Pheochromocytoma (eBook)

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.

Pheochromocytoma Diagnosis, Localization, and Treatment 1
Contents 7
1 Introduction 13
2 Historical comments 15
3 Pathology 16
4 Clinical presentation of pheochromocytoma 20
4.1 Signs and Symptoms 20
4.2 Differential Diagnosis 24
4.3 Special Presentations 26
4.3.1 Diagnosis of Pheochromocytoma in Patients with an Incidentally Discovered Adrenal Mass 26
4.3.2 Pheochromocytoma as an Endocrine Emergency 27
4.3.2.1 Hypertensive Crisis 27
4.3.2.2 Hypotension and Shock 28
4.3.2.3 Multisystem Failure 28
4.3.2.4 Cardiac Emergencies 29
4.3.2.5 Acute Peripheral Ischemia 30
4.3.2.6 Pulmonary Emergencies 31
4.3.2.7 Gastrointestinal Emergencies 31
4.3.2.8 Nephrological Emergencies 31
4.3.2.9 Neurological Emergencies 32
4.3.3 Malignant Pheochromocytoma 32
4.3.4 Pheochromocytoma in Children 36
4.3.5 Pheochromocytoma in Pregnancy 38
4.3.6 Pseudopheochromocytoma 40
4.3.7 Factitious Pheochromocytoma 41
5 Current trends in genetics of pheochromocytoma 42
5.1 MEN Syndromes 42
5.1.1 Diagnostic Approaches 45
5.2 VHL Syndrome 46
5.3 NF Type 1 48
5.4 Succinate Dehydrogenase Gene Related Pheochromocytoma 49
5.5 Genetic Problems in Sporadic and Other Pheochromocytomas 50
6 Catecholamines and adrenergic receptors 53
6.1 Synthesis and Sources of Catecholamines 53
6.2 Synthesis of Catecholamines in Pheochromocytoma 55
6.3 Storage and Release of Catecholamines by the Sympathoadrenal System 57
6.4 Uptake and Metabolism of Catecholamines Produced by the Sympathoadrenal System 58
6.5 Catecholamine Metabolism in Hepatomesenteric Organs 63
6.6 Catecholamines Metabolism and Release by Pheochromocytoma 66
6.7 Kinetics and Elimination of Catecholamines and Their Metabolites 70
6.8 Pharmacology of Catecholamine Systems: Implications for Pheochromocytoma 72
6.9 Physiology of Catecholamine Systems 72
6.9.1 Adrenal Medullary Hormone System 72
6.9.2 Peripheral Dopamine Systems 74
6.10 Adrenergic Receptors and Their Functions 76
6.11 Actions of the Catecholamines 81
7 Current trends in biochemical diagnosis of pheochromocytoma 84
7.1 Biochemical Tests of Catecholamine Excess 84
7.2 Measurement Methods 86
7.3 Reference Intervals 88
7.4 Initial Biochemical Testing 90
7.5 Follow-up Biochemical Testing 93
7.6 Collection and Storage of Plasma and Urine Specimens 96
7.7 Interferences from Diet and Drugs 97
7.8 Pharmacologic Tests 100
7.9 Additional Interpretative Considerations 103
7.10 Summary 103
8 Current trends in localization of pheochromocytoma 105
8.1 Anatomical Imaging of Pheochromocytoma 106
8.1.1 Computed Tomography 106
8.1.2 Magnetic Resonance Imaging 108
8.2 Functional Imaging of Pheochromocytoma 109
8.2.1 MIBG Scintigraphy 111
8.2.2 Positron Emission Tomography 112
8.2.3 Somatostatin Receptor Scintigraphy (Octreoscan) 117
8.2.4 Current Imaging Algorithm 120
9 Treatment of pheochromocytoma 121
9.1 Medical Therapy and Preparation for Surgery 122
9.2 Postoperative Management 124
10 Future trends and perspectives 126
10.1 Genomics in Pheochromocytoma Research 126
10.2 Proteomics in Pheochromocytoma Research 127
10.3 Future Therapeutic Modalities for Pheochromocytoma 130
References 132
Index 179
A 179
B 179
C 179
D 180
E 180
F 181
G 181
H 181
I 181
K 181
L 181
M 182
N 182
O 183
P 183
Q 183
R 183
S 184
T 184
U 184
V 184
W 184
Y 184
Z 184

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