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Italian Journal of Dermatology and Venereology 2021 May 26

DOI: 10.23736/S2784-8671.20.06668-7


language: English

An expert consensus report on Mycosis fungoides in Italy: epidemiological impact and diagnostic-therapeutic pathway

Silvia ALBERTI VIOLETTI 1 , Mauro ALAIBAC 2, Marco ARDIGÒ 3, Antonello BALDO 4, Nicola DI MEO 5, Cesare MASSONE 6, Francesco ONIDA 7, Gabriele SIMONTACCHI 8, Iris ZALAUDEK 5, Nicola PIMPINELLI 9, Pietro QUAGLINO 10, Emilio BERTI 1

1 UOC Dermatologia, Dipartimento di Medicina Interna Fondazione IRCCS Ca’ Granda - Ospedale Maggiore Policlinico, Milan, Italy; 2 UOC Clinica Dermatologica, Dipartimento di Medicina Azienda Ospedaliera di Padova, Padova, Italy; 3 UOC di Dermatologia Clinica, Istituto San Gallicano-IRCCS, Rome, Italy; 4 Azienda Ospedaliera Universitaria Federico II, Naples, Italy; 5 Clinica Dermatologica, Università degli Studi di Trieste ASUITS, Trieste, Italy; 6 SC Dermatologia, Ospedali Galliera, Genova, Italy; 7 Centro Trapianti Midollo Osseo, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Università degli Studi di Milano, Milan, Italy; 8 U.O. Radioterapia, Dipartimento di Oncologia, Azienda Ospedaliero-Universitaria Careggi, Florence, Italy; 9 Dipartimento di Scienze della Salute, Sezione Dermatologia, Università degli Studi di Firenze, Florence, Italy; 10 Clinica Dermatologica, Dipartimento di Scienze Mediche, Università degli Studi di Torino, Turin, Italy


Mycosis fungoides (MF) is a rare neoplasm representing the most frequent form of primary cutaneous T-cell lymphoma (CTCL). Diagnosis of MF is generally complex, often requiring integration of clinical, histological, immunophenotypic and molecular data. Currently, there are no epidemiological data supported by registries or local studies on MF in Italy. Moreover, the clinical management of MF in Italy is heterogeneous, and differs according to the geographical area and experience of the physician who manages the disease. Considering the uncertainties in the current scenario for MF in Italy, a consensus project involving experts on CTCL was initiated to define the epidemiological impact of MF and obtain information about the current diagnostic and therapeutic pathway of this disease in Italy. The prevalence of MF in Italy was estimated to be 6,800 patients, 4,900 of whom with early stage of disease; the estimated incidence ranged between 270 and 330 new cases per year. Among the clinical figures involved in the multidisciplinary management of MF, dermatologists were recognised as a reference point for both diagnosis and therapeutic decisions. These findings suggest the importance of monitoring both the disease and its management; it is, therefore, interesting to set up regional registries for monitoring and recognition of rare tumor status for MF. The results further indicate the need to train physicians to favour more rapid diagnosis and simplify the pathway for referring patients to reference centres with adequate diagnostic and treatment standards. In light of the forthcoming introduction of new therapies, the development of a nationwide PDTA (Path of Diagnostic Therapeutic Care, in Italian defined as Percorso Diagnostico-Terapeutico Assistenziale) is also of substantial importance.

KEY WORDS: Primary cutaneous T-cell lymphoma; Mycosis fungoides; Diagnosis; Treatment; Multidisciplinary

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