Pheochromocytomas and paragangliomas are genetically determined in 40% of cases.
PPGL are classified in either the Cluster 1 (pseudohypoxic) or the cluster 2 (MAPK/mTOR).
Several Cluster 2-related knockout mouse models are predisposed to pheochromocytoma.
No Cluster 1-related knockout mouse models ever developed a pheochromocytoma.
Xeno- and allograft models are an alternative to test new therapeutic strategies.