Current Hypertension Reports

, Volume 14, Issue 2, pp 130–137

Screening for Pheochromocytomas and Paragangliomas

Hypertension: Kidney, Sodium, and Renin-Angiotensin System (A Mimran and R Carey, Section Editors)

Abstract

Pheochromocytomas and paragangliomas are highly heterogeneous neuroendocrine tumors that must be considered not only in patients with hypertension and other manifestations of catecholamine excess but also in patients with incidentalomas or mutations in one of the ten tumor susceptibility genes identified to date. To first think of the tumor remains the critical step for screening in patients with signs and symptoms. In these patients, biochemical testing is straightforward and should include measurements of plasma or urinary metanephrines, comprising separately measured normetanephrine and metanephrine. Tumors due to an underlying germline mutation are often found in the absence of hypertension or other signs or symptoms of the tumor. Screening for disease in these patients can benefit from an individualized approach according to the particular mutation. Additional measurements of methoxytyramine, the metabolite of dopamine, can be useful in patients with mutations of succinate dehydrogenase genes or patients who are at risk for malignancy.

Keywords

Pheochromocytoma Paraganglioma Screening Incidentaloma Adrenal Extra-adrenal Metastases Malignant Chromaffin cell tumor Diagnosis Normetanephrine Metanephrine Methoxytyramine Norepinephrine Epinephrine Dopamine Catecholamines von Hippel-Lindau Multiple endocrine neoplasia type 2 Neurofibromatosis Succinate dehydrogenase 

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© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Institute of Clinical Chemistry and Laboratory Medicine and the Department of MedicineUniversity of DresdenDresdenGermany

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