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The diagnosis and treatment of gastrinoma and Zollinger-Ellison syndrome

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Book cover Diagnosis and Management of Endocrine-related Tumors

Part of the book series: Cancer Treatment and Research ((CTAR,volume 20))

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Abstract

Twenty-eight years ago, Robert Zollinger and Edwin Ellison described the syndrome which now bears their names [1]. The Zollinger-Ellison syndrome (ZES) consisted of fulminant peptic ulcer disease not relieved by the usual gastric surgical procedures, accompanied by severe hypersecretion of gastric acid and associated with the presence of nonspecific islet cell tumors in the pancreas. While not the first to note the association between ulcers and pancreatic endocrine tumors, they were the first to document the massive hypersecretion of gastric acid which linked the conditions and which remains the essential requirement for diagnosis. They suggested that a hormone released by the tumor caused the stomach to secrete acid. This inspired guess was followed within five years by extraction from the tumor of a ‘gastrin-like’ substance active on bioassay [2], and later by demonstration that the material was identical to gastrin in its chemical structure [3]. This led to the tumor being designated ‘gastrinoma,’ and to the short-lived belief that solitary gastrinomas, often benign, were the main cause of the syndrome. Within two years of its first description, the association of ZES with multiple endocrine neoplasia — type I (MEN-I) was recognized [4], though much remains unclear as to the precise nature of the association.

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  1. Denis M. McCarthy
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  1. Division of Endocrinology, The Milton S. Hershey Medical Center, The Pennsylvania State University, 17033, P.O. Box 850, Hershey, PA, USA

    Richard J. Santen M.D. (Professor of Medicine, Chief) & Andrea Manni M.D. (Associate Professor of Medicine) (Professor of Medicine, Chief) &  (Associate Professor of Medicine)

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McCarthy, D.M. (1984). The diagnosis and treatment of gastrinoma and Zollinger-Ellison syndrome. In: Santen, R.J., Manni, A. (eds) Diagnosis and Management of Endocrine-related Tumors. Cancer Treatment and Research, vol 20. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-2849-0_13

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