Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine 
tumors of the adrenal glands and the sympathetic and parasympathetic 
paraganglia. They can occur sporadically or as a part of different hereditary 
tumor syndromes. About 30% of PCCs and PGLs are currently believed to be caused 
by germline mutations and several novel susceptibility genes have recently been 
discovered. The clinical presentation, including localization, malignant 
potential, and age of onset, varies depending on the genetic background of the 
tumors. By reviewing more than 1700 reported cases of hereditary PCC and PGL, a 
thorough summary of the genetics and clinical features of these tumors is given, 
both as part of the classical syndromes such as multiple endocrine neoplasia 
type 2 (MEN2), von Hippel-Lindau disease, neurofibromatosis type 1, and 
succinate dehydrogenase-related PCC-PGL and within syndromes associated with a 
smaller fraction of PCCs/PGLs, such as Carney triad, Carney-Stratakis syndrome, 
and MEN1. The review also covers the most recently discovered susceptibility 
genes including KIF1Bβ, EGLN1/PHD2, SDHAF2, TMEM127, SDHA, and MAX, as well as a 
comparison with the sporadic form. Further, the latest advances in elucidating 
the cellular pathways involved in PCC and PGL development are discussed in 
detail. Finally, an algorithm for genetic testing in patients with PCC and PGL 
is proposed.