Table 3. Key Points for Managing Pheochromocytoma/Paraganglioma.

Low threshold of consideration of PPGL is key for early diagnosis

Search for clinical clues that require biochemical testing for PPGL

Consider syndromic features related to hereditary pheochromocytoma syndrome

Use as initial biochemical test: plasma or urinary metanephrines

Blood sampling: preferably after at least 20 minutes of supine rest

Consider proper pre-analytical test conditions, including use of interfering drugs

Check creatinine excretion for completeness of 24-hour urine sampling

Preferred assay method: use LC-MS/MS or HPLC-ED

Use as first imaging test: CT scan; MRI reserved for specific indications

Choice of functional imaging based on location and genetic background

Consider genetic testing in all patients in the framework of genetic counselling

Preoperative evaluation and medical preparation using α-adrenoceptor blockade are essential

Annual postsurgical follow-up for at least 10 years is mandatory for all patients

Follow-up should be lifelong in patients with an increased risk for recurrence

PPGL, pheochromocytoma or paraganglioma; LC-MS/MS, liquid chromatography with tandem mass spectrometry; HPLC-ED, high pressure liquid chromatography with electrochemical detection; CT, computed tomography; MRI, magnetic resonance imaging.