title: The current management of mycosis fungoides and Sézary syndrome and the role of radiotherapy: Principles and indications.
abstract: OBJECTIVE
  To evaluate the current treatment of mycosis fungoides (MF) and Sézary syndrome (SS) focusing on the role of radiotherapy (RT), its principles and indications, and the perspectives of the novel irradiation technologies.
  
  
  BACKGROUND
  MF and SS are rare lymphoproliferative diseases whose incidence is increasing. For a long time RT has been used as a single modality or in integrated treatment programs for these diseases.
  
  
  METHODS
  The latest systematic reviews, primary studies and new diagnostic and treatment guidelines on MF and SS were analyzed. Clinical outcomes together with the technical aspects and the role of RT were also evaluated.
  
  
  RESULTS
  New data are available on pathogenesis, diagnostic criteria, classification and staging procedures for MF and SS and several local and systemic therapies are proposed. Localized RT can cure "minimal stage" MF while total skin electron beam irradiation (TSEI) may cure initial-stage disease and may offer important symptom relief (itch, erythroderma) in a more advanced setting. Despite its efficacy, RT is not largely used, mainly because of some technical difficulties but new RT technologies may be proposed to treat large skin surfaces.
  
  
  CONCLUSIONS
  New treatment programs offer good results, with median survival of more than 12 years in early-stage MF, but the median survival of 2.5 years or less in advanced stages is still a challenge. RT remains an option for all stages with a good cost/effectiveness ratio in a curative or palliative setting. New RT technologies can overcome some technical problems of treating large skin surfaces.
authors.affiliation: Department of Radiation Oncology, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy.
authors.forename: Ercole
authors.initials: E
authors.lastname: Mazzeo
authors.affiliation: Department of Radiation Oncology, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Rubino
authors.affiliation: Department of Radiation Oncology, Brescia University, Istituto del Radio "O. Alberti", Brescia, Italy.
authors.forename: Michela
authors.initials: M
authors.lastname: Buglione
authors.affiliation: Department of Radiotherapy, Azienda Ospedaliera Universitaria Ospedale di Circolo e Fondazione Macchi, Varese, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Antognoni
authors.affiliation: Department of Radiation Oncology, Brescia University, Istituto del Radio "O. Alberti", Brescia, Italy.
authors.forename: Stefano Maria
authors.initials: SM
authors.lastname: Magrini
authors.affiliation: Department of Radiation Oncology, Brescia University, Istituto del Radio "O. Alberti", Brescia, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Bertoni
authors.affiliation: Department of Radiation Oncology, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy.
authors.forename: Manuela
authors.initials: M
authors.lastname: Parmiggiani
authors.affiliation: Department of Radiation Oncology, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Barbieri
authors.affiliation: Department of Radiation Oncology, Azienda Ospedaliero-Universitaria Policlinico, Modena, Italy.
authors.forename: Filippo
authors.initials: F
authors.lastname: Bertoni
keywords: Mycosis fungoides; Radiotherapy; Sézary syndrome; Total skin electron irradiation