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Biochemical and Clinical Manifestations of Dopamine-Producing Paragangliomas: Utility of Plasma Methoxytyramine

Clinical Neurocardiology Section (G.E., D.S.G.), National Institute of Neurological Disorders and Stroke, Department of Laboratory Medicine, Clinical Center (P.S., G.C.), and the Pediatric and Reproductive Endocrinology Branch (F.M.B., E.W.L., K.T.A., K.P.), National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892-1620

Address all correspondence and requests for reprints to: Graeme Eisenhofer, Building 10, Room 6N252, National Institutes of Health, 10 Center Drive, MSC-1620, Bethesda, Maryland 20892-1620. E-mail: ge{at}box-g.nih.gov.

Measurements of plasma-free normetanephrine and metanephrine provide a sensitive test for diagnosis of pheochromocytoma but may fail to detect tumors that produce predominantly dopamine. Such tumors are extremely rare, usually found as extraadrenal paragangliomas. This report describes measurements of plasma concentrations of free methoxytyramine, the O-methylated metabolite of dopamine, in 120 patients with catecholamine-producing tumors, including nine with extraadrenal paragangliomas secreting predominantly dopamine. In seven of these nine patients, tumors were found incidentally or secondary to the space-occupying complications of the lesions. Plasma concentrations of free methoxytyramine and dopamine were increased in all nine patients, including two with normal plasma and urinary normetanephrine and metanephrine and normal urinary outputs of dopamine. Relative increases above normal for plasma methoxytyramine (104-fold) and dopamine (56-fold) were much greater (P < 0.001) than those for urinary dopamine (3-fold). Insensitivity of the latter for identification of dopamine-secreting tumors was due to dependence of the urinary amine on renal extraction and decarboxylation of circulating 3,4-dihydroxyphenylalanine. Measurements of plasma-free methoxytyramine, in addition to normetanephrine and metanephrine, are unlikely to improve diagnosis of pheochromocytomas in hypertensive patients with symptoms of catecholamine excess but may be useful in selected patients for identification of tumors that produce predominantly dopamine.

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