Biochemical and clinical manifestations of dopamine-producing paragangliomas: Utility of plasma methoxytyramine

被引:170
作者
Eisenhofer, G
Goldstein, DS
Sullivan, P
Csako, G
Brouwers, FM
Lai, EW
Adams, KT
Pacak, K
机构
[1] NINDS, Clin Neurocardiol Sect, Dept Lab Med, Clin Ctr,NIH, Bethesda, MD 20892 USA
[2] NICHHD, Pediat & Reprod Endocrinol Branch, NIH, Bethesda, MD 20892 USA
关键词
D O I
10.1210/jc.2004-2025
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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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页码:2068 / 2075
页数:8
相关论文
共 31 条
[1]   Large dopamine-secreting pheochromocytoma: Case report [J].
Awada, SH ;
Grisham, A ;
Woods, SE .
SOUTHERN MEDICAL JOURNAL, 2003, 96 (09) :914-917
[2]   DOPAMINE-SECRETING AND NOREPINEPHRINE-SECRETING INTRAPERICARDIAL PHEOCHROMOCYTOMA IN A NORMOTENSIVE PATIENT [J].
AWOKE, S ;
PERLSTEIN, RS .
SOUTHERN MEDICAL JOURNAL, 1985, 78 (08) :994-998
[3]  
AZZARELLI B, 1988, LARYNGOSCOPE, V98, P573
[4]   On the association of succinate dehydrogenase mutations with hereditary paraganglioma [J].
Baysal, BE .
TRENDS IN ENDOCRINOLOGY AND METABOLISM, 2003, 14 (10) :453-459
[5]   A SPECIFIC RADIOENZYMATIC ASSAY FOR DIHYDROXYPHENYLALANINE (DOPA) - PLASMA DOPA MAYBE THE PRECURSOR OF URINE FREE DOPAMINE [J].
BROWN, MJ ;
DOLLERY, CT .
BRITISH JOURNAL OF CLINICAL PHARMACOLOGY, 1981, 11 (01) :79-83
[6]   REGIONAL RELEASE AND REMOVAL OF CATECHOLAMINES AND EXTRANEURONAL METABOLISM TO METANEPHRINES [J].
EISENHOFER, G ;
RUNDQUIST, B ;
ANEMAN, A ;
FRIBERG, P ;
DAKAK, N ;
KOPIN, IJ ;
JACOBS, MC ;
LENDERS, JWM .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 1995, 80 (10) :3009-3017
[7]  
Eisenhofer G, 2004, FRONT HORM RES, V31, P76
[8]   Plasma normetanephrine and metanephrine for detecting pheochromocytoma in Von Hippel-Lindau disease and multiple endocrine neoplasia type 2 [J].
Eisenhofer, G ;
Lenders, JWM ;
Linehan, WM ;
Walther, MM ;
Goldstein, DS ;
Keiser, HR .
NEW ENGLAND JOURNAL OF MEDICINE, 1999, 340 (24) :1872-1879
[9]  
EISENHOFER G, 1986, CLIN CHEM, V32, P2030
[10]   Benign paragangliomas: Clinical presentation and treatment outcomes in 236 patients [J].
Erickson, D ;
Kudva, YC ;
Ebersold, MJ ;
Thompson, GB ;
Grant, CS ;
Van Heerden, JA ;
Young, WF .
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2001, 86 (11) :5210-5216