The Endocrine Society has developed clinical practice guidelines for the diagnosis, management, and treatment of pheochromocytoma and paraganglioma (PPGLs). The guidelines are based on a systematic review of the literature and the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system. Key recommendations include: 1. **Biochemical Testing**: Initial biochemical testing should include measurements of plasma free or urinary fractionated metanephrines. Liquid chromatography with mass spectrometric or electrochemical detection methods are recommended over other laboratory methods. 2. **Imaging Studies**: Computed tomography (CT) is recommended as the first-choice imaging modality, while magnetic resonance imaging (MRI) is suggested for patients with metastatic PPGLs, skull base and neck paragangliomas, or when radiation exposure must be limited. 3. **Genetic Testing**: Genetic testing is recommended for all patients with PPGLs, with a focus on succinate dehydrogenase (SDH) mutations in paragangliomas and SDHB mutations in metastatic disease. 4. **Perioperative Medical Management**: Preoperative blockade with α-adrenergic receptor blockers is recommended for all patients with hormonally functional PPGLs to prevent perioperative cardiovascular complications. 5. **Surgery**: Minimally invasive adrenalectomy is recommended for most adrenal pheochromocytomas, while open resection is suggested for large or invasive tumors. Open resection is also recommended for paragangliomas, but laparoscopic resection can be performed for small, noninvasive tumors. 6. **Personalized Management**: A personalized approach to patient management is recommended, including biochemical testing, imaging, surgery, and follow-up, with multidisciplinary teams providing evaluation and treatment. The guidelines aim to improve the diagnosis, management, and outcomes for patients with PPGLs.The Endocrine Society has developed clinical practice guidelines for the diagnosis, management, and treatment of pheochromocytoma and paraganglioma (PPGLs). The guidelines are based on a systematic review of the literature and the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system. Key recommendations include: 1. **Biochemical Testing**: Initial biochemical testing should include measurements of plasma free or urinary fractionated metanephrines. Liquid chromatography with mass spectrometric or electrochemical detection methods are recommended over other laboratory methods. 2. **Imaging Studies**: Computed tomography (CT) is recommended as the first-choice imaging modality, while magnetic resonance imaging (MRI) is suggested for patients with metastatic PPGLs, skull base and neck paragangliomas, or when radiation exposure must be limited. 3. **Genetic Testing**: Genetic testing is recommended for all patients with PPGLs, with a focus on succinate dehydrogenase (SDH) mutations in paragangliomas and SDHB mutations in metastatic disease. 4. **Perioperative Medical Management**: Preoperative blockade with α-adrenergic receptor blockers is recommended for all patients with hormonally functional PPGLs to prevent perioperative cardiovascular complications. 5. **Surgery**: Minimally invasive adrenalectomy is recommended for most adrenal pheochromocytomas, while open resection is suggested for large or invasive tumors. Open resection is also recommended for paragangliomas, but laparoscopic resection can be performed for small, noninvasive tumors. 6. **Personalized Management**: A personalized approach to patient management is recommended, including biochemical testing, imaging, surgery, and follow-up, with multidisciplinary teams providing evaluation and treatment. The guidelines aim to improve the diagnosis, management, and outcomes for patients with PPGLs.