caa a22 4500 606248080 CHVBK 20210128101348.0 cr unu---uuuuu 210128e20151001xx s 000 0 eng 10.1007/s12262-015-1360-2 doi (NATIONALLICENCE)springer-10.1007/s12262-015-1360-2 Pancreatic Neuroendocrine Tumors: an Update [Elektronische Daten] [Alessandro Paniccia, Barish Edil, Richard Schulick] Pancreatic neuroendocrine tumors (pNETs) are rare and comprise only 1-2% of all pancreatic neoplastic disease. Although the majority of these tumors are sporadic (90%), pNETs can arise in the setting of several different hereditary genetic syndromes, most commonly multiple endocrine neoplasia type 1 (MEN1). The presentation of pNETs varies widely, with over 60% having malignant distant disease at the time of initial diagnosis involving the liver or other distant sites. Functioning pNETs represent approximately 10% of all pNETs, secrete a variety of peptide hormones, and are responsible for several clinical syndromes caused by profound hormonal derangement. Surgery remains the cornerstone of therapy and the only curative approach. It should be pursued for localized disease and for metastatic lesions amenable to resection. Multimodality therapies, including liver-directed therapies and medical therapy, are gaining increasing favor in the treatment of advanced pNETs. Their utility is multifold and spans from ameliorating symptoms of hormonal excess (functional pNETs) to controlling the local and systemic disease burden (non-functional pNETs). The recent introduction of target molecular therapy has promising results especially for the treatment of progressive well-differentiated G1/G2 tumor. In this review, we summarize the current knowledge and give an update on recent advancements made in the therapeutic strategies for pNETs. Association of Surgeons of India, 2015 Neuroendocrine tumor nationallicence pNET nationallicence Pancreatic cancer nationallicence Paniccia Alessandro Division of GI, Tumor and Endocrine Surgery, Department of Surgery, University of Colorado at Denver, Aurora, CO, USA aut Edil Barish Division of GI, Tumor and Endocrine Surgery, Department of Surgery, University of Colorado at Denver, Aurora, CO, USA aut Schulick Richard Division of GI, Tumor and Endocrine Surgery, Department of Surgery, University of Colorado at Denver, Aurora, CO, USA aut Indian Journal of Surgery Springer India 77/5(2015-10-01), 395-402 0972-2068 77:5<395 2015 77 12262 https://doi.org/10.1007/s12262-015-1360-2 text/html Onlinezugriff via DOI BK010053 XK010053 XK010000 Metadata rights reserved Springer special CC-BY-NC licence nationallicence 1 research-article jats NATIONALLICENCE NATIONALLICENCE NL-springer NATIONALLICENCE 856 40 https://doi.org/10.1007/s12262-015-1360-2 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Paniccia Alessandro Division of GI, Tumor and Endocrine Surgery, Department of Surgery, University of Colorado at Denver, Aurora, CO, USA aut NATIONALLICENCE 700 1- Edil Barish Division of GI, Tumor and Endocrine Surgery, Department of Surgery, University of Colorado at Denver, Aurora, CO, USA aut NATIONALLICENCE 700 1- Schulick Richard Division of GI, Tumor and Endocrine Surgery, Department of Surgery, University of Colorado at Denver, Aurora, CO, USA aut NATIONALLICENCE 773 0- Indian Journal of Surgery Springer India 77/5(2015-10-01), 395-402 0972-2068 77:5<395 2015 77 12262