Diagnosis and management of metastatic pheochromocytoma and paraganglioma

Abstract

Pheochromocytoma (PHEO) and paraganglioma (PGL) are rare catecholamine-producing neuroendocrine tumors arising from adrenal medulla or extra-adrenal sympathetic and parasympathetic ganglia. Most of the PHEOs/PGLs are benign tumors, but metastatic disease is common, especially in patients with particular genetic background. Although PHEOs/PGLs were described more than a century ago, diagnosis and therapy of metastatic disease are still challenging. Advances in understanding molecular and metabolic changes associated with tumorigenesis lead us to identification of the background of these tumors. Novel information allowed for development of more precise diagnostic methods and molecular therapeutic targets identification, which will improve care of patients with metastatic PHEO/PGL.Key words: diagnostics - management - paraganglioma - pheochromocytoma - therapy.