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 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 [(123)I]-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.
Zelinka
T
T
Reproductive Biology and Medicine Branch, National Institutes of Child Health and Human Development, Bethesda, MD 20892-1109, USA.
Eisenhofer
G
G
Pacak
K
K
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis; D000465:Algorithms; D006261:Headache / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007506:Iron-Sulfur Proteins / Q000235:genetics; D008676:Metanephrine / Q000032:analysis; D018813:Multiple Endocrine Neoplasia Type 2a / Q000150:complications; D010235:Paraganglioma / Q000150:complications / Q000235:genetics; D010673:Pheochromocytoma / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D013385:Succinate Dehydrogenase / Q000235:genetics; D014057:Tomography, X-Ray Computed; D006623:von Hippel-Lindau Disease / Q000150:complications
D007506:Iron-Sulfur Proteins; C491545:SDHD protein, human; D008676:Metanephrine; C108095:SDHB protein, human; D013385:Succinate Dehydrogenase