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

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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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 which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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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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