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). Here, PIK3CA is linked to benign neoplasm.