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Neuroblastoma

10

 

Paul Imbach

 

Contents

10.1

Definition ...................................................................................................................

114

10.2

Incidence

...................................................................................................................

114

10.3

Etiology and Pathogenesis.........................................................................................

114

10.4

Molecular Cytogenetics .............................................................................................

115

10.5

Pathology ...................................................................................................................

115

 

10.5.1

Macroscopic Features ................................................................................

115

 

10.5.2

Microscopic Features.................................................................................

116

10.6

Clinical Manifestations..............................................................................................

116

 

10.6.1

Common Symptoms ..................................................................................

116

 

10.6.2

Symptoms Associated with Catecholamine Production ............................

117

 

10.6.3

Paraneoplastic Syndromes .........................................................................

117

 

10.6.4

Local Symptoms and Classic Signs...........................................................

117

10.7

Metastatic Spread.......................................................................................................

118

10.8

Laboratory Findings...................................................................................................

118

 

10.8.1

Urinary Catecholamine Metabolites (Tyrosine Metabolism) ....................

118

 

10.8.2

Other Laboratory Findings ........................................................................

119

 

10.8.3

Bone Marrow .............................................................................................

119

10.9

Diagnostic Imaging....................................................................................................

119

 

10.9.1

Conventional X-Ray ..................................................................................

119

 

10.9.2

Methylisobenzyl Guanidinium (MIBG) Scintigraphy...............................

119

10.9.3Ultrasound, Computed Tomography CT and/or MRI or PET/CT, if Not Enough Information by MIBG Scan

 

and Bone Scan...........................................................................................................

119

 

10.9.4

Bone Scintigraphy (Technetium) ...............................................................

119

10.10

Differential Diagnosis................................................................................................

120

10.11

International Staging (including the classic Evans staging) ......................................

120

 

10.11.1

The International Neuroblastoma Risk Group Classification....................

120

10.12

Therapy

...................................................................................................................

121

 

10.12.1

Surgical Procedure.....................................................................................

121

 

10.12.2

Chemotherapy............................................................................................

121

 

10.12.3

Radiotherapy..............................................................................................

121

 

10.12.4

Risk-Adapted Management .......................................................................

121

 

10.12.5

Therapy in Relapse ....................................................................................

122

10.13

Prognosis

...................................................................................................................

122

 

10.13.1

Futuristic Therapeutic Approaches............................................................

123

P. Imbach et al. (eds.), Pediatric Oncology,

113

DOI 10.1007/978-3-642-20359-6_10, © Springer-Verlag Berlin Heidelberg 2011

 

114

 

P. Imbach

 

 

10.14 Special Forms ............................................................................................................

123

10.14.1

Ganglioneuroblastoma...............................................................................

123

10.14.2

Ganglioneuroma ........................................................................................

123

10.14.3

Olfactory Neuroblastoma...........................................................................

124

10.14.4Neuroblastoma Arising from Organ of Zuckerkandl (Location at the Bifurcation of the Aorta or Origin of the Inferior Mesenteric

Artery)........................................................................................................

124

10.14.5 Pheochromocytoma ...................................................................................

124

10.1Definition

Malignant embryonal tumor of precursor cells of sympathetic ganglia and adrenal medulla

Entity characterized by:

Spontaneous regression and differentiation to benign tumor, especially in infants less than 12 months of age is high

In children >18 months of age and in the advanced stage neuroblastoma is extremely malignant

10.2Incidence

Eight percent of all neoplasia in childhood

Annually, new diagnosis in 11 in 1 million children less than 18 years of age

Frequent solid neoplasia in infants

Mean age at diagnosis, 2.5 years

Cumulative age distribution

<1 year of age

35%

<2 years of age

50%

<4 years of age

75%

<10 years of age

90%

Rarely observed in adolescents and adults

Male:Female ratio of 1.1:1.0

10.3Etiology and Pathogenesis

Etiology unknown

Incidence of neuroblastic precursor cells in autopsies of infants less than 3 months old who have died from other causes is 40 times higher than expected

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