open access

Vol 19, No 2 (2014)
Special Issue Papers
Published online: 2014-03-01
Submitted: 2013-04-16
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The current management of mycosis fungoides and Sézary syndrome and the role of radiotherapy: Principles and indications

Ercole Mazzeo, Laura Rubino, Michela Buglione, Paolo Antognoni, Stefano Maria Magrini, Francesco Bertoni, Manuela Parmiggiani, Paola Barbieri, Filippo Bertoni
DOI: 10.1016/j.rpor.2013.07.009
·
Rep Pract Oncol Radiother 2014;19(2):77-91.

open access

Vol 19, No 2 (2014)
Special Issue Papers
Published online: 2014-03-01
Submitted: 2013-04-16

Abstract

Aim

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.

Materials and 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.

Abstract

Aim

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.

Materials and 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.

Get Citation

Keywords

Radiotherapy; Mycosis fungoides; Sézary syndrome; Total skin electron irradiation

About this article
Title

The current management of mycosis fungoides and Sézary syndrome and the role of radiotherapy: Principles and indications

Journal

Reports of Practical Oncology and Radiotherapy

Issue

Vol 19, No 2 (2014)

Pages

77-91

Published online

2014-03-01

DOI

10.1016/j.rpor.2013.07.009

Bibliographic record

Rep Pract Oncol Radiother 2014;19(2):77-91.

Keywords

Radiotherapy
Mycosis fungoides
Sézary syndrome
Total skin electron irradiation

Authors

Ercole Mazzeo
Laura Rubino
Michela Buglione
Paolo Antognoni
Stefano Maria Magrini
Francesco Bertoni
Manuela Parmiggiani
Paola Barbieri
Filippo Bertoni

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