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Role of Immunohistochemistry and Molecular Genetics in Neuroendocrine Tumors

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Neuroendocrine Tumors: Review of Pathology, Molecular and Therapeutic Advances

Abstract

Neuroendocrine tumor (NET) is a family of neoplasms that arises from nervous (neuro-) system and hormonal (endocrine) cells of multiple organs and functions. NET cells in the GI tract share common histological characteristics typically associated with neuroendocrine differentiation. Immunohistochemically, NET cells produce common polypeptide hormones and a variety of biogenic amines related to modulating the biological functions of the organs where these tumors arise. The immunophenotypical profiles of these neoplasms are relatively specific: they are positive for markers of neuroendocrine differentiation and may produce various polypeptides and hormones unique to their subtypes. Immunohistochemical examination of these neuroendocrine markers, such as synaptophysin, chromogranin, CD56, and other markers such as neuron-specific enolase (NSE), as well as proliferative markers (Ki-67 labeling index), should be carried out for the proper classification and grading of any NET. Additionally, in this era of molecular medicine and personalized precision medicine, the molecular background and the involved molecules are gaining more recognition. The current knowledge on the molecular genetics of neuroendocrine tumors will be introduced at the end of this chapter. In short, this chapter will focus on immunophenotypical profile and currently understood molecular genetics of gastrointestinal neuroendocrine tumors.

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Abbreviations

ATRX α Thalassemia/mental retardation syndrome X-linked

DAXX Death-domain-associated protein

NET Neuroendocrine tumor

NSE Neuron-specific enolase

GI Gastrointestinal

SCC Small cell carcinoma

LCNEC Large cell neuroendocrine carcinoma

MANEC Mixed adenoneuroendocrine carcinoma

VHL Von Hippel-Lindau

MEN Multiple endocrine neoplasms

SSTRs Somatostatin receptors (1-5)

mTOR Mammalian target of rapamycin

Author information

Authors and Affiliations

  1. Anatomic Pathology, H. Lee Moffitt Cancer Center, Tampa, FL, USA

    Kun Jiang MD, PHD

  2. Department of Oncologic Science, University of South Florida, Tampa, FL, 33612, USA

    Kun Jiang MD, PHD

  3. Department of Anatomic Pathology, Moffitt Cancer Center, Tampa, FL, USA

    Ujalla Sheikh MD & Domenico Coppola MD

  4. Diagnostic & Experimental Pathology (Tailored Therapeutics), Lilly Research Laboratories, Eli Lilly & Co., Indianapolis, IN, USA

    Aejaz Nasir MD, MPhil, FCAP

Authors
  1. Kun Jiang MD, PHD
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  2. Ujalla Sheikh MD
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  3. Aejaz Nasir MD, MPhil, FCAP
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  4. Domenico Coppola MD
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Corresponding authors

Correspondence to Aejaz Nasir MD, MPhil, FCAP or Domenico Coppola MD .

Editor information

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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Jiang, K., Sheikh, U., Nasir, A., Coppola, D. (2016). Role of Immunohistochemistry and Molecular Genetics in Neuroendocrine 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_4

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  • DOI: https://doi.org/10.1007/978-1-4939-3426-3_4

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