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Case Reports
. 2013 Jan;7(1):1-6.
doi: 10.1159/000342355. Epub 2013 Jan 3.

An unusual presentation of zollinger-ellison syndrome

Emanuele Sinagra  1 , Giovanni Perricone, Cristina Linea, Luigi Montalbano, Stefania Plano, Rosa Giovanna Simonetti, Ambrogio Orlando, Claudia Romano, Georgios Amvrosiadis, Marco Messina, Andrea Scalisi, Maria Rosa Rizzuto, Aroldo Gabriele Rizzo, Mario Cottone
Affiliations
Case Reports

An unusual presentation of zollinger-ellison syndrome

Emanuele Sinagra et al. Case Rep Gastroenterol. 2013 Jan.

Abstract

Zollinger-Ellison syndrome is an often progressive, persistent and frequently life-threatening disease, described for the first time as characterized by ulceration of the upper jejunum, hypersecretion of gastric acid and non-beta islet cell tumors of the pancreas; this syndrome is due to the hypersecretion of gastrin. We report a case of Zollinger-Ellison syndrome presenting as severe esophagitis evolving in stenosis, which demonstrates how a delayed diagnosis may induce risk of disease spreading. In this setting new diagnostic approaches, such as somatostatin receptor scanning and positron emission tomography with 68 Ga-labeled octreotide, could be particularly useful, as well as further new therapeutic options, such as molecular targeted treatments and peptide receptor radionuclide therapy, though surgery is currently the only form of curative treatment, and the role of the therapeutic options mentioned needs to be clarified by forthcoming studies.

Keywords: Esophageal strictures; Octreoscan; Positron emission tomography; Zollinger-Ellison syndrome.

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Figures

Fig. 1
Fig. 1
Severe esophagitis with a tight lower esophageal stenosis.
Fig. 2
Fig. 2
Liver immunohistochemistry with cell positivity for gastrin (arrows), scale bar = 100 μm.
Fig. 3
Fig. 3
Liver immunohistochemistry with cell positivity for chromogranin (arrows), scale bar = 100 μm.
See this image and copyright information in PMC

References

    1. Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cells tumors of the pancreas. Ann Surg. 1955;142:709–723. - PMC - PubMed
    1. Isenberg JI, Walsh JH, Grossman MI. Zollinger-Ellison syndrome. Gastroenterology. 1973;65:140–165. - PubMed
    1. Chandrasekharappa SC, Guru SC, Manickam P, et al. Positional cloning of the gene for multiple endocrine neoplasia-type 1 (MEN1) Science. 1997;276:404–407. - PubMed
    1. Zhuang Z, Vortmeyer AO, Pack S, et al. Somatic mutations of the MEN1 tumor suppressor gene in sporadic gastrinomas and insulinomas. Cancer Res. 1997;57:4682–4686. - PubMed
    1. Wilkinson S, Teh BT, Davey KR, McArdle JP, Young M, Shepherd JJ. Cause of death in multiple endocrine neoplasia type 1. Arch Surg. 1993;128:683–690. - PubMed

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