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Pancreatic Neuroendocrine Neoplasms

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The Spectrum of Neuroendocrine Neoplasia

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Abstract

Neuroendocrine neoplasms that arise from the neuroendocrine cells of the pancreas, the islets of Langerhans, include well-differentiated neuroendocrine tumors (NETs) that are clinically classified as functioning and nonfunctioning. Functioning tumors are associated syndromes due to excess insulin, glucagon, somatostatin, and/or pancreatic polypeptide, but they can also secrete gastrin, vasoactive intestinal peptide, or serotonin. Aggressive tumors can produce ectopic hormones such as ACTH, GHRH, GH, vasopressin, and/or parathyroid hormone-related peptide causing Cushing’s syndrome, acromegaly, SIAD, and hypercalcemia. Nonfunctioning tumors are more common and are usually diagnosed because of mass effects or the presence of metastases. The morphology of these tumors is highly variable, and they are graded based on their proliferative activity. They frequently arise in patients with hereditary predisposition syndromes. Poorly differentiated neuroendocrine carcinomas (NECs), subclassified as small cell or large cell variant, are high-grade malignancies that have a distinct biology and require a different therapeutic approach. Mixed neuroendocrine and non-neuroendocrine neoplasms (MiNENs) are composite lesions composed of different types of non-neuroendocrine neoplasms, including adenocarcinomas or squamous cell carcinomas, along with various grades of NET or with poorly differentiated NEC.

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Author information

Authors and Affiliations

  1. Department of Pathology, University Hospitals Cleveland Medical Center and Case Western Reserve University, Cleveland, OH, USA

    Sylvia L. Asa

  2. Department of Pathology, University Health Network, Toronto, ON, Canada

    Sylvia L. Asa

  3. Department of Pathology, Memorial Sloan Kettering Cancer Center, Department of Pathology, New York, NY, USA

    David S. Klimstra

Authors
  1. Sylvia L. Asa
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  2. David S. Klimstra
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Editors and Affiliations

  1. University Hospitals Cleveland Medical Center, Case Western Reserve University, Cleveland, OH, USA

    Sylvia L. Asa

  2. Institute of Pathology, University Hospital of Lausanne, Lausanne, Vaud, Switzerland

    Stefano La Rosa

  3. Department of Pathology, University Health Network, Toronto, ON, Canada

    Ozgur Mete

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Asa, S.L., Klimstra, D.S. (2021). Pancreatic Neuroendocrine Neoplasms. In: Asa, S.L., La Rosa, S., Mete, O. (eds) The Spectrum of Neuroendocrine Neoplasia. Springer, Cham. https://doi.org/10.1007/978-3-030-54391-4_11

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  • DOI: https://doi.org/10.1007/978-3-030-54391-4_11

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