title: European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome - Update 2017.
abstract: In order to provide a common standard for the treatment of mycosis fungoides (MF) and Sézary syndrome (SS), the European Organisation for Research and Treatment of Cancer-Cutaneous Lymphoma Task Force (EORTC-CLTF) published in 2006 its consensus recommendations for the stage-adapted selection of management options for these neoplasms. Since then, the understanding of the pathophysiology and epidemiology of MF/SS has advanced, the staging system has been revised, new outcome data have been published and novel treatment options have been introduced. The purpose of the present document is to update the original recommendations bearing in mind that there are still only a limited number of controlled studies to support treatment decisions for MF/SS and that often treatment is determined by institutional experience and availability. This consensus on treatment recommendations was established among the authors through a series of consecutive consultations in writing and a round of discussion. Recommended treatment options are presented according to disease stage, whenever possible categorised into first- and second-line options and supported with levels of evidence as devised by the Oxford Centre for Evidence-Based Medicine (OCEBM). Skin-directed therapies are still the most appropriate option for early-stage MF, and most patients can look forward to a normal life expectancy. For patients with advanced disease, prognosis is still grim, and only for a highly selected subset of patients, prolonged survival can be achieved with allogeneic stem cell transplantation (alloSCT). There is a high need for the development and investigation in controlled clinical trials of treatment options that are based on our increasing understanding of the molecular pathology of MF/SS.
authors.affiliation: 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, St. Pölten, Austria. Electronic address: franz.trautinger@klpu.eu.
authors.forename: Franz
authors.initials: F
authors.lastname: Trautinger
authors.affiliation: 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, St. Pölten, Austria.
authors.forename: Johanna
authors.initials: J
authors.lastname: Eder
authors.affiliation: Department of Dermatology, HELIOS Klinikum Krefeld, Krefeld, Germany.
authors.forename: Chalid
authors.initials: C
authors.lastname: Assaf
authors.affiliation: Department of Dermatology, Hôpital Saint Louis, Université Paris 7, INSERM U976, Paris, France.
authors.forename: Martine
authors.initials: M
authors.lastname: Bagot
authors.affiliation: Department of Dermatology and Allergology, Kantonsspital St. Gallen, St. Gallen, Switzerland.
authors.forename: Antonio
authors.initials: A
authors.lastname: Cozzio
authors.affiliation: Department of Dermatology, University of Zurich, Zurich, Switzerland.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Dummer
authors.affiliation: Department of Dermatology, University of Copenhagen, Copenhagen, Denmark; Division of Dermatology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Robert
authors.initials: R
authors.lastname: Gniadecki
authors.affiliation: Hautklinik, Städtisches Klinikum Karlsruhe, Karlsruhe, Germany.
authors.forename: Claus-Detlev
authors.initials: CD
authors.lastname: Klemke
authors.affiliation: Department of Dermatology, Hospital Universitaria 12 de Octubre, Madrid, Spain.
authors.forename: Pablo L
authors.initials: PL
authors.lastname: Ortiz-Romero
authors.affiliation: 2nd Department of Dermatology and Venereology, Attikon General Hospital, University of Athens, Chaidari, Greece.
authors.forename: Evangelia
authors.initials: E
authors.lastname: Papadavid
authors.affiliation: Department of Surgery and Translational Medicine, Division of Dermatology, University of Florence, Florence, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Pimpinelli
authors.affiliation: Department of Medical Sciences, Section of Dermatology, University of Turin, Turin, Italy.
authors.forename: Pietro
authors.initials: P
authors.lastname: Quaglino
authors.affiliation: Department of Dermatology and Allergology, Inflammation Center, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Annamari
authors.initials: A
authors.lastname: Ranki
authors.affiliation: Department of Dermatology, University Hospital Birmingham, Birmingham, United Kingdom.
authors.forename: Julia
authors.initials: J
authors.lastname: Scarisbrick
authors.affiliation: Department of Dermatology, Johannes Wesling Medical Centre, Minden, Germany.
authors.forename: Rudolf
authors.initials: R
authors.lastname: Stadler
authors.affiliation: Department of Dermatology and Allergology, Inflammation Center, Helsinki University Central Hospital, Helsinki, Finland.
authors.forename: Liisa
authors.initials: L
authors.lastname: Väkevä
authors.affiliation: Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Maarten H
authors.initials: MH
authors.lastname: Vermeer
authors.affiliation: St. John's Institute of Dermatology, Division of Genetics and Molecular Medicine, Faculty of Life Sciences and Medicine, King's College London, London, United Kingdom.
authors.forename: Sean
authors.initials: S
authors.lastname: Whittaker
authors.affiliation: Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Rein
authors.initials: R
authors.lastname: Willemze
authors.affiliation: Department of Dermatology, Medical University of Vienna, Vienna, Austria.
authors.forename: Robert
authors.initials: R
authors.lastname: Knobler
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001685:Biological Factors / Q000627:therapeutic use; D003131:Combined Modality Therapy / Q000379:methods; D003879:Dermatologic Agents / Q000627:therapeutic use; D004583:Electrons / Q000627:therapeutic use; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D056572:Histone Deacetylase Inhibitors / Q000627:therapeutic use; D006801:Humans; D007167:Immunotherapy / Q000379:methods; D016898:Interferon-alpha / Q000627:therapeutic use; D009182:Mycosis Fungoides / Q000473:pathology / Q000628:therapy*; D009367:Neoplasm Staging; D010789:Phototherapy / Q000379:methods; D017410:Practice Guidelines as Topic; D012176:Retinoids / Q000627:therapeutic use; D012751:Sezary Syndrome / Q000473:pathology / Q000628:therapy*; D012878:Skin Neoplasms / Q000473:pathology / Q000628:therapy*; D057832:Watchful Waiting
chemical_list: D001685:Biological Factors; D003879:Dermatologic Agents; D056572:Histone Deacetylase Inhibitors; D016898:Interferon-alpha; D012176:Retinoids
keywords: Chemotherapy; Cutaneous T-cell lymphomas; Immunotherapy; Mycosis fungoides; Phototherapy; Radiotherapy; Retinoids; Skin-directed therapy; Sézary syndrome; Total skin electron beam therapy