ea0032p534 | Endocrine tumours and neoplasia | ECE2013
Tsokos Maria
, Fliedner Stephanie
, Prodanov Tamara
, Abu-Asab Mones
, Osman Jailan
, Lehnert Hendrik
, Pacak Karel
Mutations in the mitochondrial succinate dehydrogenase (SDH) subunits A, B, C, and D have been shown to hamper oxidative phosphorylation and predispose to pheochromocytomas (PHEOs) and paragangliomas (PGLs). These tumors are characterized by a glycolytic and pseudohypoxic phenotype, which is also seen in most PHEOs/PGLs occurring as part of von Hippel–Lindau (VHL) syndrome, due to VHL gene mutations. The rate of extra-adrenal tumor origin and malignancy however is particu...