ADRENAL CORTEX AND MEDULLA: Edited by Irina BancosMetastatic pheochromocytoma and paraganglioma: recent advances in prognosis and managementHamidi, OksanaAuthor Information Division of Endocrinology and Metabolism, University of Texas Southwestern Medical Center, Dallas, Texas, USA Correspondence to Oksana Hamidi, DO, Division of Endocrinology and Metabolism, University of Texas Southwestern Medical Center, 2001 Inwood Rd, Dallas, TX 75235, USA. Tel: +1 214 645 2800; fax: +1 214 645 2828; e-mail: Oksana.firstname.lastname@example.org Current Opinion in Endocrinology & Diabetes and Obesity: June 2019 - Volume 26 - Issue 3 - p 146-154 doi: 10.1097/MED.0000000000000476 Buy Metrics Abstract Purpose of review Metastatic pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors with variable prognosis. This review highlights recent studies on outcomes and management of patients with metastatic PPGL. Recent findings Latest advances were made in identifying predictors of favorable outcomes of patients with metastatic PPGL. Recent studies evaluated the efficacy of tyrosine kinase inhibitors, high-specific-activity radiopharmaceuticals, and peptide receptors radionuclide therapy in treatment of metastatic disease. Moreover, ongoing studies are assessing the effects of hypoxia-inducible factor 2αα and heat shock protein 90 inhibitors as potential therapies. Summary Several active studies are evaluating the efficacy of systemic chemo, immuno, radiopharmaceutical, and peptide receptor radionuclide therapies to relieve local and adrenergic symptoms and provide survival benefit for patients with symptomatic and/or progressive advanced metastatic PPGL. Owing to rarity and wide-outcome variability, multidisciplinary team effort and personalized approach are central in caring for patients with metastatic PPGL. Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.