, Birmingham, UK.; University Department of Dermatology, Venereology, Allergology and Phlebology, Skin Cancer Center, Johannes Wesling Medical Centre Minden, Ruhr University Bochum, Bochum, Germany.; Department of Dermatology, Leiden University Medical Center, Leiden, the Netherlands.; Department of Dermatology, University Hospital Schleswig-Holstein, Campus Kiel, Kiel, Germany; Medical Department, Medical School of Hamburg, Hamburg, Germany.; St. John's Institute of Dermatology, School of Basic and Medical Biosciences, Faculty of Life Sciences and Medicine, King's College London, London, UK.; Department of Dermatology and Venereology, University Hospital of St. Pölten, Karl Landsteiner University of Health Sciences, St. Pölten, Austria; Karl Landsteiner Institute of Dermatological Research, Department of Dermatology and Venereology, University Hospital of St. Pölten, St. Pölten, Austria.

DOI: 10.1016/j.ejca.2023.113343

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MeSH Terms: Humans, Mycosis Fungoides, Sezary Syndrome, Consensus, Quality of Life, Lymphoma, T-Cell, Cutaneous, Skin Neoplasms, Immunologic Factors