Pancreatic Islet Cell Tumors

Ulrich Knigge, Mikkel Andreassen, Birgitte Federspiel, Carsten Palnæs Hansen, Andreas Kjær, Seppo W. Langer

Abstract

The incidence of pancreatic neuroendocrine neoplasms is more than 0.5/100.000. They are divided into Grade 1 (Ki67 < 2%), Grade 2 (Ki67 3%-20%), and Grade 3 (Ki67 > 20%). Tumors may be functioning (25%), that is, induce a hormone specific syndrome, or nonfunctioning (75%). Diagnosis is documented by histology, biochemistry, and somatostatin receptor imaging with CT (PET-CT). Surgery is the only treatment able to cure the patient. In patients with disseminated disease somatostatin analogs, interferon-alpha, everolimus or sunitinib is indicated in patients with a Ki67 < 20% and chemotherapy in patients with a Ki67 > 10%. Peptide receptor radionuclide therapy is an option in patients with disseminated disease and a Ki67 < 30%. Life-long follow-up is indicated in most patients.

Original languageEnglish
Title of host publicationEncyclopedia of Endocrine Diseases
EditorsIlpo Huhtaniemi, Luciano Martini
PublisherElsevier
Publication date1 Jan 2018
Edition2.
Pages626-634
ISBN (Print)9780128122006
DOIs
Publication statusPublished - 1 Jan 2018

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