PMID- 28257328 OWN - NLM STAT- MEDLINE DCOM- 20190809 LR - 20190809 IS - 1528-1140 (Electronic) IS - 0003-4932 (Linking) VI - 267 IP - 6 DP - 2018 Jun TI - Management of MEN1 Related Nonfunctioning Pancreatic NETs: A Shifting Paradigm: Results From the DutchMEN1 Study Group. PG - 1155-1160 LID - 10.1097/SLA.0000000000002183 [doi] AB - OBJECTIVE: To assess if surgery for Multiple Endocrine Neoplasia type 1 (MEN1) related nonfunctioning pancreatic neuroendocrine tumors (NF-pNETs) is effective for improving overall survival and preventing liver metastasis. BACKGROUND: MEN1 leads to multiple early-onset NF-pNETs. The evidence base for guiding the difficult decision who and when to operate is meager. METHODS: MEN1 patients diagnosed with NF-pNETs between 1990 and 2014 were selected from the DutchMEN1 Study Group database, including > 90% of the Dutch MEN1 population. The effect of surgery was estimated using time-dependent Cox analysis with propensity score restriction and adjustment. RESULTS: Of the 152 patients, 53 underwent surgery and 99 were managed by watchful waiting. In the surgery group, tumors were larger and faster-growing, patients were younger, more often male, and were more often treated in centers that operated more frequently. Surgery for NF-pNETs was not associated with a significantly lower risk of liver metastases or death, [adjusted hazard ratio (HR) = 0.73 (0.25-2.11)]. Adjusted HR's after stratification by tumor size were: NF-pNETs <2 cm = 2.04 (0.31-13.59) and NF-pNETs 2-3 cm = 1.38 (0.09-20.31). Five out of the 6 patients with NF-pNETs >3 cm managed by watchful waiting developed liver metastases or died compared with 6 out of the 16 patients who underwent surgery. CONCLUSIONS: MEN1 patients with NF-pNETs <2 cm can be managed by watchful waiting, hereby avoiding major surgery without loss of oncological safety. The beneficial effect of a surgery in NF-pNETs 2 to 3 cm requires further research. In patients with NF-pNETs >3 cm, watchful waiting seems not advisable. FAU - Nell, Sjoerd AU - Nell S AD - Department of Endocrine Surgical Oncology and Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Verkooijen, Helena M AU - Verkooijen HM AD - Imaging Division, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Pieterman, Carolina R C AU - Pieterman CRC AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - de Herder, Wouter W AU - de Herder WW AD - Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - Hermus, Ad R AU - Hermus AR AD - Department of Endocrinology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Dekkers, Olaf M AU - Dekkers OM AD - Departments of Endocrinology and Metabolism and Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands. FAU - van der Horst-Schrivers, Anouk N AU - van der Horst-Schrivers AN AD - Department of Endocrinology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. FAU - Drent, Madeleine L AU - Drent ML AD - Department of Internal Medicine, Section of Endocrinology, VU University Medical Center, Amsterdam, The Netherlands. FAU - Bisschop, Peter H AU - Bisschop PH AD - Department of Endocrinology and Metabolism, Academic Medical Center, Amsterdam, The Netherlands. FAU - Havekes, Bas AU - Havekes B AD - Department of Internal Medicine, Division of Endocrinology, Maastricht University Medical Center, Maastricht, The Netherlands. FAU - Borel Rinkes, Inne H M AU - Borel Rinkes IHM AD - Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Vriens, Menno R AU - Vriens MR AD - Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Valk, Gerlof D AU - Valk GD AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - Ann Surg JT - Annals of surgery JID - 0372354 RN - Non functioning pancreatic endocrine tumor MH - Adult MH - Female MH - Humans MH - Liver Neoplasms/prevention & control/secondary MH - Male MH - Multiple Endocrine Neoplasia Type 1/complications/pathology/*surgery MH - Pancreatic Neoplasms/complications/pathology/*surgery MH - Proportional Hazards Models MH - Watchful Waiting EDAT- 2017/03/04 06:00 MHDA- 2019/08/10 06:00 CRDT- 2017/03/04 06:00 PHST- 2017/03/04 06:00 [pubmed] PHST- 2019/08/10 06:00 [medline] PHST- 2017/03/04 06:00 [entrez] AID - 10.1097/SLA.0000000000002183 [doi] PST - ppublish SO - Ann Surg. 2018 Jun;267(6):1155-1160. doi: 10.1097/SLA.0000000000002183.