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Management of functional neuroendocrine tumors of the pancreas
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Endocrine Tumor Biology.ORCID iD: 0000-0002-3432-1182
2018 (English)In: Gland surgery, ISSN 2227-684X, E-ISSN 2227-8575, Vol. 7, no 1, p. 20-27Article, review/survey (Refereed) Published
Abstract [en]

Pancreatic neuroendocrine tumors (pNETs) constitute a heterogenous group of malignancies with varying clinical presentation, tumor biology and prognosis. The incidence of pNETs has steadily increased during the last decades with an estimated incidence 2012 of 4.8/100,000. Recent whole genome sequencing of pNETs has demonstrated mutations in the DNA repair genes MUTYH and point mutations and gene fusions in four main pathways from chromatin remodeling, DNA damage repair, activation of mechanistic target of rapamycin (mTOR) signaling and the telomere maintenance. This new information will be the foundation for new therapies in the near future for malignant pNETs. The functioning pNETs constitute about 30-40% of all pNETs displaying nine different clinical syndromes: insulinoma, Zollinger-Ellison, Verner-Morrison, glucagonoma, somatostatinomas, ectopic adrenocorticotropic hormone (ACTH) and parathyroid hormone related peptide (PTH-rP) syndromes. Single patients might also present carcinoid syndrome. The diagnostic work-up include histopathology with the new WHO 2017 Classification, biomarkers (CgA, NSE), radiology and molecular imaging including CT-scan, magnetic resonance imaging (MRI), ultrasound and PET-scan. A cornerstone in the treatment of pNETs is surgery which is rarely curative but can reduce the clinical symptoms by debulking which also include radiofrequency ablation, embolization of liver metastases. Medical treatment includes chemotherapy and the targeted agents such as everolimus, sunitinib and peptide receptor radiotherapy (PRRT). Somatostatin analogs has for the last decades been the main stay for management for clinical symptoms related to functioning pNETs and is often combined with new targeted agents as well as chemotherapy. Long-term management of functioning pNETs need a combination of different procedures, surgery, local ablation, targeted agents and somatostatin analogs. Future therapies might be based on the recent advances in molecular genetics and tumor biology.

Place, publisher, year, edition, pages
AME PUBL CO , 2018. Vol. 7, no 1, p. 20-27
Keywords [en]
Functioning pancreatic neuroendocrine tumors (FpNETs), positron emission tomography scan (PET scan), chemotherapy, targeted agents, everolimus, sunitinib, peptide receptor radiotherapy (PRRT), surgery
National Category
Endocrinology and Diabetes Cancer and Oncology
Identifiers
URN: urn:nbn:se:uu:diva-350109DOI: 10.21037/gs.2017.10.08ISI: 000423441800003PubMedID: 29629316OAI: oai:DiVA.org:uu-350109DiVA, id: diva2:1204321
Available from: 2018-05-07 Created: 2018-05-07 Last updated: 2018-05-07Bibliographically approved

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Öberg, Kjell

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