Abstract

Pheochromocytomas are rare neuroendocrine tumors that arise from the adrenal medulla cells. When a pheochromocytoma is suspected, the first step is to demonstrate that there is excessive catecholamine production. Among the different available tests for the diagnosis of pheochromocytoma, those with the greatest diagnostic accuaracy are fractionated metanephrines in urine or plasma. If false positives that justify the elevation of metanephrines have been ruled out, the next step is the localization diagnosis, with a CT scan considered the initial test of choice. Nuclear medicine tests are useful for initial tumor staging as well as for detecting lesions not localized by conventional imaging techniques. Once the diagnosis has been confirmed and taking into account that 15% – 40% of pheochromocytomas are hereditary, it is recommended to request a genetic study for all patients, ideally through massive parallel sequencing techniques.

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