Pheochromocytoma and Paraganglioma are highly vascular neuroendocrine tumors arising from chromaffin cells of the adrenal medulla or neuronal crest progenitors located outside of the adrenal gland, respectively. As they are relatively rare compared to other neuroendocrine tumors, these might go undetected or undiagnosed.
Moreover, the clinical manifestation of these tumors is so variable it has been described as “the great masquerader”. This variability is attributed to hemodynamic and metabolic actions of the catecholamines produced and secreted. While most of these tumors are benign, about 10% of pheochromocytomas and 25% of paragangliomas are malignant. Indeed, benign pheochromocytoma and paraganglioma can progress into highly malignant phenotypes many years after the initial diagnosis, though the exact mechanisms are poorly understood.
With this article collection, we welcome articles focusing on the recent advances in prognosis, treatment, and new molecular mechanisms of this disease. Particular attention will be given to articles on early manifestations, prognosis, control, and treatment of Pheochromocytoma and Paraganglioma. Manuscripts reporting new genetic variations and molecular mechanisms are also welcome.
Pheochromocytoma and Paraganglioma are highly vascular neuroendocrine tumors arising from chromaffin cells of the adrenal medulla or neuronal crest progenitors located outside of the adrenal gland, respectively. As they are relatively rare compared to other neuroendocrine tumors, these might go undetected or undiagnosed.
Moreover, the clinical manifestation of these tumors is so variable it has been described as “the great masquerader”. This variability is attributed to hemodynamic and metabolic actions of the catecholamines produced and secreted. While most of these tumors are benign, about 10% of pheochromocytomas and 25% of paragangliomas are malignant. Indeed, benign pheochromocytoma and paraganglioma can progress into highly malignant phenotypes many years after the initial diagnosis, though the exact mechanisms are poorly understood.
With this article collection, we welcome articles focusing on the recent advances in prognosis, treatment, and new molecular mechanisms of this disease. Particular attention will be given to articles on early manifestations, prognosis, control, and treatment of Pheochromocytoma and Paraganglioma. Manuscripts reporting new genetic variations and molecular mechanisms are also welcome.