Abstract: Pheochromocytomas and paragangliomas (PPGLs) are a type of neuroendocrine tumors characterized by strong hereditary and metastatic potential,whose pathogenesis is closely associated with signaling pathways such as the pseudohypoxia signaling and the kinase signaling.Based on differences in driver gene mutation profiles,PPGLs can be classified into distinct molecular subtypes,each with unique clinical phenotypes,biological behaviors,and prognostic features.Clinically,plasma free metanephrines measurement and imaging examinations are the primary diagnostic methods for PPGLs,while surgical resection remains the preferred treatment.Currently,there is still lacking standard treatment for locally advanced and metastatic PPGLs,and management of these patients remains challenging.Clinical trials of targeted agents have initially demonstrated therapeutic potential,thereby offering novel treatment avenues for this patient population.

Key words: pheochromocytomas, paragangliomas, molecular classification, hereditary, diagnosis and ttreatment