ea0049ep100 | Adrenal medulla | ECE2017
Matos Maria Joao
, Costa Claudia
, Santos Ana Paula
, Teixeira Manuel
, Franco Carlos
, Bento Joao
, Azevedo Isabel
, Dias Susana
, Sampaio Ines Lucena
, Cunha Ana Luisa
, Torres Isabel
Introduction: Patients with germline mutations in one of the succinate dehydrogenase (SDH) genes are at substantially increased risk of developing paragangliomas (PGL) and pheochromocytomas (PCC). Mutations in SDHB are the most commonly found gene mutations in PC/PGL and are associated with younger ages at presentation, higher rates of metastases and poorer prognosis. Although familial PGL syndromes were initially thought to predispose only for PCC and PGL, other tumor types s...