PMID- 36436191 OWN - NLM STAT- MEDLINE DCOM- 20230418 LR - 20230418 IS - 1720-8386 (Electronic) IS - 0391-4097 (Print) IS - 0391-4097 (Linking) VI - 46 IP - 5 DP - 2023 May TI - Sporadic and MEN1-related gastrinoma and Zollinger-Ellison syndrome: differences in clinical characteristics and survival outcomes. PG - 957-965 LID - 10.1007/s40618-022-01961-w [doi] AB - PURPOSE: Gastrinoma with Zollinger-Ellison syndrome (ZES) may occur sporadically (Sp) or as part of the inherited syndrome of multiple endocrine neoplasia 1 (MEN-1). Data comparing Sp and MEN-1/ZES are scanty. We aimed to identify and compare their clinical features. METHODS: Consecutive patients with ZES were evaluated between 1992 and 2020 among a monocentric Italian patient cohort. RESULTS: Of 76 MEN-1 patients, 41 had gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN), 18 of whom had ZES; of 320 Sp-GEP-NEN, 19 had Sp-ZES. MEN-1/ZES patients were younger (p = 0.035) and the primary MEN-1/ZES gastrinoma was smaller than Sp-ZES (p = 0.030). Liver metastases occurred in both groups, but only Sp-ZES developed extrahepatic metastases. 13 Sp-ZES and 8 MEN-1/ZES underwent surgery. 8 Sp-ZES and 7 MEN-1/ZES received somatostatin analogs (SSAs). Median overall survival (OS) was higher in MEN-1/ZES than in Sp-ZES (310 vs 168 months, p = 0.034). At univariate-logistic regression, age at diagnosis (p = 0.01, OR = 1.1), G3 grading (p = 0.003, OR = 21.3), Sp-ZES (p = 0.02, OR = 0.3) and presence of extrahepatic metastases (p = 0.001, OR = 7.2) showed a significant association with OS. At multivariate-COX-analysis, none of the variables resulted significantly related to OS. At univariate-logistic regression, age (p = 0.04, OR = 1.0), size (p = 0.039, OR = 1.0), G3 grade (p = 0.008, OR = 14.6) and extrahepatic metastases (p = 0.005, OR = 4.6) were independently associated with progression-free survival (PFS). In multivariate-COX-analysis, only extrahepatic metastases (p = 0.05, OR = 3.4) showed a significant association with PFS. Among SSAs-treated patients, MEN-1/ZES showed better PFS (p = 0.0227). After surgery, the median PFS was 126 and 96 months in MEN-1 and Sp, respectively. CONCLUSION: MEN-1/ZES patients generally show better OS and PFS than Sp-ZES as well as better SSAs response. CI - (c) 2022. The Author(s). FAU - Massironi, S AU - Massironi S AUID- ORCID: 0000-0003-3214-8192 AD - Division of Gastroenterology and Center for Autoimmune Liver Diseases, San Gerardo Hospital, Via Pergolesi 3, Monza, Italy. sara.massironi@libero.it. AD - Department of Medicine and Surgery, European Reference Network on Hepatological Diseases (ERN RARE LIVER), San Gerardo Hospital, University of Milano-Bicocca, Monza, Italy. sara.massironi@libero.it. FAU - Rossi, R E AU - Rossi RE AD - Gastroenterology and Endoscopy Unit, IRCCS Humanitas Research Hospital, Via Manzoni 56, Rozzano, 20089, Milan, Italy. FAU - Laffusa, A AU - Laffusa A AD - Division of Gastroenterology and Center for Autoimmune Liver Diseases, San Gerardo Hospital, Via Pergolesi 3, Monza, Italy. AD - Department of Medicine and Surgery, European Reference Network on Hepatological Diseases (ERN RARE LIVER), San Gerardo Hospital, University of Milano-Bicocca, Monza, Italy. FAU - Eller-Vainicher, C AU - Eller-Vainicher C AD - Endocrinology, Fondazione IRCCS Ca' Granda Ospedale Policlinico di Milano, Milan, Italy. FAU - Cavalcoli, F AU - Cavalcoli F AD - Diagnostic and Therapeutic Endoscopy Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy. FAU - Zilli, A AU - Zilli A AD - Gastroenterology and Endoscopy, IRCCS Ospedale San Raffaele, Vita-Salute San Raffaele University, Milan, Italy. FAU - Ciafardini, C AU - Ciafardini C AD - Gastroenterology and Endoscopy, Fondazione IRCCS Ca' Granda Ospedale Policlinico di Milano, Milan, Italy. FAU - Sciola, V AU - Sciola V AD - Gastroenterology and Endoscopy, Fondazione IRCCS Ca' Granda Ospedale Policlinico di Milano, Milan, Italy. FAU - Invernizzi, P AU - Invernizzi P AD - Division of Gastroenterology and Center for Autoimmune Liver Diseases, San Gerardo Hospital, Via Pergolesi 3, Monza, Italy. AD - Department of Medicine and Surgery, European Reference Network on Hepatological Diseases (ERN RARE LIVER), San Gerardo Hospital, University of Milano-Bicocca, Monza, Italy. FAU - Peracchi, M AU - Peracchi M AD - Gastroenterology and Endoscopy, Fondazione IRCCS Ca' Granda Ospedale Policlinico di Milano, Milan, Italy. LA - eng PT - Journal Article DEP - 20221127 PL - Italy TA - J Endocrinol Invest JT - Journal of endocrinological investigation JID - 7806594 RN - 51110-01-1 (Somatostatin) SB - IM MH - Humans MH - *Zollinger-Ellison Syndrome/diagnosis/drug therapy/surgery MH - *Gastrinoma/pathology MH - *Multiple Endocrine Neoplasia Type 1/complications MH - *Neuroendocrine Tumors/complications MH - Somatostatin/therapeutic use MH - *Pancreatic Neoplasms/pathology PMC - PMC10105668 OTO - NOTNLM OT - Gastrinoma OT - MEN-1 OT - Multiple endocrine neoplasia type 1 OT - Neuroendocrine neoplasms OT - Neuroendocrine tumors OT - Zollinger-Ellison syndrome COIS- All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript. EDAT- 2022/11/28 06:00 MHDA- 2023/04/18 06:41 PMCR- 2022/11/27 CRDT- 2022/11/27 14:08 PHST- 2022/09/06 00:00 [received] PHST- 2022/11/04 00:00 [accepted] PHST- 2023/04/18 06:41 [medline] PHST- 2022/11/28 06:00 [pubmed] PHST- 2022/11/27 14:08 [entrez] PHST- 2022/11/27 00:00 [pmc-release] AID - 10.1007/s40618-022-01961-w [pii] AID - 1961 [pii] AID - 10.1007/s40618-022-01961-w [doi] PST - ppublish SO - J Endocrinol Invest. 2023 May;46(5):957-965. doi: 10.1007/s40618-022-01961-w. Epub 2022 Nov 27.