Endocrine Abstracts

The first patient with a catecholamine-secreting paraganglioma that was sucessfully resected was in 1926. Over the past 97 years, there has been a dramatic evolution in the clinical presentation, methods used to diagnose and localize, and germline genetic testing of paragangliomas (PGLs). When contrasted to adrenal pheochromocytoma, PGLs present the clinician with unique challenges, which include: 1) diverse location—they may be located anywhere from the tympanic membrane in the ear to the scrotum; 2) markedly variable size at detection—from 0.5 to 25 cm; 3) secretory status—sympathetic PGLs typically hypersecrete catecholamines (some uniquely secrete dopamine) and are located primarily between the mid-thorax to the pelvis; whereas, parasympathetic PGLs usually do not hypersecrete catecholamines and are located primarily between the skull base and upper mediastinum; 4) multiplicity—PGLs are frequently multiple and associated with pathogenic variants in the succinate dehydrogenase subunit genes; and, 5) increased risk of metastatic disease when compared to adrenal pheochromocytoma. These challenges will be discussed and practical clinical perspectives provided.