hrp0095p1-139 | Multisystem Endocrine Disorders | ESPE2022
Rivetti Giulio
, Caterina Luongo
, Aiello Francesca
, Miraglia Del Giudice Emanuele
, Marzuillo Pierluigi
, Di Iorio Giovanni
, Pellino Valeria
, Grandone Anna
Background: Carney complex (CNC) is a rare autosomal dominant multiple endocrine neoplasia and lentiginosis syndrome, characterized by abnormal cutaneous and mucosal pigmentation, myxomas of the heart, skin, breast and other non-endocrine tumors. CNC is principally caused by loss-of-function mutations of PRKAR1A gene located at 17q22-24 coding for the regulatory subunit type I alpha of protein kinase A (PKA) gene. Cushing’s syndrome is a rare disorder in...