Pheochromocytoma: recommendations for clinical practice from the First International Symposium

Pacak, Karel, Eisenhofer, Graeme, Ahlman, Håkan, Bornstein, Stefan R., Gimenez-Roqueplo, Anne-Paule, Grossman, Ashley B., Kimura, Noriko, Mannelli, Massimo, McNicol, Anne Marie and Tischler, Arthur S. (2007) Pheochromocytoma: recommendations for clinical practice from the First International Symposium. Nature Clinical Practice Endocrinology and Metabolism, 3 2: 92-102. doi:10.1038/ncpendmet0396


Author Pacak, Karel
Eisenhofer, Graeme
Ahlman, Håkan
Bornstein, Stefan R.
Gimenez-Roqueplo, Anne-Paule
Grossman, Ashley B.
Kimura, Noriko
Mannelli, Massimo
McNicol, Anne Marie
Tischler, Arthur S.
Title Pheochromocytoma: recommendations for clinical practice from the First International Symposium
Journal name Nature Clinical Practice Endocrinology and Metabolism   Check publisher's open access policy
ISSN 1745-8366
1745-8374
Publication date 2007
Sub-type Critical review of research, literature review, critical commentary
DOI 10.1038/ncpendmet0396
Volume 3
Issue 2
Start page 92
End page 102
Total pages 11
Place of publication London, UK
Publisher Nature Publishing Group
Language eng
Subject 11 Medical and Health Sciences
Abstract Pheochromocytomas are rare, often hereditary, catecholamine producing tumors that can be difficult to diagnose and manage. This Review summarizes the recommendations for biochemical and genetic testing, localization and treatment, and is based on discussions at the First International Symposium on Pheochromocytoma, held in October 2005. The First International Symposium on Pheochromocytoma, held in October 2005, included discussions about developments concerning these rare catecholamine-producing tumors. Recommendations were made during the symposium for biochemical diagnosis, localization, genetics, and treatment. Measurement of plasma or urinary fractionated metanephrines, the most accurate screening approach, was recommended as the first-line test for diagnosis; reference intervals should favor sensitivity over specificity. Localization studies should only follow reasonable clinical evidence of a tumor. Preoperative pharmacologic blockade of circulatory responses to catecholamines is mandatory. Because approximately a quarter of tumors develop secondary to germ-line mutations in any one of five genes, mutation testing should be considered; however, it is not currently cost effective to test every gene in every patient. Consideration of tumor location, presence of multiple tumors, presence of metastases, and type of catecholamine produced is useful in deciding which genes to test. Inadequate methods to distinguish malignant from benign tumors and a lack of effective treatments for malignancy are important problems requiring further resolution.
Keyword diagnosis
genetics
localization
paraganglioma
pheochromocytoma
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Unknown

Document type: Journal Article
Sub-type: Critical review of research, literature review, critical commentary
Collections: Excellence in Research Australia (ERA) - Collection
School of Medicine Publications
 
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Created: Tue, 16 Mar 2010, 15:36:52 EST by Laura McTaggart on behalf of Faculty Of Health Sciences