PMID- 25713781 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150225 LR - 20201001 IS - 2227-684X (Print) IS - 2227-8575 (Electronic) IS - 2227-684X (Linking) VI - 4 IP - 1 DP - 2015 Feb TI - Management of pancreatic neuroendocrine tumors in patients with MEN 1. PG - 63-8 LID - 10.3978/j.issn.2227-684X.2014.12.01 [doi] AB - Pancreatic neuroendocrine tumors (PNETs) are frequent and can be non-functional (NF) in patients with multiple endocrine neoplasia type 1 (MEN1). Their identification is of clinical importance because malignant PNETs are reported to be the most common cause of death in patients with MEN1. Once the diagnosis of MEN1 is established in an individual based on clinical manifestations and/or genetic testing results, an active surveillance program is instituted for early detection and treatment of MEN1-associated disease. Ultrasonography, endoscopic ultrasonography (EUS), CT, MRI, selective arterial angiography and somatostatin receptor scintigraphy are all used for localization of tumors. Managing PNETs can be challenging and includes diagnosis, surveillance, adequate staging, and interdisciplinary, multimodal treatments to optimize patient outcome. Treatment includes surgical resection for loco-regional disease, as well as liver directed and targeted chemotherapies for advanced progressive disease. To date, the recommendation for surgical resection in NF-PNETs is based on tumor size, as a higher rate of metastases was found in patients with larger tumors. This review summarizes key concepts in managing PNETs in patients with MEN1. FAU - Sadowski, Samira M AU - Sadowski SM AD - 1 Endocrine Oncology Branch, National Institutes of Health, Bethesda, MD 20892, USA ; 2 Thoracic and Endocrine Surgery, University Hospitals of Geneva, 1211 Geneva, Switzerland. FAU - Triponez, Frederic AU - Triponez F AD - 1 Endocrine Oncology Branch, National Institutes of Health, Bethesda, MD 20892, USA ; 2 Thoracic and Endocrine Surgery, University Hospitals of Geneva, 1211 Geneva, Switzerland. LA - eng PT - Journal Article PT - Review PL - China (Republic : 1949- ) TA - Gland Surg JT - Gland surgery JID - 101606638 PMC - PMC4321051 OTO - NOTNLM OT - Pancreatic neuroendocrine tumors (PNETs) OT - multiple endocrine neoplasia type 1 (MEN1) OT - surgical management EDAT- 2015/02/26 06:00 MHDA- 2015/02/26 06:01 PMCR- 2015/02/01 CRDT- 2015/02/26 06:00 PHST- 2014/11/11 00:00 [received] PHST- 2014/11/28 00:00 [accepted] PHST- 2015/02/26 06:00 [entrez] PHST- 2015/02/26 06:00 [pubmed] PHST- 2015/02/26 06:01 [medline] PHST- 2015/02/01 00:00 [pmc-release] AID - gs-04-01-063 [pii] AID - 10.3978/j.issn.2227-684X.2014.12.01 [doi] PST - ppublish SO - Gland Surg. 2015 Feb;4(1):63-8. doi: 10.3978/j.issn.2227-684X.2014.12.01.