Frank Berthold Thorsten Simon

Contents

7.1 Introduction 63

7.2 Diagnosis 64

7.2.1 Diagnostic Tumor Tissue 64

7.3 Clinical Presentation 65

7.3.1 Symptoms 65

7.3.1.1 Frequent Symptoms 65

7.3.1.2 Rare but Characteristic Symptoms 66

7.3.2 Tumor Markers 67

7.3.2.1 Catecholamine Metabolites 68

7.3.2.2 Neuron-Specific Enolase 68

7.3.2.3 Ferritin 68

7.3.2.4 Lactate Dehydrogenase 68

7.3.2.5 Other Tumor Markers 68

7.3.3 Primary Tumors 69

7.3.3.1 Sites of the Primary Tumor 69

7.3.4 Metastases 69

7.3.4.1 Metastatic Sites 69

7.3.4.2 Bone Marrow Assessment 70

7.3.4.3 Definition of Cortical Bone Metastases. ... 72

7.4 Differential Diagnosis 72

7.4.1 Small Blue Round Cell Tumors 72

7.4.2 Adrenal Hemorrhage in the Newborn ... 72

7.4.3 Nephroblastoma 73

7.4.4 Esthesioneuroblastoma

(Olfactory Neuroblastoma) 73

7.4.5 Ganglioneuroma, Pheochromocytoma, Paraganglioma,Chemodectoma 73

7.5 Clinical and Laboratory Evaluation 73

7.5.1 Staging 73

7.5.2 Biological Types of Neuroblastoma 75

7.5.2.1 Maturative Subtype 75

7.5.2.2 Regressive Subtype 76

7.5.2.3 Progressive Subtype 77

7.5.3 Prognostic Risk Groups 77

7.5.4 Response Criteria 82

7.6 Conclusions 82

References 83

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