First treatment of mycosis fungoides by total skin electron beam (TSEB) therapy in Greece

BackgroundMycosis fungoides (MF), the most common subtype of cutaneous T-Cell Lymphoma (CTCL), is a rare chronic skin neoplasia. Total skin electron irradiation has been employed along with a variety of other topical or systemic treatments for MF management.AimTo report the first case treated by TSEB irradiation protocol in Greece.Materials and methodsA fractionated 36[[ce:hsp sp="0.25"/]]Gy total skin electron beam (TSEB) therapy was prescribed to a 65-years-old male patient with mycosis fungoides (MF), stage IIB, refractory to several treatments during a 20-year period. Dose uniform delivery was monitored by thermo-luminescence dosimetry.Results and discussionThe homogeneous skin dose distribution resulted in a complete clinical response. Limited, irradiation-oriented, side effects appeared.ConclusionsThe first TSEB irradiation prescription in Greek medical chronicles was proved effective in this case of tumor stage MF (T3-IIB), which had been refractory to several single or combination treatments

Expert opinions and clinical experiences with chlormethine gel as maintenance treatment for patients with mycosis fungoides

Maintenance treatment can be recommended for patients with mycosis fungoides (MF) whose disease responds to primary treatment. While positive outcomes have been observed in small studies with maintenance therapy, there is a lack of practical guidelines and agreement on when and how maintenance therapy for MF should be approached. In this article, we discuss expert opinions and clinical experiences on the topic of maintenance therapy for patients with MF, with a focus on chlormethine gel. Ideally, patients should have a durable response before initiating maintenance therapy. The definition of and required duration of durable response are topics that are open to debate and currently have no consensus. Chlormethine gel has several attributes that make it suitable for maintenance therapy; it can be easily applied at home, can be combined with other treatment options for maintenance, and has a manageable safety profile. Chlormethine gel as maintenance therapy can be applied at decreasing frequencies after active treatment with chlormethine gel or other therapies until the minimally effective dose is reached. Patients generally tend to adhere well to chlormethine gel maintenance regimens and may remain on treatment for several years. The experiences described here may be useful for clinicians when deciding on maintenance treatment regimens for their patients. Development of guidelines based on clinical trial outcomes will be important to ensure the most effective maintenance treatment strategies are used for patients with MF

European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sezary syndrome - Update 2017

In order to provide a common standard for the treatment of mycosis fungoides (MF) and Sezary 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. (C) 2017 The Authors. Published by Elsevier Ltd.Peer reviewe

Global patterns of care in advanced stage mycosis fungoides/Sezary syndrome: a multicenter retrospective follow-up study from the Cutaneous Lymphoma International Consortium

ABSTRACT Background Advanced-stage mycosis fungoides (MF)/Sezary syndrome (SS) patients are weighted by an unfavorable prognosis and share an unmet clinical need of effective treatments. International guidelines are available detailing treatment options for the different stages but without recommending treatments in any particular order due to lack of comparative trials. The aims of this second CLIC study were to retrospectively analyze the pattern of care worldwide for advanced-stage MF/SS patients, the distribution of treatments according to geographical areas (USA versus non-USA), and whether the heterogeneity of approaches has potential impact on survival. Patients and methods This study included 853 patients from 21 specialist centers (14 European, 4 USA, 1 each Australian, Brazilian, and Japanese). Results Heterogeneity of treatment approaches was found, with up to 24 different modalities or combinations used as first-line and 36% of patients receiving four or more treatments. Stage IIB disease was most frequently treated by total-skin-electron-beam radiotherapy, bexarotene and gemcitabine; erythrodermic and SS patients by extracorporeal photochemotherapy, and stage IVA2 by polychemotherapy. Significant differences were found between USA and non-USA centers, with bexarotene, photopheresis and histone deacetylase inhibitors most frequently prescribed for first-line treatment in USA while phototherapy, interferon, chlorambucil and gemcitabine in non-USA centers. These differences did not significantly impact on survival. However, when considering death and therapy change as competing risk events and the impact of first treatment line on both events, both monochemotherapy (SHR = 2.07) and polychemotherapy (SHR = 1.69) showed elevated relative risks. Conclusion This large multicenter retrospective study shows that there exist a large treatment heterogeneity in advanced MF/SS and differences between USA and non-USA centers but these were not related to survival, while our data reveal that chemotherapy as first treatment is associated with a higher risk of death and/or change of therapy and thus other therapeutic options should be preferable as first treatment approach

Cutaneous lymphoma international consortium study of outcome in advanced stages of mycosis fungoides and Sézary syndrome: effect of specific prognostic markers on survival and development of a prognostic model

Advanced-stage mycosis fungoides (MF; stage IIB to IV) and Sézary syndrome (SS) are aggressive lymphomas with a median survival of 1 to 5 years. Clinical management is stage based; however, there is wide range of outcome within stages. Published prognostic studies in MF/SS have been single-center trials. Because of the rarity of MF/SS, only a large collaboration would power a study to identify independent prognostic markers. PATIENTS AND METHODS: Literature review identified the following 10 candidate markers: stage, age, sex, cutaneous histologic features of folliculotropism, CD30 positivity, proliferation index, large-cell transformation, WBC/lymphocyte count, serum lactate dehydrogenase, and identical T-cell clone in blood and skin. Data were collected at specialist centers on patients diagnosed with advanced-stage MF/SS from 2007. Each parameter recorded at diagnosis was tested against overall survival (OS). RESULTS: Staging data on 1,275 patients with advanced MF/SS from 29 international sites were included for survival analysis. The median OS was 63 months, with 2- and 5-year survival rates of 77% and 52%, respectively. The median OS for patients with stage IIB disease was 68 months, but patients diagnosed with stage III disease had slightly improved survival compared with patients with stage IIB, although patients diagnosed with stage IV disease had significantly worse survival (48 months for stage IVA and 33 months for stage IVB). Of the 10 variables tested, four (stage IV, age > 60 years, large-cell transformation, and increased lactate dehydrogenase) were independent prognostic markers for a worse survival. Combining these four factors in a prognostic index model identified the following three risk groups across stages with significantly different 5-year survival rates: low risk (68%), intermediate risk (44%), and high risk (28%). CONCLUSION: To our knowledge, this study includes the largest cohort of patients with advanced-stage MF/SS and identifies markers with independent prognostic value, which, used together in a prognostic index, may be useful to stratify advanced-stage patients

Immunolocalisation of the adhesion molecules epithelial cadherin and α-β-γ catenins in skin development and in precancerous and cancerous cutaneous lesions

ΧΡΗΣΙΜΟΠΟΙΗΣΑΜΕ ΤΑ ΜΟΝΟΚΛΩΝΙΚΑ ΑΝΤΙΣΩΜΑΤΑ HEDD-1(ΑΝΤΙ-Ε-ΚΑΤΕΝΙΝΗ) ΚΑΙ ΑΝΤΙ α-β-γ-ΚΑΤΕΝΙΝΕΣ ΣΕ ΤΟΜΕΣ ΠΑΡΑΦΙΝΗΣ ΣΕ ΦΟΥΡΝΟ ΜΙΚΡΟΚΥΜΑΤΩΝ ΣΕ ΠΡΟΚΑΡΚΙΝΙΚΕΣ ΚΑΙ ΚΑΡΚΙΝΙΚΕΣ ΒΛΑΒΕΣ ΔΕΡΜΑΤΟΣ.ΕΠΙΣΗΣ ΜΕΛΕΤΗΣΑΜΕ ΤΗΝ ΑΝΟΣΟΛΟΓΙΚΗ ΔΡΑΣΤΗΡΙΟΤΗΤΑ ΣΕ ΦΥΣΙΟΛΟΓΙΚΟ ΔΕΡΜΑ ΚΑΙ ΒΛΕΝΝΟΓΟΝΟ ΣΤΟΜΑΤΟΣ ΣΕ 24 ΕΜΒΡΥΑ ΚΑΙ 30 ΕΝΗΛΙΚΕΣ.ΟΛΑ ΤΑ ΜΟΡΙΑ ΣΥΓΚΟΛΛΗΣΗΣ ΗΤΑΝ ΠΑΡΟΝΤΑ ΣΤΙΣ ΒΑΣΙΚΗ ΚΑΙ ΥΠΕΡΚΕΙΜΕΝΕΣ ΤΗΣ ΒΑΣΙΚΗΣ ΣΤΙΒΑΔΕΣ ΤΩΝ ΔΥΟ ΕΠΙΘΗΛΙΩΝ ΣΕ ΟΛΕΣ ΤΙΣ ΗΛΙΚΙΕΣ ΕΚΤΟΣ ΑΠΟ ΤΑ ΝΕΑΡΑ ΕΜΒΡΥΑ (<24ΕΕΚ) ΟΠΟΥ ΟΙ Ε-ΚΑΝΤΕΡΙΝΗ ΚΑΙ α-β-γ-ΚΑΤΕΝΙΝΕΣ ΕΔΕΙΞΑΝ ΑΡΝΗΤΙΚΗ ΜΕΜΒΡΑΝΩΔΗ ΕΚΦΡΑΣΗ ΜΟΝΟ ΣΤΙΣ ΠΕΡΙΟΧΕΣ ΤΗΣ ΒΑΣΙΚΗΣ ΣΤΙΒΑΔΑΣ ΟΠΟΥ ΤΑ ΚΥΤΤΑΡΑ ΠΟΛΛΑΠΛΑΣΙΑΖΟΝΤΑΙ ΜΕ ΣΥΣΣΩΡΕΥΣΗ ΤΩΝ ΠΥΡΗΝΩΝ ΤΟΥΣ,ΑΛΛΑ ΚΑΙ ΠΟΛΥ ΓΡΗΓΟΡΑ ΘΕΤΙΚΟΠΟΙΗΘΗΚΑΝ ΕΦΟΣΟΝ ΤΑ ΚΥΤΤΑΡΑ ΑΥΤΑ ΔΙΑΦΟΡΟΠΟΙΗΘΟΥΝ Η ΚΕΡΑΤΙΝΗ ΣΤΙΒΑΔΑ ΗΤΑΝ ΑΡΝΗΤΙΚΗ.ΣΤΑ ΑΚΑΝΘΟΚΥΤΤΑΡΙΚΑ ΚΑΡΚΙΝΩΜΑΤΑ (ΑΚα) Η Ε-ΚΑΝΤΕΡΙΝΗ ΠΑΡΟΥΣΙΑΖΕΙ ΕΛΑΤΤΩΜΕΝΗ ΕΚΦΡΑΣΗ ΜΕ ΤΗΝ ΕΠΙΔΕΙΝΩΣΗ ΤΟΥ ΒΑΘΜΟΥ ΔΙΑΦΟΡΟΠΟΙΗΣΗΣ ΚΑΙ ΔΙΗΘΗΣΗ ΤΩΝ ΑΚα.ΣΤΙΣ ΒΛΑΒΕΣ ΤΗΣ ΝΟΣΟΥ ΤΟΥ BOWEN Η Ε-ΚΑΝΤΕΡΙΝΗ ΗΤΑΝ ΕΛΑΤΤΩΜΕΝΗ Η ΑΠΟΥΣΑ ΣΕ ΠΕΡΙΟΧΕΣ ΕΝΤΟΝΗΣ ΔΥΣΠΛΑΣΙΑΣ ΕΝΩ ΒΡΕΘΗΚΕ ΔΙΑΤΗΡΗΜΕΝΗ ΣΤΟ 74% ΤΩΝ ΑΚΤΙΝΙΚΩΝ ΥΠΕΡΚΕΡΑΤΩΣΕΩΝ ΚΑΙ 100% ΤΩΝ ΜΥΡΜΗΓΚΙΩΝ.Η α-ΚΑΤΕΝΙΝΗ ΑΚΟΛΟΥΘΟΥΣΕ ΤΗΝ ΕΚΦΡΑΣΗ ΤΗΣ Ε-ΚΑΝΤΕΡΙΝΗΣ ΣΤΗ ΝΟΣΟ ΤΟΥ BOWEN ΚΑΙ ΟΙ α-β-ΚΑΤΕΝΙΝΕΣ ΣΤΑ ΑΚα.ΣΥΜΠΕΡΑΙΝΟΥΜΕ ΟΤΙ Η Ε-ΚΑΝΤΕΡΙΝΗ ΚΑΙ ΚΑΤΙΝΙΝΕΣ ΕΙΝΑΙ ΔΕΙΚΤΕΣ ΔΙΑΦΟΡΟΠΟΙΗΣΗΣ ΚΑΙ Η Ε-ΚΑΝΤΕΡΙΝΗ ΚΑΙ ΚΑΤΕΝΙΝΕΣ ΕΙΝΑΙ ΔΕΙΚΤΕΣ ΔΙΑΦΟΡΟΠΟΙΗΣΗΣ ΚΑΙ Η Ε-ΚΑΝΤΕΡΙΝΗ ΚΑΙ α-ΚΑΤΕΝΙΝΗ ΜΠΟΡΟΥΝ ΝΑ ΧΡΗΣΙΜΟΠΟΙΗΘΟΥΝ ΩΣ ΔΕΙΚΤΕΣ ΔΥΣΠΛΑΣΙΑΣ ΣΤΙΣ ΕΠΙΘΗΛΙΑΚΕΣ ΒΛΑΒΕΣ

Understanding the enigmatic association between mycosis fungoides and psoriasis: Report of two cases and review of the literature.

Psoriatic patients present an increased risk for developing lymphoma, particularly cutaneous T-cell lymphoma (CTCL). To what degree psoriasis itself through chronic immune stimulation, or the immunosuppressive medications used for its treatment or comorbidities (obesity, diabetes mellitus, etc), or lifestyle (smoking, alcohol, diet, etc) may play a role in the onset of MF is not yet clear. Psoriasis and Mycosis Fungoides (MF), the most common variant of CTCL, represent two distinct entities sharing common pathogenetic mechanisms and a wide spectrum of common clinical features associated with the abnormal activation of T-cells. The aim of this study is to explore the relationship between MF and psoriasis by presenting two cases with clinical and histopathologic features of both psoriasis and MF with a particular emphasis on the time of presentation of both disorders, the use of previous immunosuppressive drugs as well as the therapeutic management of patients. Biopsy of the cutaneous lesions before the introduction of biologics should be incorporated in clinical practice. Biopsy of the cutaneous lesion should also be performed in the case of appearance of psoriasiform lesions during biologic treatment for autoimmune disorders because this may represent an indolent form of MF. Psoriatic patients with poor or no-response to treatment should be examined thoroughly for MF using immunochemistry and, if necessary, molecular biology techniques. In concomitant MF and psoriasis, combination treatment may be beneficial for both entities. Finally, a large multicentric registry of MF patients who were treated for benign dermatoses (i.e. eczema, psoriasis) with classic immunosuppressive drugs and/or biologics is needed to collect data and further clarify the enigmatic relationship between psoriasis, MF and immunosuppressive treatment