Pheochromocytoma

Professor Karel Pacak put together the Pheochromocytoma Research Program at the NIH, one of the most prestigious and largest programs world-wide; published over 80 articles/book chapters on this topic. Introduced new biochemical and imaging approaches to this tumor, especially the use of 18F-fluorodopamine in localization of pheochromocytoma. He also developed new biochemical and imaging algorithms for pheochromocytoma for practicing physicians. Moreover, developed new animal model of metastatic pheochromocytoma. Currently working as the Chief of Section on Medical Neuroendocrinology, NICHD. He is also Professor of Medicine at Georgetown University, Washington DC and Charles University, Prague, Czech Republic. Dr. Eisenhofer is a clinical biochemist with broad experience in basic and patient-oriented research on neuroedocrine and autonomic nervous system function in health and disease. Relevant achievements include codevelopment with Dr. Jacques Lenders of the first HPLC method for measurements of plasma free metanephrines, a test now used for improved biochemical diagnosis of pheochromocytoma. Dr. Eisenhofer was also responsible for initial development of 18F-fluorodopamine as a positron emission tomographic imaging agent for visualizing sympathetic nerves and neuroendocrine tumors. Dr. Eisenhofer co-chairs the Pheochromocytoma RESsearch Support ORganization (PRESSOR - http://www.pressor.org). Professor Jacques Lenders did his medical study at the University of Nijmegen, the Netherlands. In 1982 he finished his training for internist and in 1988 he defended his thesis on ‘Blood pressure and catecholamines in essential hypertension’. From august 1991 till january 1993 he was ‘visiting associate’ at the ‘Clinical Neuroscience Branch’ of the ‘National Institute of Neurological Disorders and Stroke’ (National Institutes of Health’, Bethesda, USA). In 2005 he was appointed as professor of Vascular Medicine at the University Medical Center in Nijmegen, The Netherlands. He is deputy-chairman of the Department of General Internal Medicine.

1 Introduction. 2 Historical comments.

3 Pathology.

4 Clinical presentation of pheochromocytoma.

4.1 Signs and Symptoms.

4.2 Differential Diagnosis.

4.3 Special Presentations.

4.3.1 Diagnosis of Pheochromocytoma in Patients with an Incidentally Discovered Adrenal Mass.

4.3.2 Pheochromocytoma as an Endocrine Emergency.

4.3.2.1 Hypertensive Crisis.

4.3.2.2 Hypotension and Shock.

4.3.2.3 Multisystem Failure.

4.3.2.4 Cardiac Emergencies.

4.3.2.5 Acute Peripheral Ischemia.

4.3.2.6 Pulmonary Emergencies.

4.3.2.7 Gastrointestinal Emergencies.

4.3.2.8 Nephrological Emergencies.

4.3.2.9 Neurological Emergencies.

4.3.3 Malignant Pheochromocytoma.

4.3.4 Pheochromocytoma in Children.

4.3.5 Pheochromocytoma in Pregnancy.

4.3.6 Pseudopheochromocytoma.

4.3.7 Factitious Pheochromocytoma.

5 Current trends in genetics of pheochromocytoma.

5.1 MEN Syndromes.

5.1.1 Diagnostic Approaches.

5.2 VHL Syndrome.

5.3 NF Type 1.

5.4 Succinate Dehydrogenase Gene Related Pheochromocytoma.

5.5 Genetic Problems in Sporadic and Other Pheochromocytomas.

6 Catecholamines and adrenergic receptors.

6.1 Synthesis and Sources of Catecholamines.

6.2 Synthesis of Catecholamines in Pheochromocytoma.

6.3 Storage and Release of Catecholamines by the Sympathoadrenal System.

6.4 Uptake and Metabolism of Catecholamines Produced by the Sympathoadrenal System.

6.5 Catecholamine Metabolism in Hepatomesenteric Organs.

6.6 Catecholamines Metabolism and Release by Pheochromocytoma.

6.7 Kinetics and Elimination of Catecholamines and Their Metabolites.

6.8 Pharmacology of Catecholamine Systems: Implications for Pheochromocytoma.

6.9 Physiology of Catecholamine Systems.

6.9.1 Adrenal Medullary Hormone System.

6.9.2 Peripheral Dopamine Systems.

6.10 Adrenergic Receptors and Their Functions.

6.11 Actions of the Catecholamines.

7 Current trends in biochemical diagnosis of pheochromocytoma.

7.1 Biochemical Tests of Catecholamine Excess.

7.2 Measurement Methods.

7.3 Reference Intervals.

7.4 Initial Biochemical Testing.

7.5 Follow-up Biochemical Testing.

7.6 Collection and Storage of Plasma and Urine Specimens.

7.7 Interferences from Diet and Drugs.

7.8 Pharmacologic Tests.

7.9 Additional Interpretative Considerations.

7.10 Summary.

8 Current trends in localization of pheochromocytoma.

8.1 Anatomical Imaging of Pheochromocytoma.

8.1.1 Computed Tomography.

8.1.2 Magnetic Resonance Imaging.

8.2 Functional Imaging of Pheochromocytoma.

8.2.1 MIBG Scintigraphy.

8.2.2 Positron Emission Tomography.

8.2.3 Somatostatin Receptor Scintigraphy (Octreoscan).

8.2.4 Current Imaging Algorithm.

9 Treatment of pheochromocytoma.

9.1 Medical Therapy and Preparation for Surgery.

9.2 Postoperative Management.

10 Future trends and perspectives.

10.1 Genomics in Pheochromocytoma Research.

10.2 Proteomics in Pheochromocytoma Research.

10.3 Future Therapeutic Modalities for Pheochromocytoma.

References.

Index