Mayo Clinic, Rochester, Minnesota, USA.
Objective: To review the first reported cases of successfully treated pheochromocytoma and primary aldosteronism and document the diagnostic and therapeutic advances that have occurred since the initial descriptions.
Methods: The original case descriptions and the subsequent pertinent literature were reviewed.
Results: The successful management of the initial cases of pheochromocytoma in 1926 and primary aldosteronism in 1954 was highlighted by keen clinical observation, clinical intuition, and the application of scientific principles. Since those prismatic case descriptions, the technological advances in laboratory-based diagnosis, radiology-based tumor localization, and surgical approaches to the adrenal glands have been truly remarkable.
Conclusions: The evolution in the diagnosis and treatment of pheochromocytoma will continue to progress as we identify more genetic causes, develop biochemical markers for pre-clinical pheochromocytoma, identify better markers for malignant disease, and develop more effective treatment options for malignant pheochromocytoma. Over the next decade we hope to determine the pathophysiology for bilateral idiopathic hyperaldosteronism, develop less invasive and less technically demanding tests to distinguish between unilateral aldosterone-producing adenoma and bilateral idiopathic hyperaldosteronism, determine where low renin hypertension stops and primary aldosteronism starts, and determine the impact of genetic and environmental factors on aldosterone secretion in patients with and without primary aldosteronism.