Pheochromocytoma and paraganglioma are the primary types of neuroendocrine tumors, although they are relatively rare compared to other tumors, originating from chromaffin tissue in the adrenal medulla and/or autonomic nervous system ganglia. Because they are so rare, these tumors may go undiagnosed or undetected. Associated symptoms like hypertension are disease non-specific and may not clinically present themselves due to the fact that catecholamines can convert into their biologically inactive forms in the tumor, reducing the appearance of other symptoms as well.
These tumors produce excessive catecholamines, the effects of which are manifested through various cardiac-related symptoms, such as hypertension due to increased total peripheral resistance, heart attacks despite no prior history, non-cardiogenic pulmonary shock, oedema, arrhythmias, and sudden death. In addition, these tumors have been associated with pseudo-obstruction of the bowels, diabetic ketoacidosis, and multisystem crises involving lactic acidosis. The benign pheochromocytoma and paraganglioma can progress into highly malignant phenotypes many years after the initial diagnosis, though the exact mechanisms of this are poorly understood.
These tumors are considered the most familial in humans, with 25% of such tumors being hereditary, and contain mutations in twenty-nine associated genes. Thus the genetic factors causing them are highly diverse, making them extremely heterogenic.
This Research Topic aims to present recent advances in Pheochromocytoma and Paraganglioma through our understanding of the underlying molecular and genetic spectrum of pheochromocytoma and paraganglioma and their clinical applications, which could provide a better understanding of the disease, improve the clinical impacts/diagnosis, and therapeutics. We welcome reviews, original research, methods, as well as perspective articles.
Pheochromocytoma and paraganglioma are the primary types of neuroendocrine tumors, although they are relatively rare compared to other tumors, originating from chromaffin tissue in the adrenal medulla and/or autonomic nervous system ganglia. Because they are so rare, these tumors may go undiagnosed or undetected. Associated symptoms like hypertension are disease non-specific and may not clinically present themselves due to the fact that catecholamines can convert into their biologically inactive forms in the tumor, reducing the appearance of other symptoms as well.
These tumors produce excessive catecholamines, the effects of which are manifested through various cardiac-related symptoms, such as hypertension due to increased total peripheral resistance, heart attacks despite no prior history, non-cardiogenic pulmonary shock, oedema, arrhythmias, and sudden death. In addition, these tumors have been associated with pseudo-obstruction of the bowels, diabetic ketoacidosis, and multisystem crises involving lactic acidosis. The benign pheochromocytoma and paraganglioma can progress into highly malignant phenotypes many years after the initial diagnosis, though the exact mechanisms of this are poorly understood.
These tumors are considered the most familial in humans, with 25% of such tumors being hereditary, and contain mutations in twenty-nine associated genes. Thus the genetic factors causing them are highly diverse, making them extremely heterogenic.
This Research Topic aims to present recent advances in Pheochromocytoma and Paraganglioma through our understanding of the underlying molecular and genetic spectrum of pheochromocytoma and paraganglioma and their clinical applications, which could provide a better understanding of the disease, improve the clinical impacts/diagnosis, and therapeutics. We welcome reviews, original research, methods, as well as perspective articles.