“…9,11,19,27 Compared with VHL and MEN2, PHEO in NF1 is more frequently unilateral (90% of cases), diagnosed incidentally, later in life and in patients with no familial history. 9,11,19,27,29,30 These differences might be related to the fact that current guidelines do not recommend routine biochemical or imaging screening of PHEO in asymptomatic/normotensive patients with NF1, despite the recommendation of doing it for patients with VHL or MEN2. 31 Recent studies reinforce the idea that PHEO prevalence in NF1 is underestimated and it may have atypical presentation (asymptomatic/normotensive or associated with non-specific symptoms).…”