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The Surgery of Childhood Tumors pp 231–256Cite as

Neuroblastoma and Other Adrenal Tumors

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Abstract

This chapter addresses the medical presentation, histology, biological features, risk stratification, and surgical management of malignant adrenal disease. Neuroblastoma is the most common adrenal neoplasm in the pediatric population, accounting for 97 % of adrenal neoplasms in children younger than 15 months. It is 50 times more common than pheochromocytoma, the next most common tumor. The good prognosis associated with early-stage, biologically favorable disease, with a survival rate of more than 90 %, stands in dramatic contradistinction to the poor prognosis associated with metastatic or biologically unfavorable presentations. Treatment is typically determined by the risk stratification schemes of the Children’s Oncology Group, the International Neuroblastoma Staging System, and the International Neuroblastoma Research Group.

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Abbreviations

3F8:

A murine IgG3 monoclonal antibody that binds to GD2

ACTH:

Adrenocorticotropic hormone

ALK:

Anaplastic lymphoma kinase

BDNF:

Brain-derived neurotrophic factor

CCG:

Children’s Cancer Group

COG:

Children’s Oncology Group

CT:

Computed tomography

CYP:

Cytochrome P

DHEA:

dehydroepiandrosterone

FISH:

Fluorescence in situ hybridization

GCSF:

Granulocyte colony stimulating factor

GD2:

Disialyl ganglioside 2

GDNF:

Glial cell line-derived neurotrophic factor

HVA:

Homovanillic acid

IL-2:

Interleukin 2

INPC:

International Neuroblastoma Pathology Committee

INSS:

International Neuroblastoma Staging System

IV:

intravenous

LDH:

Lactate dehydrogenase

LOH:

Loss of heterozygosity

MEN:

Multiple endocrine neoplasia

MIBG:

Metaiodobenzylguanidine

MRI:

Magnetic resonance imaging

NF1:

Neurofibromatosis type I

NGF:

Nerve growth factor

N-MYC:

Myelocytomatosis viral related oncogene neuroblastoma derived

PCR:

Polymerase chain reaction

PNET:

Primitive neuro-ectodermal tumor

RET:

“rearranged during transfection”

TNM:

Tumor-node-metastasis

TRK-NGF:

Tyrosine kinase receptor-nerve growth factor

TRKS:

Tyrosine kinase family of receptors

VEGF:

Vascular endothelial growth factor

VMA:

Vanillylmandelic acid

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Authors and Affiliations

  1. Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY, USA

    Emily R. Christison-Lagay MD & Michael P. La Quaglia MD, FACS, FAAP, FRCS (Ed Hon)

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  1. Emily R. Christison-Lagay MD
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  2. Michael P. La Quaglia MD, FACS, FAAP, FRCS (Ed Hon)
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Correspondence to Michael P. La Quaglia MD, FACS, FAAP, FRCS (Ed Hon) .

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  1. Surgical Paediatrics, University of Glasgow, Glasgow, United Kingdom

    Robert Carachi

  2. Indianapolis, Indiana, USA

    Jay L. Grosfeld

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Christison-Lagay, E.R., La Quaglia, M.P. (2016). Neuroblastoma and Other Adrenal Tumors. In: Carachi, R., Grosfeld, J. (eds) The Surgery of Childhood Tumors. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-48590-3_14

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