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Ménétrier disease and gastrointestinal stromal tumors: hyperproliferative disorders of the stomach
Robert J. Coffey, … , Christopher L. Corless, Michael C. Heinrich
Robert J. Coffey, … , Christopher L. Corless, Michael C. Heinrich
Published January 2, 2007
Citation Information: J Clin Invest. 2007;117(1):70-80. https://doi.org/10.1172/JCI30491.
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Review Series

Ménétrier disease and gastrointestinal stromal tumors: hyperproliferative disorders of the stomach

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Abstract

Ménétrier disease and gastrointestinal stromal tumors (GISTs) are hyperproliferative disorders of the stomach caused by dysregulated receptor tyrosine kinases (RTKs). In Ménétrier disease, overexpression of TGF-α, a ligand for the RTK EGFR, results in selective expansion of surface mucous cells in the body and fundus of the stomach. In GISTs, somatic mutations of the genes encoding the RTK KIT (or PDGFRA in a minority of cases) result in constitutive kinase activity and neoplastic transformation of gut pacemaker cells (interstitial cells of Cajal). On the basis of the involvement of these RTKs in the pathogenesis of these disorders, Ménétrier disease patients have been effectively treated with a blocking monoclonal antibody specific for EGFR and GIST patients with KIT and PDGFRA tyrosine kinase inhibitors.

Authors

Robert J. Coffey, Mary Kay Washington, Christopher L. Corless, Michael C. Heinrich

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Figure 1

Anatomy of the human stomach.

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Anatomy of the human stomach.
The inset provides more detail of the laye...
The inset provides more detail of the layers of the stomach wall and indicates the location of the myenteric plexus of Auerbach. Interstitial cells of Cajal (ICCs), which are specialized, spindle-shaped cells located in the gut wall that function as pacemaker cells and help regulate slow-wave peristalsis of the gut, form a network around the myenteric plexus of Auerbach and serve to regulate neural input to the smooth muscle cells of the muscularis propria. The ICC cell bodies lie in close proximity to myenteric nerves, whereas their processes extend deeply into the inner and outer layers of the muscularis, contacting individual myocytes.

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ISSN: 0021-9738 (print), 1558-8238 (online)

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