ABSTRACT

Catecholamine-producing tumors, including pheochromocytomas, paragangliomas, neuroblastomas, ganglioneuroblastomas and ganglioneuromas, develop from neural crest-derived progenitors and chromaffin cells of the adrenals and paraganglia. The sympathetic ganglia are formed during embryogenesis after migration of thoracic neural crest progenitors, sympathogonia, to locations along the aorta to form the sympathetic chain (1). The adrenals are first noticeable during the sixth week of gestation as an accumulation of mesodermal cells that gives rise to the adrenal cortex. Sympatho-chromaffin cells from adjacent ganglionic masses then invade the cortex to form the adrenal medulla.