PMID- 24363522 OWN - NLM STAT- MEDLINE DCOM- 20140612 LR - 20211021 IS - 2219-2840 (Electronic) IS - 1007-9327 (Print) IS - 1007-9327 (Linking) VI - 19 IP - 45 DP - 2013 Dec 7 TI - Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome. PG - 8312-20 LID - 10.3748/wjg.v19.i45.8312 [doi] AB - AIM: To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic. METHODS: Between January 1992 and June 2012, 28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1 (MEN1) syndrome underwent surgery at our institution. This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome (ZES). Surgical treatment consisted of duodenopancreatectomy (DP) or total pancreatectomy (TP). Regional lymphadenectomy was always performed. Any hepatic tumoral lesions found were removed during surgery. In MEN1 patients, removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia. One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors. This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree. RESULTS: Seventeen MEN1 patients affected with ZES were analyzed. The mean age was 40 years. Fifteen patients underwent DP and two TP. On histopathological examination, duodeno pancreatic endocrine tumors were found in all 17 patients. Eighty-one gastrinomas were detected in the first three portions of the duodenum. Only one gastrinoma was found in the pancreas. The mean number of gastrinomas per patient was 5 (range 1-16). Malignancy was established in 12 patients (70.5%) after lymph node, liver and omental metastases were found. Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s). In two cases, the ectopic gastrinoma was removed at the same time as pancreatic surgery, while in the third case, the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES. CONCLUSION: These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery. CI - (c) 2013 Baishideng Publishing Group Co., Limited. All rights reserved. FAU - Tonelli, Francesco AU - Tonelli F AD - Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy. FAU - Giudici, Francesco AU - Giudici F AD - Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy. FAU - Nesi, Gabriella AU - Nesi G AD - Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy. FAU - Batignani, Giacomo AU - Batignani G AD - Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy. FAU - Brandi, Maria Luisa AU - Brandi ML AD - Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy. LA - eng PT - Case Reports PT - Journal Article PT - Review PL - United States TA - World J Gastroenterol JT - World journal of gastroenterology JID - 100883448 SB - IM MH - Adult MH - Bile Duct Neoplasms/*pathology/surgery MH - Biopsy MH - Duodenal Neoplasms/*pathology/surgery MH - Female MH - Gastrinoma/*pathology/surgery MH - Humans MH - Lymph Node Excision MH - Male MH - Middle Aged MH - Multiple Endocrine Neoplasia Type 1/*pathology/surgery MH - Pancreatectomy MH - Pancreatic Neoplasms/*pathology/surgery MH - Pancreaticoduodenectomy MH - Retrospective Studies MH - Time Factors MH - Tomography, X-Ray Computed MH - Treatment Outcome MH - Young Adult MH - Zollinger-Ellison Syndrome/*pathology/surgery PMC - PMC3857454 OTO - NOTNLM OT - Biliary tree OT - Duodenopancreatectomy OT - Ectopic gastrinoma OT - Gastrinoma OT - Multiple endocrine neoplasia type 1 OT - Zollinger-Ellison Syndrome EDAT- 2013/12/24 06:00 MHDA- 2014/06/13 06:00 PMCR- 2013/12/07 CRDT- 2013/12/24 06:00 PHST- 2013/04/29 00:00 [received] PHST- 2013/06/29 00:00 [revised] PHST- 2013/07/04 00:00 [accepted] PHST- 2013/12/24 06:00 [entrez] PHST- 2013/12/24 06:00 [pubmed] PHST- 2014/06/13 06:00 [medline] PHST- 2013/12/07 00:00 [pmc-release] AID - 10.3748/wjg.v19.i45.8312 [doi] PST - ppublish SO - World J Gastroenterol. 2013 Dec 7;19(45):8312-20. doi: 10.3748/wjg.v19.i45.8312.