THE . HE PATIENTS with pheochromocytoma offers the physician an opportunity to cure a potentially fatal disease. For this reason, pheochromocytoma has engendered great interest among physicians, but, unfortunately, the diagnosis is frequently missed with disastrous results.The purpose of this paper is to outline modern methods of management for this disorder as we review the course of 17 patients with pheochromocytoma. We also have included in our survey one patient with hypertension associated with bilateral adrenal medullary hyperplasia.
MaterialOur patient group comprised ten men and eight women, ranging in age from 7 to 78 years, gathered at University Hospitals Medical Center in the last 23 years. Three patients were under the age of 14; one with paroxysmal hypertension was followed for almost five years before the diagnosis was made. The diagnosis was made preoperatively in 11 patients, at autopsy in 6, and in 1 at the time of operation. While none of our patients had malignant tumors, three had bilateral ones.One patient with bilateral adrenal medullary hyper¬ plasia is of special interest. Unfortunately, no pre¬ operative catecholamine assays were made, nor were other diagnostic procedures for pheochromocytoma carried out before death. The patient had paroxysmal hypertension which was unresponsive to the usual drug therapy. She entered the hospital in coma from a cerebral hemorrhage and died within 48 hours.Autopsy showed only the ruptured intracerebral blood vessel and evidence of pronounced adrenal medullary hyperplasia which, together with the observations of Drukker et al1 and Montalbano et al 2 demonstrating that adrenal medullary hyperplasia may be associated with the syndrome simulating pheochromocytoma, strongly suggests that this patient's hypertension was related to adrenal medullary hyperfunction.
Physiological ConsiderationsExcess levarterenal and epinephrine cause most symptoms and signs in patients with pheochromocytoma. It is necessary, therefore, to understand the systemic effects of these hormones in order to find and treat these pa¬ tients.A useful classification of the effects of catecholamines has been devised by Ahlquist,3 who divides the cardiovascular effects of the catechols into those resulting from alpha re¬ ceptor stimulation, which may be blocked by dichloroisoproterenol. Alpha receptor stimula¬ tion causes arteriolar and venular constriction but has little direct myocardial effect. Beta re¬ ceptor stimulation results in venoconstriction,4 arteriolar dilatation, and a positive inotropic and chronotropic effect on the heart. At the moment, we cannot clearly assign response of blood vessels in the gut and in the brain to either alpha or beta stimulation alone and it would appear that other receptor mechanisms for catecholamines are available in certain circum¬ stances, or that the action of certain receptors may be variable.Although levarterenol and epinephrine may on occasion activate either alpha or beta re¬ ceptor sites, in general we can consider levarterenol chiefly an alpha...