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Well-Differentiated Neuroendocrine Tumors of the pancreas (PanNET Grade 1,2,3)

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  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 26 Accesses

Synonyms

Islet cell tumor; Pancreatic endocrine tumor

Definition

PanNET is a well-differentiated epithelial neoplasm with morphological and immunohistochemical features of neuroendocrine differentiation, of low, intermediate, or high grade. PanNETs associated with specific clinical syndromes related to hormone hypersecretion are defined functioning PanNETs, while PanNETs not associated with specific endocrine syndromes are defined nonfunctioning, although they can be symptomatic presenting local symptoms due to tumor growth.

Clinical Classification of PanNET

  • Nonfunctioning PanNETs are not associated with specific endocrine syndromes, but most tumors are positive for either pancreatic (insulin, glucagon, somatostatin, pancreatic polypeptide) or ectopic hormones (gastrin, vasoactive intestinal peptide, ACTH, serotonin, neurotensin) when tested with immunohistochemistry.

  • Insulinoma: accounts for 40% of functioning PanNETs, secretes insulin, proinsulin, and amylin (islet amyloid...

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References and Further Reading

  • Basturk, O., Yang, Z., Tang, L. H., Hruban, R. H., Adsay, V., McCall, C. M., Krasinskas, A. M., Jang, K. T., Frankel, W. L., Balci, S., Sigel, C., & Klimstra, D. S. (2015). The high-grade (WHO G3) pancreatic neuroendocrine tumor category is morphologically and biologically heterogenous and includes both well differentiated and poorly differentiated neoplasms. The American Journal of Surgical Pathology, 39, 683–690.

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  • Falconi, M., Eriksson, B., Kaltsas, G., Bartsch, D. K., Capdevila, J., Caplin, M., Kos-Kudla, B., Kwekkeboom, D., Rindi, G., Klöppel, G., Reed, N., Kianmanesh, R., Jensen, R. T., & Vienna Consensus Conference participants. (2016). ENETS consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology, 103, 153–171.

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  • Klimstra, D. S., Klöppel, G., La Rosa, S., & Rindi, G. (2019). Classification of neuroendocrine neoplasms of the digestive system. In WHO Classification of Tumours Editorial Board (Ed.), WHO classification of tumours. Digestive system tumours (5th ed., pp. 16–19). Lyon: IARC.

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  • Leoncini, E., Carioli, G., La Vecchia, C., Boccia, S., & Rindi, G. (2016). Risk factors for neuroendocrine neoplasms: A systematic review and meta-analysis. Annals of Oncology, 27, 68–81.

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  • Leoncini, E., Boffetta, P., Shafir, M., Aleksovska, K., Boccia, S., & Rindi, G. (2017). Increased incidence trend of low-grade and high-grade neuroendocrine neoplasms. Endocrine, 58, 368–379.

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  • Rindi, G., Klersy, C., Albarello, L., et al. (2018). Competitive testing of the WHO 2010 versus the WHO 2017 grading of pancreatic neuroendocrine neoplasms: Data from a large international cohort study. Neuroendocrinology, 107, 375–386.

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  • Scarpa, A., Chang, D. K., Nones, K., et al. (2017). Whole-genome landscape of pancreatic neuroendocrine tumours. Nature, 543, 65–71.

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  • Shi, C., & Klimstra, D. S. (2014). Pancreatic neuroendocrine tumors: Pathologic and molecular characteristics. Seminars in Diagnostic Pathology, 31, 498–511.

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  • Uccella, S., La Rosa, S., Volante, M., & Papotti, M. (2018). Immunohistochemical biomarkers of gastrointestinal, pancreatic, pulmonary, and thymic neuroendocrine neoplasms. Endocrine Pathology, 29, 150–168.

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Author information

Authors and Affiliations

  1. Institute of Pathology, University Hospital and University of Lausanne, Lausanne, Switzerland

    Stefano La Rosa

  2. Department of Histopathology, Central Institute, Valais Hospital, Sion, Switzerland

    Amedeo Sciarra

Authors
  1. Stefano La Rosa
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  2. Amedeo Sciarra
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Corresponding author

Correspondence to Stefano La Rosa .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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La Rosa, S., Sciarra, A. (2021). Well-Differentiated Neuroendocrine Tumors of the pancreas (PanNET Grade 1,2,3). In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5544-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5544-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

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