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Endocrinology and Metabolism |
1 Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD.
2 Department of General Internal Medicine, St. Radboud University Hospital, Nijmegen, The Netherlands.
3 Department of Clinical Pathophysiology, University of Florence, Florence, Italy.
4 Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD.
5 Urologic Oncology Branch, National Cancer Institute, NIH, Bethesda, MD.
6 Pediatric and Reproductive Endocrinology Branch, National Institute of Child Health and Human Development, NIH, Bethesda, MD.
aAddress correspondence to this author at: Building 10, Room 6N252, National Institutes of Health, 10 Center Dr., MSC-1620, Bethesda, MD 20892-1620. Fax 301-402-0180; e-mail ge{at}box-g.nih.gov.
Background: Measurements of plasma free metanephrines (normetanephrine and metanephrine) provide a useful test for diagnosis of pheochromocytoma and may provide other information about the nature of these tumors.
Methods: We examined relationships of tumor size, location, and catecholamine content with plasma and urinary metanephrines or catecholamines in 275 patients with pheochromocytoma. We then prospectively examined whether measurements of plasma free metanephrines could predict tumor size and location in an additional 16 patients.
Results: Relative proportions of epinephrine and norepinephrine in tumor tissue were closely matched by relative increases of plasma or urinary metanephrine and normetanephrine, but not by epinephrine and norepinephrine. Tumor diameter showed strong positive relationships with summed plasma concentrations or urinary outputs of metanephrine and normetanephrine (r = 0.81 and 0.77; P <0.001), whereas relationships with plasma or urinary catecholamines were weaker (r = 0.41 and 0.44). All tumors in which increases in plasma metanephrine were >15% of the combined increases of normetanephrine and metanephrine either had adrenal locations or appeared to be recurrences of previously resected adrenal tumors. Measurements of plasma free metanephrines predicted tumor diameter to within a mean of 30% of actual diameter, and high plasma concentrations of free metanephrine relative to normetanephrine accurately predicted adrenal locations.
Conclusions: Measurements of plasma free metanephrines not only provide information about the likely presence or absence of a pheochromocytoma, but when a tumor is present, can also help predict tumor size and location. This additional information may be useful for clinical decision-making during tumor localization procedures.
The following articles in journals at HighWire Press have cited this article:
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  H.-B. Fiebrich, A. H. Brouwers, M. N. Kerstens, M. E. J. Pijl, I. P. Kema, J. R. de Jong, P. L. Jager, P. H. Elsinga, R. A. J. O. Dierckx, J. E. van der Wal, et al. 6-[F-18]Fluoro-L-Dihydroxyphenylalanine Positron Emission Tomography Is Superior to Conventional Imaging with 123I-Metaiodobenzylguanidine Scintigraphy, Computer Tomography, and Magnetic Resonance Imaging in Localizing Tumors Causing Catecholamine Excess J. Clin. Endocrinol. Metab., October 1, 2009; 94(10): 3922 - 3930. [Abstract] [Full Text] [PDF]
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 H. J L M Timmers, A.-P. Gimenez-Roqueplo, M. Mannelli, and K. Pacak Clinical aspects of SDHx-related pheochromocytoma and paraganglioma Endocr. Relat. Cancer, June 1, 2009; 16(2): 391 - 400. [Abstract] [Full Text] [PDF]
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H. J. L. M. Timmers, K. Pacak, T. T. Huynh, M. Abu-Asab, M. Tsokos, M. J. Merino, B. E. Baysal, K. T. Adams, and G. Eisenhofer Biochemically Silent Abdominal Paragangliomas in Patients with Mutations in the Succinate Dehydrogenase Subunit B Gene J. Clin. Endocrinol. Metab., December 1, 2008; 93(12): 4826 - 4832. [Abstract] [Full Text] [PDF]
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 R. J. Singh and G. Eisenhofer High-Throughput, Automated, and Accurate Biochemical Screening for Pheochromocytoma: Are We There Yet? Clin. Chem., September 1, 2007; 53(9): 1565 - 1567. [Full Text] [PDF]
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 S. Cleary, J. K Phillips, T.-T. Huynh, K. Pacak, A. G Elkahloun, J. Barb, R. A Worrell, D. S Goldstein, and G. Eisenhofer Neuropeptide Y expression in phaeochromocytomas: relative absence in tumours from patients with von Hippel-Lindau syndrome J. Endocrinol., May 1, 2007; 193(2): 225 - 233. [Abstract] [Full Text] [PDF]
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T. Scholz, G. Eisenhofer, K. Pacak, H. Dralle, and H. Lehnert Current Treatment of Malignant Pheochromocytoma J. Clin. Endocrinol. Metab., April 1, 2007; 92(4): 1217 - 1225. [Abstract] [Full Text] [PDF]
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H. J. L. M. Timmers, A. Kozupa, G. Eisenhofer, M. Raygada, K. T. Adams, D. Solis, J. W. M. Lenders, and K. Pacak Clinical Presentations, Biochemical Phenotypes, and Genotype-Phenotype Correlations in Patients with Succinate Dehydrogenase Subunit B-Associated Pheochromocytomas and Paragangliomas J. Clin. Endocrinol. Metab., March 1, 2007; 92(3): 779 - 786. [Abstract] [Full Text] [PDF]
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K. L. Brain, J. Kay, and B. Shine Measurement of Urinary Metanephrines to Screen for Pheochromocytoma in an Unselected Hospital Referral Population Clin. Chem., November 1, 2006; 52(11): 2060 - 2064. [Abstract] [Full Text] [PDF]
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 T.-T. Huynh, K. Pacak, F. M Brouwers, M. S Abu-Asab, R. A Worrell, M. M Walther, A. G Elkahloun, D. S Goldstein, S. Cleary, and G. Eisenhofer Different expression of catecholamine transporters in phaeochromocytomas from patients with von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2 Eur. J. Endocrinol., October 1, 2005; 153(4): 551 - 563. [Abstract] [Full Text] [PDF]
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