Pheochromocytoma is the tumor that produces catecholamines and originates from chromaffin cells, which are differentiated from sympathoadrenal progenitor cells of neural crest under the influence of glucocorticoids. Genetic abnormalities of familial pheochromocytomas have elucidated oncogenic genetic bases of the tumor, including gene abnormalities of the RET proto-oncogene in multiple endocrine neoplasia type 2, VHL gene in von Hippel Lindau's disease or the NF1 gene in neurofibromatosis. Co-localization of various substances with catecholamines in the tumor, including neuropeptide Y, opioid peptides or adrenomedulOFF peptide elevating cAMP production, is recognized. The significance of these substances in modulating clinical features of pheochromocytomas is not fully understood.