In this Research Topic, we have collected recent developments in research into Pheochromocytomas and Paragangliomas (PPGLs), highlighting their molecular mechanisms, clinical manifestations, and improved therapeutic management.

PPGLs are the primary types of neuroendocrine tumors, and are relatively rare, originating from chromaffin tissue in the adrenal medulla and/or autonomic nervous system ganglia (1). The symptoms experienced by PPGL patients include, but are not limited to, secretion of excess catecholamines, which can manifest as various cardiovascular-related indications such as hypertension via increased total peripheral resistance, heart attack without previous history, shock (non-cardiogenic pulmonary), arrhythmias, and oedemas (2). Also, pseudo-bowel obstructions, diabetic ketoacidosis, and multisystem crises involving lactic acidosis have been associated with PPGL patients (3). Since PPGLs are rare and symptoms are non-specific, they have potential for underdiagnosis. This may lead to the progression of undetected tumors into highly metastatic phenotypes in patients with PPGLs. Recent advances in understanding of the molecular biology of PPGLs, however, offer potential to open pathways to improve therapeutic interventions for PPGL tumors (4–9).