Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Neuroendocrine Neoplasms

  • Annette Schmitt-Graeff
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_4032-2

Synonyms

Definition

Neuroendocrine neoplams (NENs) are neoplasms with a broad range of morphologic patterns, grade of differentiation, and biological behavior that share common features of neuroendocrine (NE) programming. NENs contain various amounts of molecules involved in the regulated release of neuropeptides, neurotransmitters and hormones, and may be associated with hypersecretory syndromes or be nonfunctioning.

It is a matter of ongoing discussion whether NENs originate from precursor cells that are committed to endocrine cell differentiation or from epithelial stem cells. They exhibit phenotypic and functional features observed in cells of the disseminated/diffuse NE system (DES) of endodermal (foregut,...

Keywords

Carcinoid Tumor Small Cell Carcinoma Merkel Cell Carcinoma Small Cell Lung Carcinoma Carcinoid Syndrome 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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Notes

Glossary

Chromogranin A

Chromogranin A is an acid 48 kDa glycoproteins proteins localized to the matrix of large dense core vesicles of neurons and neuroendocrine cells. Immunohistochemical detection or measurement of serum levels is useful for diagnosis and monitoring of neuroendocrine tumors. However, chromogranin A may be absent in poorly differentiated neoplasms. (50)

Disseminated/diffuse neuroendocrine system

Single or small groups of cells of different embryonic origin dispersed throughout the body which share a number of phenotypic and functional features with neural cells while axons are absent. Peptide hormones, biogenic amines, and neurotransmitters are stored in membrane-bound vesicles and released by regulated exocytosis. (49)

Foregut

Foregut neuroendocrine tumors originate from tissues of endodermal origin forming the respiratory tract, lower esophagus, stomach, pancreas, duodenum, and upper jejunum. (13)

Hindgut

Hindgut neuroendocrine tumors are localized in the distal colon and rectum.

Midgut

Midgut neuroendocrine tumors arise in the lower jejunum, ileum, appendix, and caecum.

NSE

NSE (neuron-specific enolase) is a neuron-specific isoform of the glycolytic enzyme 2-hospho-D-glycerate enolase and serves as a cytosolic marker of neurons and neuroendocrine cells.

Rabs

Rabs are members of a family of small guanosine triphosphatases. These components of trafficking organelles are considered as key regulators of membrane fusion events.

SNAP-25

SNAP-25 (25-kDa synaptosome- associated protein) is a SNARE protein of the exocytotic machinery localized at the plasma and synaptic vesicle membranes of neurons and components of the disseminated neuroendocrine system. Isoforms of SNAP-25 are SNAP-23 and the two alternative splice variants SNAP-25a and SNAP-25b which contain two SNARE motifs.

SNARE

SNARE (soluble N-ethylmaleimide-sensitive factor attachment protein receptor) proteins mediate the membrane fusion of trafficking vesicles with the target compartments. These key components of the secretory pathway belong to a superfamily of proteins which share a SNARE motif composed of 60 to 70 amino acids arranged in heptad repeats.

Synaptophysin

Synaptophysin is a membrane protein of small synaptic vesicles which store neurotransmitters and undergo Ca2+ dependent exocytosis in presynaptic nerve terminals. Synaptophysin is the most abundant protein of the synaptic-vesicle like microvesicles of neuroendocrine cells and an excellent marker of well and poorly differentiated neuroendocrine tumors. (50)

TNM staging of gastroenteropancreatic (neuro)endocrine tumors (NET)

Guidelines for tumor-node-metastasis classification and grading of NETs of fore- mid-, and hindgut. Assessment of localization, diameter, extension, and differentiation of the primary tumor, angioinvasion, infiltration of adjacent tissues, distant metastasis, mitotic count/ Ki-67 index, and functionality is required for prognostic stratification.

VAMP1, 2

VAMP1, 2 (vesicle-associated membrane protein) also known as synaptobrevin 1, 2 are SNAREs present in the membranes of synaptic vesicles in brain and neuroendocrine tissues. They are implicated in membrane fusion by forming a tight complex with plasma membrane SNAREs syntaxin and SNAP 25.

References

  1. Bosman FT (2010) World Health Organization, International Agency for Research on Cancer. WHO classification of tumours of the digestive system, 4th edn. International Agency for Research on Cancer, LyonGoogle Scholar
  2. Klöppel G (2011) Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms. Endocr Relat Cancer 18:S1–S16CrossRefPubMedGoogle Scholar
  3. Jiao Y, ShiC EBH et al (2011) DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrinetumors. Science 331:1199–1203CrossRefPubMedPubMedCentralGoogle Scholar
  4. Rindi G, Klersy C, Inzani F et al (2013) Grading the neuroendocrine tumors of the lung: an evidence-based proposal. Endocr Relat Cancer 21:1–16CrossRefPubMedGoogle Scholar
  5. Travis WD, Brambilla E, Burke AP et al (2015a) WHO classification of tumours of the lung, pleura, thymus and heart. International Agency for Research on Cancer, LyonGoogle Scholar
  6. Travis WD, Brambilla E, Nicholson AG et al (2015b) The 2015 World Health Organization classification of lung tumors. Impact of genetic, clinical and radiologic advances since the 2004 classification. J Thorac Oncol 10:1243–1260CrossRefPubMedGoogle Scholar

See Also

  1. (2012) Chromogranin A. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 834. doi:10.1007/978-3-642-16483-5_1135Google Scholar
  2. (2012) Cytokeratins. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1051. doi:10.1007/978-3-642-16483-5_1472Google Scholar
  3. (2012) Eaton-Lambert syndrome. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1200. doi:10.1007/978-3-642-16483-5_1792Google Scholar
  4. (2012) Ki-67. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1943. doi:10.1007/978-3-642-16483-5_3213Google Scholar
  5. (2012) Loss of heterozygosity. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, pp 2075–2076. doi:10.1007/978-3-642-16483-5_3415Google Scholar
  6. (2012) Medullary thyroid carcinoma. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, pp 2199–2200. doi:10.1007/978-3-642-16483-5_3600Google Scholar
  7. (2012) Octreotide. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2597. doi:10.1007/978-3-642-16483-5_4190Google Scholar
  8. (2012) P53. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 2747. doi:10.1007/978-3-642-16483-5_4331Google Scholar
  9. (2012) Serotonin. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3389. doi:10.1007/978-3-642-16483-5_5262Google Scholar
  10. (2012) Tumor suppressor. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3803. doi:10.1007/978-3-642-16483-5_6056Google Scholar
  11. (2012) Zollinger–Ellison syndrome. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3980. doi:10.1007/978-3-642-16483-5_6303Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of PathologyUniversity hospital FreiburgFreiburgGermany