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.

Total Skin Electron Beam Therapy as Part of Multimodal Treatment Strategies for Primary Cutaneous T-Cell Lymphoma

“…Recently published national and international treatment guidelines are used to inform treatment selection on the basis of patients' clinical condition and tumor stage [7,[11][12][13][14]. Many topical and systemic therapies have been developed in recent decades.…”

Primary cutaneous lymphomas – diagnostic and therapeutic guidelines of the Polish Dermatological Society

“…Consequently, patients diagnosed with MF/SS should receive "palliative" treatment in stages depending on the progression of the disease (Tab. 7) [23]. Clinical evaluation of therapeutic outcomes is sufficient in grade IA (resolution of erythema), however histopathological evaluation should be considered at more advanced stages.…”

“…Stąd też chorych z rozpoznaniem MF/SS powinno się traktować jak pacjentów "paliatywnych" i w leczeniu postępować etapowo, zgodnie z postępem choroby (tab. 7) [23]. Kliniczna ocena efektów terapii jest wystarczająca w stopniu IA (ustępowanie rumienia), natomiast w stadiach bardziej zaawansowanych należy rozważyć weryfikację histopatologiczną.…”

Current Approach to Phototherapy Applications in Dermatology Practice: Part 2. Phototherapy in Vitiligo, Mycosis Fungoides, Atopic Dermatitis, Sclerosing Diseases and Psoriasis

“…Dar bant UVB, erken evre hastalıkta, sadece yama lezyonlar varsa tercih edilmelidir. Plak lezyonlar, folikülotropik hastalık, dar bant UVB'ye yetersiz yanıt veya dar bant UVB sonrası hızlı relaps gibi durumlarda tercih edilecek fototerapi yöntemi PUVA olmalıdır (35,44,45). MF'si olan çocuk hastalarda, plak lezyonlar yoksa, öncelikli tercih edilecek fototerapi yöntemi dar bant UVB'dir, hipopigmente yamalar UVB'ye çok iyi yanıt vermektedir (46,47) (Resim 2).…”

“…Bu rehberde önerilen bu protokol de, idamenin relapsı azalttığına dair herhangi bir veriye dayandırılmaksızın yapılmıştır. EORTC'nin 2017 rehberinde, tam remisyon sonrası idame için kullanılabilecek tedavi yöntemleri arasında, UVB ve PUVA'ya da yer verilmiştir (45).…”