Tumors of Neural Crest Origin

Abstract
DIAGNOSIS of tumors of neural crest origin—neuroblastoma, ganglioneuroma, ganglioneuroblastoma, and pheochromocytoma— has been aided by biochemical tests which measure increased urinary excretion of certain metabolites of the naturally occurring catecholamines: norepinephrine (NE), epinephrine (E), dopamine (DM) and dopa (DA).1-13This provides rapid identification of the general tumor type preoperatively and offers a laboratory guide for evaluating the efficacy of treatment. Chemical analyses of tumor tissue2,5,14-19as well as electron microscopy19,20have provided additional information concerning the secretory nature of these tumors. These studies evolved from a recognition of the embryological relationship between these tumors and other known catechol-producing tissues: the adrenal medulla, sympathetic nerves, and ganglia. The adrenergic mediator, 1-norepinephrine, is produced in sympathetic nervous tissue following a well-defined chemical sequence starting with tyrosine (Fig 1). In the adrenal medulla, the identical chemical transformation takes place, following which NE may be methylated to 1-epinephrine. These two