PMID- 32352164 OWN - NLM STAT- MEDLINE DCOM- 20210301 LR - 20210301 IS - 1365-2168 (Electronic) IS - 0007-1323 (Print) IS - 0007-1323 (Linking) VI - 107 IP - 11 DP - 2020 Oct TI - Surgery for multiple endocrine neoplasia type 1-related insulinoma: long-term outcomes in a large international cohort. PG - 1489-1499 LID - 10.1002/bjs.11632 [doi] AB - BACKGROUND: Insulinomas are found in 10-15 per cent of patients with multiple endocrine neoplasia type 1 (MEN1) and lead to life-threatening hypoglycaemia. Surgical outcome and the optimal surgical strategy for MEN1-related insulinoma are unknown. METHODS: Patients with MEN1-related insulinomas were identified in 46 centres in Europe and North America between 1990 and 2016. Insulinomas were considered localized if the lesion was in the pancreatic head or body/tail. Patients with pancreatic neuroendocrine tumours throughout the pancreas were suspected of having multifocal insulinoma. The primary outcome was postoperative hypoglycaemia, defined as persistent hypoglycaemia, or recurrent hypoglycaemia caused by a new insulinoma or insulin-producing liver metastases. Hypoglycaemia-free survival was estimated by the Kaplan-Meier method. RESULTS: Ninety-six patients underwent resection for MEN1-related insulinoma. Sixty-three and 33 patients had localized and multifocal insulinomas respectively. After a median follow-up of 8 (range 1-22) years, one patient (1 per cent) had persistent disease and six (6 per cent) had developed recurrent disease, of whom four had a new insulinoma. The 10-year hypoglycaemia-free survival rate was 91 (95 per cent c.i. 80 to 96) per cent. Of those with localized disease, 46 patients underwent pancreatic resection and 17 enucleation. One of these patients had persistent disease and one developed recurrent insulinoma. Among patients with multifocal disease, three developed new insulinomas and two developed insulin-producing liver metastases. CONCLUSION: Surgery for MEN1-related insulinoma is more successful than previously thought. CI - (c) 2020 The Authors. British Journal of Surgery published by John Wiley & Sons Ltd on behalf of BJS Society Ltd. FAU - van Beek, D J AU - van Beek DJ AUID- ORCID: 0000-0001-7834-8128 AD - Department of Endocrine Surgical Oncology, Utrecht, the Netherlands. FAU - Nell, S AU - Nell S AUID- ORCID: 0000-0001-7333-566X AD - Department of Endocrine Surgical Oncology, Utrecht, the Netherlands. AD - Department of Endocrine Oncology, Utrecht, the Netherlands. FAU - Verkooijen, H M AU - Verkooijen HM AUID- ORCID: 0000-0001-9480-1623 AD - Imaging Division, University Medical Centre Utrecht, Utrecht, the Netherlands. FAU - Borel Rinkes, I H M AU - Borel Rinkes IHM AUID- ORCID: 0000-0003-2122-7207 AD - Department of Endocrine Surgical Oncology, Utrecht, the Netherlands. FAU - Valk, G D AU - Valk GD AUID- ORCID: 0000-0001-5841-8344 AD - Department of Endocrine Oncology, Utrecht, the Netherlands. CN - (on behalf of the DutchMEN study group) FAU - Vriens, M R AU - Vriens MR AD - Department of Endocrine Surgical Oncology, Utrecht, the Netherlands. CN - International MEN1 Insulinoma Study Group LA - eng PT - Journal Article PT - Multicenter Study PT - Research Support, Non-U.S. Gov't DEP - 20200430 PL - England TA - Br J Surg JT - The British journal of surgery JID - 0372553 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Aged, 80 and over MH - Child MH - Child, Preschool MH - Clinical Decision-Making MH - Female MH - Follow-Up Studies MH - Humans MH - Hypoglycemia/epidemiology/etiology MH - Insulinoma/complications/*surgery MH - Kaplan-Meier Estimate MH - Male MH - Middle Aged MH - Multiple Endocrine Neoplasia Type 1/complications/*surgery MH - Neoplasm Recurrence, Local/epidemiology MH - *Pancreatectomy MH - Pancreatic Neoplasms/complications/*surgery MH - Postoperative Complications/epidemiology MH - Retrospective Studies MH - Treatment Outcome MH - Young Adult PMC - PMC7540387 FIR - Goudet, P IR - Goudet P FIR - Vella, A IR - Vella A FIR - Donegan, D IR - Donegan D FIR - Bartsch, D K IR - Bartsch DK FIR - Manoharan, J IR - Manoharan J FIR - Perrier, N D IR - Perrier ND FIR - Christakis, I IR - Christakis I FIR - Brandi, M L IR - Brandi ML FIR - Zarnegar, R IR - Zarnegar R FIR - Postma, E L IR - Postma EL FIR - Kebebew, E IR - Kebebew E FIR - Nockel, P IR - Nockel P FIR - Brunaud, L IR - Brunaud L FIR - Pasternak, J D IR - Pasternak JD FIR - Kluijfhout, W P IR - Kluijfhout WP FIR - Sturgeon, C IR - Sturgeon C FIR - Giri, S IR - Giri S FIR - Bonsing, B A IR - Bonsing BA FIR - van Eijck, C H IR - van Eijck CH FIR - van Goor, H IR - van Goor H FIR - de Kleine, R H J IR - de Kleine RHJ FIR - van Dijkum, E J Nieveen IR - van Dijkum EJN FIR - Dejong, C H C IR - Dejong CHC EDAT- 2020/05/01 06:00 MHDA- 2021/03/02 06:00 PMCR- 2020/10/07 CRDT- 2020/05/01 06:00 PHST- 2019/12/20 00:00 [received] PHST- 2020/02/09 00:00 [revised] PHST- 2020/03/19 00:00 [accepted] PHST- 2020/05/01 06:00 [pubmed] PHST- 2021/03/02 06:00 [medline] PHST- 2020/05/01 06:00 [entrez] PHST- 2020/10/07 00:00 [pmc-release] AID - BJS11632 [pii] AID - 10.1002/bjs.11632 [doi] PST - ppublish SO - Br J Surg. 2020 Oct;107(11):1489-1499. doi: 10.1002/bjs.11632. Epub 2020 Apr 30.