| Literature DB >> 18726167 |
Jason D Fraser1, Steven E Briggs, Shawn D St Peter, Giovanni De Petris, Jacques Heppell.
Abstract
Peutz-Jeghers syndrome is an uncommon genetic defect in the signal pathways of growth. The incidence has most recently been estimated to be in the range of 1 per 120,000 live births [1]. It is characterized by hamartomas throughout the gastrointestinal tract, mucocutaneous melanotic spots and increased predisposition to malignancy. The infrequent presentation of this syndrome in most practice combined with some less well-known diagnostic features may contribute to a misdiagnosis. Further, understanding of the genetic defect leading to the phenotypic syndrome and the future implications of this defect continue to evolve. Therefore we present a review in the setting of a case of misdiagnosed Peutz-Jeghers syndrome to portray illuminating features of the syndrome and review the literature.Entities:
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Year: 2008 PMID: 18726167 DOI: 10.1007/s10689-008-9212-x
Source DB: PubMed Journal: Fam Cancer ISSN: 1389-9600 Impact factor: 2.375