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Cancers 2012, 4(1), 39-54; doi:10.3390/cancers4010039
Review

Diagnosis and Treatment of Gastrinomas in Multiple Endocrine Neoplasia Type 1 (MEN-1)

Interdisziplinäres Stoffwechsel-Centrum: Endokrinologie, Diabetes und Metabolismus, Kompetenzzentrum Seltene Stoffwechselkrankheiten, Charité-Universitätsmedizin Berlin, Campus Virchow-Klinikum, Berlin 13353, Germany
Received: 1 December 2011 / Revised: 7 January 2012 / Accepted: 12 January 2012 / Published: 20 January 2012
(This article belongs to the Special Issue Neuroendocrine Tumors)
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Abstract

Multiple endocrine neoplasia type 1 (MEN-1) is a rare autosomal-dominant disease. It is associated with a broad range of endocrine tumours, most frequently arising in the parathyroid glands, the pituitary and the pancreas. Most neuroendocrine tumours will be diagnosed in the pancreas as non-functioning neuroendocrine tumours or insulinomas. Forty-two percent of the patients will develop a gastrin-secreting neuroendocrine tumour, a gastrinoma. Gastrinomas in MEN-1 tend to be small, multiple and preferentially located in the duodenum. This paper will focus on the specific characteristics of gastrinomas in the setting of MEN-1 compared to sporadic gastrinomas. The developments in understanding the tumorigenesis of these tumours and the consequences for diagnosis and therapy will be discussed.
Keywords: MEN-1; gastrinoma; diagnosis; therapy MEN-1; gastrinoma; diagnosis; therapy
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).
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Plöckinger, U. Diagnosis and Treatment of Gastrinomas in Multiple Endocrine Neoplasia Type 1 (MEN-1). Cancers 2012, 4, 39-54.

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