Personalized Management of Pheochromocytoma and Paraganglioma

. 2021 Jun 19;43(2):199–239. doi: 10.1210/endrev/bnab019

Practical tip/synthesis (cluster 2):
• A general diagnostic flow-chart is provided by Fig. 2; a cluster-specific diagnostic flow-chart is provided by Fig. 3. • With the exception of MAX-related PCCs (mixed or noradrenergic phenotype), cluster 2–related tumors have an adrenergic phenotype (assessed by elevated plasma or urinary metanephrine). • This adrenergic signature indicates a more mature phenotype. • Adrenergic tumors are characterized by high catecholamine content and high rates of production of metanephrines, but overall low rates of catecholamine secretion and well-developed secretory control.

Practical tip/synthesis (cluster 2):

• A general diagnostic flow-chart is provided by Fig. 2; a cluster-specific diagnostic flow-chart is provided by Fig. 3.
• With the exception of MAX-related PCCs (mixed or noradrenergic phenotype), cluster 2–related tumors have an adrenergic phenotype (assessed by elevated plasma or urinary metanephrine).
• This adrenergic signature indicates a more mature phenotype.
• Adrenergic tumors are characterized by high catecholamine content and high rates of production of metanephrines, but overall low rates of catecholamine secretion and well-developed secretory control.