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

Abstract

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 recognized 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 favor more rapid diagnosis and simplify the pathway for referring patients to reference centers 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.