Part of “CHAPTER 87 – ADRENOMEDULLARY DISORDERS OF INFANCY AND CHILDHOOD“

Adrenal medullary hyperplasia is a rare disorder.28 It has been described in children who were diagnosed clinically as having pheochromocytoma but in whom no tumor could be found on surgical exploration or at autopsy. The criteria that have been proposed for the diagnosis of adrenal medullary hyperplasia include (a) a clinical history of episodic hypertension with other symptoms and signs suggesting pheochromocytoma, generally associated with increased urinary catecholamine levels during attacks; (b) diffuse expansion of the adrenal medulla into the tail of the adrenal gland; (c) a medulla composed of enlarged cells with or without pleomorphism; and (d) an increased medulla/cortex ratio, together with an increased medullary weight. The suggestion has been made that diffuse adrenal medullary hyperplasia may be the initial pathologic change that subsequently leads to the development of nodular hyperplasia and a pheochromocytoma.29