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Adrenal Neuroendocrine Tumors: Pheochromocytoma and Neuroblastic Tumors

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Neuroendocrine Tumors: Review of Pathology, Molecular and Therapeutic Advances

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Abstract

The adrenal medulla forms from sympathetic ganglion arising from the neural crest. Primitive sympathecoblasts mature into chromaffin cells which produce catecholamines. Pheochromocytomas are tumors arising from the chromaffin cells within the adrenal gland. These tumors are termed paraganglioma if arising from chromaffin cells outside of the adrenal. In embryonic development, sympathecoblasts form clusters which during embryogenesis can remain in or nearby the adrenal. These primitive clusters are hypothesized to be the source of neuroblastic tumors, such as neuroblastoma, and can arise throughout the sympathoadrenal neuroendocrine system.

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Abbreviations

131I-MIBG 131-iodine-labeled metaiodobenzylguanidine

HIF alpha Hypoxia-inducible factor alpha

IDRF Image-defined risk factors

INPC International Neuroblastoma Pathology Committee

INRG International Neuroblastoma Risk Group

INSS International Neuroblastoma Staging System

MAX MYC-associated factor X

MEN2 Multiple endocrine neoplasia type 2

MKI Mitosis-karyorrhexis

NF1 Neurofibromatosis type 1

PASS Pheochromocytoma of the Adrenal Gland Scaled Score

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Authors and Affiliations

  1. Department of Pathology and Immunology Campus, Washington University, St. Louis, MO, USA

    Carmen M. Perrino MD

  2. Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH, USA

    Debra L. Zynger MD

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  1. Carmen M. Perrino MD
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  2. Debra L. Zynger MD
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Correspondence to Carmen M. Perrino MD .

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Editors and Affiliations

  1. Diagnostic & Experimental Pathology Lilly Res Laboratories, Eli Lilly & Co., Indianapolis, Indiana, USA

    Aejaz Nasir

  2. H. Lee Moffitt Cancer Ctr & Res, University of South Florida, Tampa, Florida, USA

    Domenico Coppola

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Perrino, C.M., Zynger, D.L. (2016). Adrenal Neuroendocrine Tumors: Pheochromocytoma and Neuroblastic Tumors. In: Nasir, A., Coppola, D. (eds) Neuroendocrine Tumors: Review of Pathology, Molecular and Therapeutic Advances. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-3426-3_17

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