Horm Metab Res 2008; 40(5): 329-337
DOI: 10.1055/s-2008-1073156
Review

© Georg Thieme Verlag KG Stuttgart · New York

Current Progress and Future Challenges in the Biochemical Diagnosis and Treatment of Pheochromocytomas and Paragangliomas

G. Eisenhofer 1 , 2 , G. Siegert 1 , J. Kotzerke 3 , S. R. Bornstein 2 , K. Pacak 4
  • 1Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus Dresden, Germany
  • 2Department of Internal Medicine III, University Hospital Carl Gustav Carus Dresden, Germany
  • 3Department of Nuclear Medicine, University Hospital Carl Gustav Carus Dresden, Germany
  • 4Reproductive and Adult Endocrinology Program, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA
Further Information

Publication History

received 26.11.2007

accepted 18.12.2007

Publication Date:
19 May 2008 (online)

Abstract

Findings from five independent studies - with close to 350 patients with pheochromocytoma and more than 2 500 in whom the tumor was excluded - indicate that measurements of plasma free metanephrines provide an overall diagnostic sensitivity of 98% and specificity of 92%. The recommendation that initial testing for the tumor should always include measurements of either plasma or urinary fractionated metanephrines results from recognition of the high diagnostic sensitivity of measurements of plasma metanephrines. The few patients with pheochromocytoma in whom the test may not yield a positive result include those with very small tumors or microscopic disease and others with tumors that do not produce norepinephrine and epinephrine. Such patients are typically normotensive and do not exhibit symptoms of catecholamine excess. Additional measurements of methoxytyramine can be useful for detecting those tumors that produce only dopamine. Suboptimal diagnostic specificity and difficulties in distinguishing true- from false-positive elevations of plasma metanephrines remain challenges for diagnosis. Improvements in analytical technology (e.g., liquid chromatography with tandem mass spectrometry) and new strategies for follow-up testing provide possible solutions to these problems. The single most important remaining clinical care challenge is the development of effective cures for patients with malignant disease. Current treatments, none of which are truly satisfactory, include chemotherapy and radiopharmaceutical therapy with 131I-labelled m-iodobenzylguanidine or radioactive somatostatin analogues. Improvements in treatment may in the future come from several fronts, but proof of efficacy ideally will require well-coordinated multicenter prospective trials in larger numbers of patients than in previous studies.

References

Correspondence

G. Eisenhofer, PhD 

Institute of Clinical Chemistry and Laboratory Medicine and Department of Internal Medicine III

University Hospital Carl Gustav Carus Dresden

Fetscherstraße 74

01307 Dresden

Germany

Phone: +49/351/458 45 95

Fax: +49/351/458 58 87

Email: graeme.eisenhofer@uniklinikum-dresden.de