1, Fig. 1). The mean age at onset was 55 ± 17 years (min 11–max 94), including four pediatric cases [21, 27, 35, 41]. A significative prevalence of men compared to women was noticed (50 vs. 23 cases: 68.5% vs. 31.5%) with no significant difference in age at onset between men and women (mean: 55 ± 18 vs. 56 ± 16 years, p = 0.643). Comorbidi