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Pheochromocytoma/Paragangliomas

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Interventional Therapies for Secondary and Essential Hypertension

Part of the book series: Updates in Hypertension and Cardiovascular Protection ((UHCP))

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Abstract

Pheochromocytomas are rare catecholamine-secreting tumors derived from the chromaffin cells of the embryonic neural crest arising directly from the adrenal glands. Chromaffin tumors arising from extra-adrenal autonomic paraganglia are called paragangliomas. Paragangliomas of parasympathetic origin develop from non-chromaffin organs that act as chemoreceptors. Malignant tumors occur in about 10 % pheochromocytomas, the others are benign. The diagnosis takes often place in patients aged 40–50 years but hereditary variants may present earlier. Paroxysmal hypertension, episodic headache, sweating and tachycardia are commonly observed, however pallor, anxiety or panic feelings, fever, nausea or vomiting may co-exist. Diagnosis is based on clinical data, laboratory finding, functional and anatomical imaging. Catecholamine producing pheochromocytoma and paraganglioma should receive without any delay antihypertensive medications, preferably an alpha-adrenergic blocker, followed by a beta adrenergic blocker, if needed. Surgical resection is the only potentially curative treatment for primary, recurrent, or limited metastatic tumors, otherwise palliative treatment may be used to alleviate symptoms. All patients with a pheochromocytoma should be followed during at least 10 years after surgery.

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Abbreviations

CT:

Computed tomography

HTN:

Hypertension

PET:

Positron emission tomography

123I-MIBG SPECT:

123I-metaiodobenzylguanidine single photon emission computed tomography

18F-FDG:

18F-fluorodeoxyglucose

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  1. Department of Cardiology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium

    Philippe van de Borne

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  1. Philippe van de Borne
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Correspondence to Philippe van de Borne .

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  1. First Cardiology Clinic, National and Kapodistrian University of Athens, Athens, Greece

    Costas Tsioufis

  2. Department of Nephrology and Hypertension, University Hospital Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany

    Roland E. Schmieder

  3. IRCCS Istituto Auxologico Italiano, University Milano-Bicocca, Milano Monza, Italy

    Giuseppe Mancia

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van de Borne, P. (2016). Pheochromocytoma/Paragangliomas. In: Tsioufis, C., Schmieder, R., Mancia, G. (eds) Interventional Therapies for Secondary and Essential Hypertension. Updates in Hypertension and Cardiovascular Protection. Springer, Cham. https://doi.org/10.1007/978-3-319-34141-5_3

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  • DOI: https://doi.org/10.1007/978-3-319-34141-5_3

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