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Molecular Pathology and Diagnostics of Pancreatic Endocrine Neoplasms

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Abstract

Pancreatic Endocrine Neoplasms (PENs) are a group of rare tumors thought to arise from the endocrine cells of the pancreas. These tumors may be functional (hormone producing), or non functional. They have a wide range of presenting symptoms. Recent research efforts have shown the complex biology of these tumors, and have started to uncover the molecular alterations responsible for the genesis of these neoplasms. In this chapter we give an overview of the molecular tests available to detect such alterations, and of their diagnostic and prognostic significance.

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Abbreviations

AP1:

Activator protein-1

bFGF:

Basic fibroblast growth factor

CLP-PET:

Clinically localized pancreatic endocrine tumors

CPGs:

Candidate progression genes

EFR1:

Estrogen receptor gene

FHIT:

Fragile histidine triad

hMLH1:

Human MutL homologue

IGF:

Insulin-like growth factor

IGFBP-3:

Insulin-like growth factor binding protein 3

IHC:

Immunohistochemistry

LOH:

Loss of heterozygosity

MAG:

Metastasis-associated gene

MECC:

Monohormonal endocrine cell clusters

MEN1:

Multiple endocrine neoplasia type 1

MP:

Metastatic primary

mTOR:

Mammalian target of rapamycin

NF1:

Neuofibromatosis type 1

NGF:

Nerve growth factor

PECAs:

Pancreatic endocrine carcinomas

PENS:

Pancreatic endocrine neoplasm

PETs:

Pancreatic endocrine tumors

PRAD-1:

Parathyroid adenoma-related protein

SDHD:

Succinate dehydrogenase subunit D

SST:

Somatostatin

SSTRs:

G-protein-coupled transmembrane receptors

TGF:

Transforming growth factor

TSC1, TSC2:

Tuberous sclerosis genes 1 & 2

VHL:

von Hippel-Lindau genes

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Acknowledgments

The authors would like to acknowledge the excellent support from Brenda Barrow and Rasa Hamilton of Moffitt Cancer Center, Tampa, FL, USA.

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Nasir, A. et al. (2014). Molecular Pathology and Diagnostics of Pancreatic Endocrine Neoplasms. In: Coppola, D. (eds) Molecular Pathology and Diagnostics of Cancer. Cancer Growth and Progression, vol 16. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-7192-5_14

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