Skip to main content

Abstract

Neuroendocrine tumor (NET) arises at multiple sites in the gastrointestinal (GI) tract; in fact GI tract is among the most common sites for NET in human. GI NET may present as well-differentiated neoplasms, further classified in grade 1 and grade 2 depending on the presence or absence of necrosis and on mitotic activity; or they may present as grade 3 neuroendocrine carcinomas which include large cell and small cell carcinoma. This chapter will be focusing on differentiating neuroendocrine neoplasms from other types of morphologically similar neoplasms encountered in daily practice, predominantly those encountered in the GI tract.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 99.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 129.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 179.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Rindi G, Petrone G, Inzani F. 25 Years of neuroendocrine neoplasms of the gastrointestinal tract. Endocr Pathol. 2014;25:59–64.

    Article  CAS  PubMed  Google Scholar 

  2. Hirabayashi K, Zamboni G, Nishi T, Tanaka A, Kajiwara H, Nakamura N, et al. Histopathology of gastrointestinal neuroendocrine neoplasms. Front Oncol. 2013;3:2.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Anthony LB, Strosberg JR, Klimstra DS, Maples WJ, O’Dorisio TM, Warner RR, et al. The NANETS consensus guidelines for the diagnosis and management of gastrointestinal neuroendocrine tumors (nets): well-differentiated nets of the distal colon and rectum. Pancreas. 2010;39:767–74.

    Article  PubMed  Google Scholar 

  4. Kloppel G, Rindi G, Perren A, Komminoth P, Klimstra DS. The ENETS and UICC TNM classification of neuroendocrine tumors of the gastrointestinal tract and the pancreas: comment. Pathologe. 2010;31:353–4.

    Article  CAS  PubMed  Google Scholar 

  5. Leja J, Essaghir A, Essand M, Wester K, Oberg K, Totterman TH, et al. Novel markers for enterochromaffin cells and gastrointestinal neuroendocrine carcinomas. Mod Pathol. 2009;22:261–72.

    Article  CAS  PubMed  Google Scholar 

  6. Castano JP, Sundin A, Maecke HR, Villabona C, Vazquez-Albertino R, Navarro E, et al. Gastrointestinal neuroendocrine tumors (NETs): new diagnostic and therapeutic challenges. Cancer Metastasis Rev. 2014;33:353–9.

    Article  CAS  PubMed  Google Scholar 

  7. Fottner C, Weber MM. Neuroendocrine neoplasms of the gastrointestinal tract. Classification, clinical presentation and diagnosis. Internist (Berl). 2012;53:131–44.

    Article  CAS  Google Scholar 

  8. Verset L, Arvanitakis M, Loi P, Closset J, Delhaye M, Remmelink M, et al. TTF-1 positive small cell cancers: don’t think they’re always primary pulmonary! World J Gastrointest Oncol. 2011;3:144–7.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Schott M, Kloppel G, Raffel A, Saleh A, Knoefel WT, Scherbaum WA, et al. Neuroendocrine neoplasms of the gastrointestinal tract. Dtsch Arztebl Int. 2011;108:305–12.

    PubMed  PubMed Central  Google Scholar 

  10. Rorstad O. Prognostic indicators for carcinoid neuroendocrine tumors of the gastrointestinal tract. J Surg Oncol. 2005;89:151–60.

    Article  PubMed  Google Scholar 

  11. Grabowski P, Scherubl H. Expression of neuroendocrine markers in undifferentiated carcinomas of the gastrointestinal tract. J Clin Oncol. 2005;23:4795–6; author reply 4796–7.

    Article  PubMed  Google Scholar 

  12. Oberg K. Neuroendocrine tumors of the gastrointestinal tract: recent advances in molecular genetics, diagnosis, and treatment. Curr Opin Oncol. 2005;17:386–91.

    Article  PubMed  Google Scholar 

  13. Norton JA. Endocrine tumours of the gastrointestinal tract. Surgical treatment of neuroendocrine metastases. Best Pract Res Clin Gastroenterol. 2005;19:577–83.

    Article  PubMed  Google Scholar 

  14. Nikou GC, Lygidakis NJ, Toubanakis C, Pavlatos S, Tseleni-Balafouta S, Giannatou E, et al. Current diagnosis and treatment of gastrointestinal carcinoids in a series of 101 patients: the significance of serum chromogranin-A, somatostatin receptor scintigraphy and somatostatin analogues. Hepatogastroenterology. 2005;52:731–41.

    PubMed  Google Scholar 

  15. La Rosa S, Marando A, Sessa F, Capella C. Mixed adenoneuroendocrine carcinomas (MANECs) of the gastrointestinal tract: an update. Cancers (Basel). 2012;4:11–30.

    Article  PubMed Central  Google Scholar 

  16. Timek DT, Shi J, Liu H, Lin F. Arginase-1, HepPar-1, and Glypican-3 are the most effective panel of markers in distinguishing hepatocellular carcinoma from metastatic tumor on fine-needle aspiration specimens. Am J Clin Pathol. 2012;138:203–10.

    Article  PubMed  Google Scholar 

  17. Sangoi AR, Ohgami RS, Pai RK, Beck AH, McKenney JK. PAX8 expression reliably distinguishes pancreatic well-differentiated neuroendocrine tumors from ileal and pulmonary well-differentiated neuroendocrine tumors and pancreatic acinar cell carcinoma. Mod Pathol. 2011;24:412–24.

    Article  CAS  PubMed  Google Scholar 

  18. Erickson LA, Papouchado B, Dimashkieh H, Zhang S, Nakamura N, Lloyd RV, et al. Cdx2 as a marker for neuroendocrine tumors of unknown primary sites. Endocr Pathol. 2004;15:247–52.

    Article  CAS  PubMed  Google Scholar 

  19. Du EZ, Goldstraw P, Zacharias J, Tiffet O, Craig PJ, Nicholson AG, et al. TTF-1 expression is specific for lung primary in typical and atypical carcinoids: TTF-1-positive carcinoids are predominantly in peripheral location. Hum Pathol. 2004;35:825–31.

    Article  CAS  PubMed  Google Scholar 

  20. Vassos N, Agaimy A, Klein P, Hohenberger W, Croner RS. Solid-pseudopapillary neoplasm (SPN) of the pancreas: case series and literature review on an enigmatic entity. Int J Clin Exp Pathol. 2013;6:1051–9.

    PubMed  PubMed Central  Google Scholar 

  21. Chetty R, Serra S. Loss of expression of E-cadherin in solid pseudopapillary tumors of the pancreas. Pancreas. 2009;38:338; author reply 338–9.

    Article  PubMed  Google Scholar 

  22. Jiang K, Lawson D, Cohen C, Siddiqui MT. Galectin-3 and PTEN expression in pancreatic ductal adenocarcinoma, pancreatic neuroendocrine neoplasms and gastrointestinal tumors on fine-needle aspiration cytology. Acta Cytol. 2014;58:281–7.

    Article  CAS  PubMed  Google Scholar 

  23. Williams GT. Endocrine tumours of the gastrointestinal tract-selected topics. Histopathology. 2007;50:30–41.

    Article  CAS  PubMed  Google Scholar 

  24. McNicol AM. Histopathology and immunohistochemistry of adrenal medullary tumors and paragangliomas. Endocr Pathol. 2006;17:329–36.

    Article  CAS  PubMed  Google Scholar 

  25. He J, Wang X, Zheng W, Zhao Y. Retroperitoneal paraganglioma with metastasis to the abdominal vertebra: a case report. Diagn Pathol. 2013;8:52.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Hayes-Jordan A, Anderson PM. The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol. 2011;23:385–9.

    Article  PubMed  Google Scholar 

  27. Fischer S, Asa SL. Application of immunohistochemistry to thyroid neoplasms. Arch Pathol Lab Med. 2008;132:359–72.

    PubMed  Google Scholar 

Download references

Abbreviations

NET Neuroendocrine tumor

NEC Neuroendocrine carcinoma

NSE Neuron-specific enolase

GI Gastrointestinal

SCC Small cell carcinoma

LCNEC Large-cell neuroendocrine carcinoma

MANEC Mixed adenoneuroendocrine carcinoma

SPN Solid pseudopapillary neoplasm

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Kun Jiang MD, PHD .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Jiang, K., Coppola, D. (2016). Differential Diagnosis of 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_3

Download citation

  • DOI: https://doi.org/10.1007/978-1-4939-3426-3_3

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-3424-9

  • Online ISBN: 978-1-4939-3426-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics