原创力文档diagnosis and treatment of gastrinomas in multiple endocrine neoplasia type 1 (men-1)胃泌素瘤的诊断和治疗多发性内分泌瘤1型(men-1).pdfVIP
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diagnosis and treatment of gastrinomas in multiple endocrine neoplasia type 1 (men-1)胃泌素瘤的诊断和治疗多发性内分泌瘤1型(men-1)
Cancers 2012, 4, 39-54; doi:10.3390/cancers4010039
OPEN ACCESS
cancers
ISSN 2072-6694
/journal/cancers
Review
Diagnosis and Treatment of Gastrinomas in Multiple Endocrine
Neoplasia Type 1 (MEN-1)
Ursula Plöckinger
Interdisziplinäres Stoffwechsel-Centrum: Endokrinologie, Diabetes und Metabolismus,
Kompetenzzentrum Seltene Stoffwechselkrankheiten, Charité-Universitätsmedizin Berlin,
Campus Virchow-Klinikum, Berlin 13353, Germany; E-Mail: ursula.ploeckinger@charite.de;
Tel.: +49-30-450-553-552; Fax: +49-30-450-553-950
Received: 1 December 2011; in revised form: 7 January 2012 / Accepted: 12 January 2012 /
Published: 20 January 2012
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; ga
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