Peutz-Jeghers syndrome

Lehur, Paul‐Antoine; Madarnas, P; Devroede, Ghislain; Perey, Bernard; Ménard, Daniel B.; Hamade, Nouhad

doi:10.1007/bf01317062

Cited by 34 publications

(1 citation statement)

References 13 publications

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“…The incidence of pancreatic cancer is about 100 times that expected and occurs at unusually early ages [67,69-73]. An increased frequency of breast cancer is also observed and is usually bilateral and ductal in origin [68,70,74-76]. Neoplasms of the reproductive tract such as adenoma malignum (a uterine cancer) have also been reported in Peutz-Jegher's patients.…”

Section: Syndromes With Preexisting Hamartomatous Polypsmentioning

confidence: 99%

Familial Adenomatous Polyposis (FAP) and Other Polyposis Syndromes

Scott

2003

Hered Cancer Clin Pract

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There have been significant advances in our knowledge about the molecular changes that precede and accompany the development of inherited predispositions to colorectal cancer. In this review the clinical relationship to the molecular changes associated with the polyposis syndromes is presented. The aim is to put into context the diverse findings that have been shown to be associated with the development of colorectal cancer in persons who harbor a predisposition to develop disease at unusually early ages. The main focus will be on familial adenomatous polyposis as it serves as a model disease and is the most extensively studied of all of the polyposis syndromes. In addition some information is provided that explains the relationship between a germline change in one gene and what consequences that can have for a particular cell and the development of disease.

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Section: Syndromes With Preexisting Hamartomatous Polypsmentioning

confidence: 99%

Familial Adenomatous Polyposis (FAP) and Other Polyposis Syndromes

Scott

2003

Hered Cancer Clin Pract

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Localization of Peutz-Jeghers Macules to Psoriatic Plaques

Banse-Kupin¹

1986

Arch Dermatol

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Adenocarcinoma of the small bowel in Lynch syndrome II

Lynch

Smyrk

Lynch

et al. 1989

Cancer

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Adenocarcinoma of the small bowel is rare and accounts for about 1% of all gastrointestinal tract cancer. This disorder has been identified in association with Crohn's disease, celiac disease, Peutz-Jegher's syndrome , and familial adenomatous polyposis. We report adenocarcinoma of the small bowel in nine patients from eight Lynch syndrome 11 extended pedigrees. Each affected patient was in the direct genetic lineage or manifested multiple primary cancers (stomach, colon, endometrium, and ovary) consonant with the tumor spectrum of Lynch syndrome 11. The average age of onset for small bowel cancer was 47 years (range 31 to 56 years), versus the general population peak occurrence after the sixth decade. We conclude that small bowel cancer may be an integral component of the tumor spectrum of Lynch syndrome 11.

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Peutz-Jeghers syndrome

Abstract: A female patient with Peutz-Jeghers syndrome successively developed bilateral breast carcinoma and malignant transformation of a duodenal hamartomatous polyp, from which she died.

Cited by 34 publications

References 13 publications

Familial Adenomatous Polyposis (FAP) and Other Polyposis Syndromes

Familial Adenomatous Polyposis (FAP) and Other Polyposis Syndromes

Localization of Peutz-Jeghers Macules to Psoriatic Plaques

Adenocarcinoma of the small bowel in Lynch syndrome II

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