Abstract
This first part of a two-part review of pheochromocytoma and paragangliomas (PPGLs) addresses clinical presentation, diagnosis, management, treatment, and outcomes. In this first part, the epidemiology, prevalence, genetic etiology, clinical presentation, and biochemical and radiologic workup are discussed. In particular, recent advances in the genetics underlying PPGLs and the recommendation for genetic testing of all patients with PPGL are emphasized. Finally, the newer imaging methods for evaluating of PPGLs are discussed and highlighted.
MeSH terms
-
Abdomen
-
Adrenal Gland Neoplasms / diagnosis*
-
Adrenal Gland Neoplasms / epidemiology
-
Adrenal Gland Neoplasms / genetics
-
Adrenal Gland Neoplasms / physiopathology
-
Arrhythmias, Cardiac / physiopathology
-
Catecholamines / blood
-
Catecholamines / urine
-
Chromaffin Cells / metabolism
-
Ganglia, Parasympathetic
-
Ganglia, Sympathetic
-
Genetic Testing
-
Headache / physiopathology
-
Humans
-
Hypertension / physiopathology
-
Multiple Endocrine Neoplasia Type 2a / diagnosis
-
Multiple Endocrine Neoplasia Type 2a / genetics
-
Multiple Endocrine Neoplasia Type 2b / diagnosis
-
Multiple Endocrine Neoplasia Type 2b / genetics
-
Neoplastic Syndromes, Hereditary / diagnosis
-
Neoplastic Syndromes, Hereditary / genetics
-
Neurofibromatosis 1 / diagnosis
-
Neurofibromatosis 1 / genetics
-
Paraganglioma / diagnosis
-
Paraganglioma / epidemiology
-
Paraganglioma / genetics
-
Paraganglioma / physiopathology
-
Paraganglioma, Extra-Adrenal / diagnosis*
-
Paraganglioma, Extra-Adrenal / epidemiology
-
Paraganglioma, Extra-Adrenal / genetics
-
Paraganglioma, Extra-Adrenal / metabolism
-
Pelvis
-
Pheochromocytoma / diagnosis*
-
Pheochromocytoma / epidemiology
-
Pheochromocytoma / genetics
-
Pheochromocytoma / physiopathology
-
Sweating / physiology
-
von Hippel-Lindau Disease / diagnosis
-
von Hippel-Lindau Disease / genetics