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Published in: BMC Gastroenterology 1/2017

Open Access 01-12-2017 | Case report

A case of type 1 multiple endocrine neoplasia with esophageal stricture successfully treated with endoscopic balloon dilation and local steroid injection combined with surgical resection of gastrinomas

Authors: Hiroyuki Matsubayashi, Noboru Kawata, Naomi Kakushima, Masaki Tanaka, Kohei Takizawa, Yoshimi Kiyozumi, Yasue Horiuchi, Keiko Sasaki, Teiichi Sugiura, Katsuhiko Uesaka, Hiroyuki Ono

Published in: BMC Gastroenterology | Issue 1/2017

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Abstract

Background

In type 1 multiple endocrine neoplasia (MEN1), esophageal diseases association with excessive gastrin secretion in Zollinger-Ellison syndrome (ZES) sometimes develop. Here, we reported a case of MEN1/ZES, who developed dysphagia due to reflux esophagitis with severe esophageal stricture. Treatment for his esophageal stricture and ZES was discussed.

Case presentation

A 43-year-old man with progressive dysphagia and diarrhea was referred to the teaching hospital. He had a history of recurrent duodenojejunal perforations despite the anti-secretory medication. Blood examinations revealed elevated serum gastrin, calcium, and parathyroid hormone. Upper gastrointestinal endoscopy demonstrated a severe esophageal stricture, multiple gastroduodenal ulcer scars, and a duodenal submucosal tumor. Enhanced computed tomography showed multiple hypervascular tumors within the pancreas and duodenum, suggestive of MEN1. Genetic examination demonstrated a pathogenic MEN1 mutation. Repetitive endoscopic esophageal dilatation with intralesional corticosteroid injection, coupled with pancreatoduodenectomy were performed to improve the patient’s symptoms and to treat pancreatic tumors. The histology of multiple tumors in the duodenum and pancreas were all consistent with neuroendocrine tumors. His hypergastrinemia subsided and he remained asymptomatic in his gastrointestinal tract after these treatments.

Conclusion

For esophageal stenosis in case of MEN1/ZES, anti-secretory therapy and endoscopic dilatation with corticosteroid injection could be recommended. However, in refractory cases with repetitive and/or severe complications due to high acid secretion, surgical treatment could be considered as an option.
Literature
1.
go back to reference Giusti F, Marini F, Brandi ML. Multiple Endocrine Neoplasia Type 1. In: GeneReviews(R). edn. Edited by Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Fong CT, Mefford HC, Smith RJH et al. Seattle (WA); 1993. Bookshelf ID: NBK1538, PMID: 20301710. Giusti F, Marini F, Brandi ML. Multiple Endocrine Neoplasia Type 1. In: GeneReviews(R). edn. Edited by Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Fong CT, Mefford HC, Smith RJH et al. Seattle (WA); 1993. Bookshelf ID: NBK1538, PMID: 20301710.
2.
go back to reference Hoffmann KM, Gibril F, Entsuah LK, Serrano J, Jensen RT. Patients with multiple endocrine neoplasia type 1 with gastrinomas have an increased risk of severe esophageal disease including stricture and the premalignant condition, Barrett’s esophagus. J Clin Endocrinol Metab. 2006;91(1):204–12. doi:10.1210/jc.2005-1349.CrossRefPubMed Hoffmann KM, Gibril F, Entsuah LK, Serrano J, Jensen RT. Patients with multiple endocrine neoplasia type 1 with gastrinomas have an increased risk of severe esophageal disease including stricture and the premalignant condition, Barrett’s esophagus. J Clin Endocrinol Metab. 2006;91(1):204–12. doi:10.​1210/​jc.​2005-1349.CrossRefPubMed
4.
go back to reference Yamaguchi K, Urakami K, Ohshima K, Mochizuki T, Akiyama Y, Uesaka K, et al. Implementation of individualized medicine for cancer patients by multiomics-based analyses-the Project HOPE. Biomed Res. 2014;35(6):407–12. doi:10.2220/biomedres.35.407.CrossRefPubMed Yamaguchi K, Urakami K, Ohshima K, Mochizuki T, Akiyama Y, Uesaka K, et al. Implementation of individualized medicine for cancer patients by multiomics-based analyses-the Project HOPE. Biomed Res. 2014;35(6):407–12. doi:10.​2220/​biomedres.​35.​407.CrossRefPubMed
8.
go back to reference Hashimoto S, Kobayashi M, Takeuchi M, Sato Y, Narisawa R, Aoyagi Y. The efficacy of endoscopic triamcinolone injection for the prevention of esophageal stricture after endoscopic submucosal dissection. Gastrointest Endosc. 2011;74(6):1389–93. doi:10.1016/j.gie.2011.07.070.CrossRefPubMed Hashimoto S, Kobayashi M, Takeuchi M, Sato Y, Narisawa R, Aoyagi Y. The efficacy of endoscopic triamcinolone injection for the prevention of esophageal stricture after endoscopic submucosal dissection. Gastrointest Endosc. 2011;74(6):1389–93. doi:10.​1016/​j.​gie.​2011.​07.​070.CrossRefPubMed
9.
go back to reference Lew RJ, Kochman ML. A review of endoscopic methods of esophageal dilation. J Clin Gastroenterol. 2002;35(2):117–26.CrossRefPubMed Lew RJ, Kochman ML. A review of endoscopic methods of esophageal dilation. J Clin Gastroenterol. 2002;35(2):117–26.CrossRefPubMed
11.
go back to reference Ramage Jr JI, Rumalla A, Baron TH, Pochron NL, Zinsmeister AR, Murray JA, et al. A prospective, randomized, double-blind, placebo-controlled trial of endoscopic steroid injection therapy for recalcitrant esophageal peptic strictures. Am J Gastroenterol. 2005;100(11):2419–25. doi:10.1111/j.1572-0241.2005.00331.x.CrossRefPubMed Ramage Jr JI, Rumalla A, Baron TH, Pochron NL, Zinsmeister AR, Murray JA, et al. A prospective, randomized, double-blind, placebo-controlled trial of endoscopic steroid injection therapy for recalcitrant esophageal peptic strictures. Am J Gastroenterol. 2005;100(11):2419–25. doi:10.​1111/​j.​1572-0241.​2005.​00331.​x.CrossRefPubMed
12.
go back to reference Hernandez LV, Jacobson JW, Harris MS. Comparison among the perforation rates of Maloney, balloon, and savary dilation of esophageal strictures. Gastrointest Endosc. 2000;51(4 Pt 1):460–2.CrossRefPubMed Hernandez LV, Jacobson JW, Harris MS. Comparison among the perforation rates of Maloney, balloon, and savary dilation of esophageal strictures. Gastrointest Endosc. 2000;51(4 Pt 1):460–2.CrossRefPubMed
13.
go back to reference Silvis SE, Nebel O, Rogers G, Sugawa C, Mandelstam P. Endoscopic complications. Results of the 1974 American Society for Gastrointestinal Endoscopy Survey. JAMA. 1976;235(9):928–30.CrossRefPubMed Silvis SE, Nebel O, Rogers G, Sugawa C, Mandelstam P. Endoscopic complications. Results of the 1974 American Society for Gastrointestinal Endoscopy Survey. JAMA. 1976;235(9):928–30.CrossRefPubMed
14.
go back to reference Kishida Y, Kakushima N, Kawata N, Tanaka M, Takizawa K, Imai K, et al. Complications of endoscopic dilation for esophageal stenosis after endoscopic submucosal dissection of superficial esophageal cancer. Surg Endosc. 2015;29(10):2953–9. doi:10.1007/s00464-014-4028-2.CrossRefPubMed Kishida Y, Kakushima N, Kawata N, Tanaka M, Takizawa K, Imai K, et al. Complications of endoscopic dilation for esophageal stenosis after endoscopic submucosal dissection of superficial esophageal cancer. Surg Endosc. 2015;29(10):2953–9. doi:10.​1007/​s00464-014-4028-2.CrossRefPubMed
15.
go back to reference Falconi M, Eriksson B, Kaltsas G, Bartsch DK, Capdevila J, Caplin M, et al. ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors. Neuroendocrinology. 2016;103(2):153–71. doi:10.1159/000443171.CrossRefPubMedPubMedCentral Falconi M, Eriksson B, Kaltsas G, Bartsch DK, Capdevila J, Caplin M, et al. ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors. Neuroendocrinology. 2016;103(2):153–71. doi:10.​1159/​000443171.CrossRefPubMedPubMedCentral
16.
go back to reference Partelli S, Tamburrino D, Lopez C, Albers M, Milanetto AC, Pasquali C, Manzoni M, Toumpanakis C, Fusai G, Bartsch D, et al. Active Surveillance versus Surgery of Nonfunctioning Pancreatic Neuroendocrine Neoplasms </=2 cm in MEN1 Patients. Neuroendocrinology. 2016. doi:10.1159/000443613. Partelli S, Tamburrino D, Lopez C, Albers M, Milanetto AC, Pasquali C, Manzoni M, Toumpanakis C, Fusai G, Bartsch D, et al. Active Surveillance versus Surgery of Nonfunctioning Pancreatic Neuroendocrine Neoplasms </=2 cm in MEN1 Patients. Neuroendocrinology. 2016. doi:10.​1159/​000443613.
17.
go back to reference Sakurai A, Katai M, Yamashita K, Mori J, Fukushima Y, Hashizume K. Long-term follow-up of patients with multiple endocrine neoplasia type 1. Endocr J. 2007;54(2):295–302.CrossRefPubMed Sakurai A, Katai M, Yamashita K, Mori J, Fukushima Y, Hashizume K. Long-term follow-up of patients with multiple endocrine neoplasia type 1. Endocr J. 2007;54(2):295–302.CrossRefPubMed
18.
go back to reference Gibril F, Venzon DJ, Ojeaburu JV, Bashir S, Jensen RT. Prospective study of the natural history of gastrinoma in patients with MEN1: definition of an aggressive and a nonaggressive form. J Clin Endocrinol Metab. 2001;86(11):5282–93. doi:10.1210/jcem.86.11.8011.CrossRefPubMed Gibril F, Venzon DJ, Ojeaburu JV, Bashir S, Jensen RT. Prospective study of the natural history of gastrinoma in patients with MEN1: definition of an aggressive and a nonaggressive form. J Clin Endocrinol Metab. 2001;86(11):5282–93. doi:10.​1210/​jcem.​86.​11.​8011.CrossRefPubMed
Metadata
Title
A case of type 1 multiple endocrine neoplasia with esophageal stricture successfully treated with endoscopic balloon dilation and local steroid injection combined with surgical resection of gastrinomas
Authors
Hiroyuki Matsubayashi
Noboru Kawata
Naomi Kakushima
Masaki Tanaka
Kohei Takizawa
Yoshimi Kiyozumi
Yasue Horiuchi
Keiko Sasaki
Teiichi Sugiura
Katsuhiko Uesaka
Hiroyuki Ono
Publication date
01-12-2017
Publisher
BioMed Central
Published in
BMC Gastroenterology / Issue 1/2017
Electronic ISSN: 1471-230X
DOI
https://doi.org/10.1186/s12876-017-0597-6

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