Mucosal prolapse in the pathogenesis of Peutz-Jeghers polyposis.

M. Jansen; W.W.J. de Leng; A.F. Baas; H. Myoshi; L. Mathus-Vliegen; M.M. Taketo; J.C. Clevers; F.M. Giardiello; G.J.A. Offerhaus

Mucosal prolapse in the pathogenesis of Peutz-Jeghers polyposis.

M. Jansen, W.W.J. de Leng, A.F. Baas, H. Myoshi, L. Mathus-Vliegen, M.M. Taketo, J.C. Clevers, F.M. Giardiello, G.J.A. Offerhaus

Hubrecht Institute

Onderzoeksoutput: Bijdrage aan wetenschappelijk tijdschrift/periodieke uitgave › Artikel › Wetenschappelijk › peer review

83 Citaten (Scopus)

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Germline mutations in LKB1 cause the rare cancer prone disorder Peutz-Jeghers syndrome (PJS). Gastrointestinal hamartomatous polyps constitute the major phenotypic trait in PJS. Hamartomatous polyps arising in PJS patients are generally considered to lack premalignant potential although rare neoplastic changes in these polyps and an increased gastrointestinal cancer risk in PJS are well documented. These conflicting observations are resolved in the current hypothesis by providing a unifying explanation for these contrasting features of PJS polyposis. We postulate that a genetic predisposition to epithelial prolapse underlies the formation of the polyps associated with PJS. Conventional sporadic adenomas arising in PJS patients will similarly show mucosal prolapse and carry the associated histological features.

Originele taal-2	Engels
Pagina's (van-tot)	1-5
Tijdschrift	Gut
Volume	55
Status	Gepubliceerd - 2006

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title = "Mucosal prolapse in the pathogenesis of Peutz-Jeghers polyposis.",

abstract = "Germline mutations in LKB1 cause the rare cancer prone disorder Peutz-Jeghers syndrome (PJS). Gastrointestinal hamartomatous polyps constitute the major phenotypic trait in PJS. Hamartomatous polyps arising in PJS patients are generally considered to lack premalignant potential although rare neoplastic changes in these polyps and an increased gastrointestinal cancer risk in PJS are well documented. These conflicting observations are resolved in the current hypothesis by providing a unifying explanation for these contrasting features of PJS polyposis. We postulate that a genetic predisposition to epithelial prolapse underlies the formation of the polyps associated with PJS. Conventional sporadic adenomas arising in PJS patients will similarly show mucosal prolapse and carry the associated histological features.",

author = "M. Jansen and Leng, {W.W.J. de} and A.F. Baas and H. Myoshi and L. Mathus-Vliegen and M.M. Taketo and J.C. Clevers and F.M. Giardiello and G.J.A. Offerhaus",

note = "doi: 10.1136/gut.2005.069062",

year = "2006",

language = "English",

volume = "55",

pages = "1--5",

journal = "Gut",

issn = "0017-5749",

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TY - JOUR

T1 - Mucosal prolapse in the pathogenesis of Peutz-Jeghers polyposis.

AU - Jansen, M.

AU - Leng, W.W.J. de

AU - Baas, A.F.

AU - Myoshi, H.

AU - Mathus-Vliegen, L.

AU - Taketo, M.M.

AU - Clevers, J.C.

AU - Giardiello, F.M.

AU - Offerhaus, G.J.A.

N1 - doi: 10.1136/gut.2005.069062

PY - 2006

Y1 - 2006

N2 - Germline mutations in LKB1 cause the rare cancer prone disorder Peutz-Jeghers syndrome (PJS). Gastrointestinal hamartomatous polyps constitute the major phenotypic trait in PJS. Hamartomatous polyps arising in PJS patients are generally considered to lack premalignant potential although rare neoplastic changes in these polyps and an increased gastrointestinal cancer risk in PJS are well documented. These conflicting observations are resolved in the current hypothesis by providing a unifying explanation for these contrasting features of PJS polyposis. We postulate that a genetic predisposition to epithelial prolapse underlies the formation of the polyps associated with PJS. Conventional sporadic adenomas arising in PJS patients will similarly show mucosal prolapse and carry the associated histological features.

AB - Germline mutations in LKB1 cause the rare cancer prone disorder Peutz-Jeghers syndrome (PJS). Gastrointestinal hamartomatous polyps constitute the major phenotypic trait in PJS. Hamartomatous polyps arising in PJS patients are generally considered to lack premalignant potential although rare neoplastic changes in these polyps and an increased gastrointestinal cancer risk in PJS are well documented. These conflicting observations are resolved in the current hypothesis by providing a unifying explanation for these contrasting features of PJS polyposis. We postulate that a genetic predisposition to epithelial prolapse underlies the formation of the polyps associated with PJS. Conventional sporadic adenomas arising in PJS patients will similarly show mucosal prolapse and carry the associated histological features.

M3 - Article

SN - 0017-5749

VL - 55

SP - 1

EP - 5

JO - Gut

JF - Gut

ER -

Mucosal prolapse in the pathogenesis of Peutz-Jeghers polyposis.

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