Neuroblastoma and Other Adrenal Tumors

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


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.


Adrenal tumors Neuroblastoma Pheochromocytoma Adrenocortical carcinoma Aldosteronoma Adrenal adenoma Adrenal calcification 



A murine IgG3 monoclonal antibody that binds to GD2


Adrenocorticotropic hormone


Anaplastic lymphoma kinase


Brain-derived neurotrophic factor


Children’s Cancer Group


Children’s Oncology Group


Computed tomography


Cytochrome P




Fluorescence in situ hybridization


Granulocyte colony stimulating factor


Disialyl ganglioside 2


Glial cell line-derived neurotrophic factor


Homovanillic acid


Interleukin 2


International Neuroblastoma Pathology Committee


International Neuroblastoma Staging System




Lactate dehydrogenase


Loss of heterozygosity


Multiple endocrine neoplasia




Magnetic resonance imaging


Neurofibromatosis type I


Nerve growth factor


Myelocytomatosis viral related oncogene neuroblastoma derived


Polymerase chain reaction


Primitive neuro-ectodermal tumor


“rearranged during transfection”




Tyrosine kinase receptor-nerve growth factor


Tyrosine kinase family of receptors


Vascular endothelial growth factor


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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