Of note, while patients with variants involving the PI3K-AKT pathway have been associated with benign neoplasms (e.g., lipomas and trichoblastomas), malignancies (e.g., rhabdomyosarcomas and bladder cancer) have exclusively been reported in patients with EN associated with somatic variants of RAS genes (Supplementary Table S2). This evidence concerns the gene AKT1 and urinary bladder cancer.