Description from OMIM
Prevalence of clinical parameters (%)
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List of references:
Peutz-Jeghers syndrome: data from the Singapore Polyposis Registry and a shifting paradigm in management.
Veronique Km Tan, Poh Koon Koh, Carol Tt Loi, Kong Weng Eu, Choong Leong Tang,
Peutz-Jeghers Syndrome (PJS) is an uncommon autosomal dominant hamartomatous polyposis syndrome. Morbidity arises from polyp-related complications and increased risks of malignancy. We report on PJS patients registered in the Singapore Polyposis Registry, identified principal causes of morbidity and appraised current management strategies. A followup protocol based on recent literature has been proposed.
Annals of the Academy of Medicine, Singapore - Jan 2010
Frequency and spectrum of cancers in the Peutz-Jeghers syndrome.
Nicholas Hearle, Valérie Schumacher, Fred H Menko, Sylviane Olschwang, Lisa A Boardman, Johan J P Gille, Josbert J Keller, Anne Marie Westerman, Rodney J Scott, Wendy Lim, Jill D Trimbath, Francis M Giardiello, Stephen B Gruber, G Johan A Offerhaus, Felix W M de Rooij, J H Paul Wilson, Anika Hansmann, Gabriela Möslein, Brigitte Royer-Pokora, Tilman Vogel, Robin K S Phillips, Allan D Spigelman, Richard S Houlston,
Although an increased cancer risk in Peutz-Jeghers syndrome is established, data on the spectrum of tumors associated with the disease and the influence of germ-line STK11/LKB1 (serine/threonine kinase) mutation status are limited.
Clinical cancer research : an official journal of the American Association for Cancer Research - May 2006