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Neuroblastoma and Other Adrenal Tumors

  • Emily R. Christison-Lagay
  • Michael P. La QuagliaEmail author
Chapter

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.

Keywords

Adrenal tumors Neuroblastoma Pheochromocytoma Adrenocortical carcinoma Aldosteronoma Adrenal adenoma Adrenal calcification 

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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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Emily R. Christison-Lagay
    • 1
  • Michael P. La Quaglia
    • 1
    Email author
  1. 1.Department of SurgeryMemorial Sloan Kettering Cancer CenterNew YorkUSA

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