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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.
RESUMO
AIMS AND BACKGROUND: To evaluate results of an intensity-modulated radiotherapy with simultaneous integrated boost schedule with concurrent and neo-adjuvant platinum-based chemotherapy for the definitive treatment of locally advanced head and neck cancer in a retrospective series. METHODS AND STUDY DESIGN: Between May 2007 and February 2010, 28 consecutive patients with locally advanced head and neck cancer (stage II, 11%; III, 18%; IV, 71%) received intensity-modulated radiotherapy with simultaneous integrated boost with concurrent and neoadjuvant (20/28 patients) chemotherapy, at 1.8 G/die to 54 Gy to the elective volume and 66 Gy (2.2 Gy/die) to the tumor volume. Acute and late toxicities were scored according to RTOG/EORTC. A quality of life questionnaire for late xerostomia was also administered. Locoregional control and overall survival were estimated using Kaplan-Meier analysis. RESULTS: Median follow-up was 50 months, there was no grade 4 acute/late toxicity. Major acute toxicities were grade 2+ mucositis, 79%; grade 2+ xerostomia, 54%; grade 2+ dysphagia, 86%; 54% of patients required parenteral nutrition. The most relevant late reaction was grade 1 xerostomia (64%), which gradually recovered with time. A linear correlation between the RTOG/EORTC scale and the quality of life questionnaire value (P = 0.0120, r2 = 0.2641) was found, receiver operating characteristic analysis (ROC) confirmed sensitivity of the quality of life questionnaire to define grade 2 late salivary toxicity (P = 0.019). Five-year actuarial locoregional control and overall survival were 81% ± 7.7 SE and 82% ± 7.3 SE, respectively. CONCLUSIONS: A prospective trial of the intensity-modulated radiotherapy with simultaneous integrated boost schedule tested in this retrospective series with concurrent and neoadjuvant chemotherapy seems warranted in order to establish this approach as a standard regimen of intensity-modulated radiotherapy with simultaneous integrated boost chemoradiation.
