Pheochromocytoma as a catecholamine producing tumor: Implications for clinical practice

Pheochromocytomas are catecholamine-producing tumors presenting with various clinical symptoms, but mostly with headache, sweating, palpitations and hypertension. If not properly diagnosed, secretion of catecholamines may lead to fatal cardiovascular consequences. Biochemical testing for pheochromoc...

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Bibliographic Details
Published inStress (Amsterdam, Netherlands) Vol. 10; no. 2; pp. 195 - 203
Main Authors Zelinka, T., Eisenhofer, G., Pacak, K.
Format Journal Article
LanguageEnglish
Published England Informa UK Ltd 01.01.2007
Taylor & Francis
Subjects
Adrenal Gland Neoplasms - diagnosis
Adrenoceptor antagonists
Algorithms
catecholamines
dopamine
epinephrine
genetic testing
Headache - etiology
Humans
Hypertension - etiology
Iron-Sulfur Proteins - genetics
laparoscopic surgery
Metanephrine - analysis
Multiple Endocrine Neoplasia Type 2a - complications
Paraganglioma - complications
Paraganglioma - genetics
Pheochromocytoma - complications
Pheochromocytoma - diagnosis
Pheochromocytoma - physiopathology
Pheochromocytoma - therapy
Succinate Dehydrogenase - genetics
Tomography, X-Ray Computed
von Hippel-Lindau Disease - complications
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Summary:Pheochromocytomas are catecholamine-producing tumors presenting with various clinical symptoms, but mostly with headache, sweating, palpitations and hypertension. If not properly diagnosed, secretion of catecholamines may lead to fatal cardiovascular consequences. Biochemical testing for pheochromocytoma should be performed not only in symptomatic subjects or in subjects with adrenal incidentaloma but also in subjects with a genetic predisposition for pheochromocytoma (multiple endocrine neoplasia type 2, Von Hippel-Lindau (VHL) syndrome, neurofibromatosis type 1 (NF 1)and mutations of succinate dehydrogenase (SDH) genes). Once a pheochromocytoma is proven, computed tomography (CT), magnetic resonance imaging (MRI) and functional imaging with [123I]-MIBG may be used for tumor localization. Adequate medical pre-treatment is essential for successful operation which is performed in most cases by laparoscopy. After tumor removal, further follow-up is necessary due to possible recurrence. Although prognosis after tumor resection is excellent, a significant proportion of pheochromocytomas recur, some as metastases. Thus, appropriate follow-up is mandatory.
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ISSN:1025-3890
1607-8888
DOI:10.1080/10253890701395896