Pheochromocytomas and Paragangliomas

PPGL are rare neuroendocrine tumors that secrete catecholamines. There are over 20 driver mutations associated with PPGL. All patients who have been diagnosed with PPGL need genetic testing. Diagnosis is made by checking either plasma or urine metanephrines. Localization studies include computed tomography, magnetic resonance imaging, and functional imaging such as positron emission tomography scans. Alpha-blockers are a central component for preparation prior to surgical removal of the tumor, which is the mainstay of therapy. For patients with metastatic disease, several different modalities can be employed from palliative surgery, chemotherapy, radionuclide therapy, HIF-2α inhibitor, and drugs in clinical trials.