Literature DB >> 28391433

A Peutz-Jeghers syndrome family associated with sinonasal adenocarcinoma: 28 years follow up report.

Jy-Ming Chiang1,2, Tse-Ching Chen3,4.   

Abstract

Peutz-Jeghers syndrome (PJS) is a rare hereditary disorder characterized by hamartomatous polyps in both of the gastrointestinal tract and mucosal pigmentation. It could increase in risk of intestinal and extra-intestinal neoplasms. We here described three cases of sinonasal polyposis in a PJS family and two developed sinonasal type adenocarcinoma. Genetic study revealed a germline STK11/LKB1 mutation on codon 179 (c.C536G, p.P179R) of exon 4. LOH analysis of the LKB1 locus confirms this to be a deleterious mutation. Sinonasal polyposis with malignant transformation could be encountered in PJS patients. Regular follow-up was recommended for the risk of malignant changes in nasal polyps.

Entities:  

Keywords:  Nasal polyps; Peutz-Jeghers syndrome; STK11 mutation

Mesh：

Substances：

Year:  2017        PMID： 28391433     DOI： 10.1007/s10689-017-9983-z

Source DB:  PubMed          Journal:  Fam Cancer        ISSN： 1389-9600            Impact factor:   2.375


  14 in total

1.  Molecular genetic evidence of an association between nasal polyposis and the Peutz-Jeghers syndrome.

Authors:  Josbert J Keller; Anne Marie Westerman; Felix W M de Rooij; J H Paul Wilson; Herman van Dekken; Francis M Giardiello; Marian A J Weterman; G Johan A Offerhaus
Journal:  Ann Intern Med       Date:  2002-06-04       Impact factor: 25.391

2.  High cancer risk and increased mortality in patients with Peutz-Jeghers syndrome.

Authors:  Margot G F van Lier; Anne Marie Westerman; Anja Wagner; Caspar W N Looman; J H Paul Wilson; Felix W M de Rooij; Valery E P P Lemmens; Ernst J Kuipers; Elisabeth M H Mathus-Vliegen; Monique E van Leerdam
Journal:  Gut       Date:  2011-02       Impact factor: 23.059

Review 3.  Extraintestinal polyps in Peutz-Jeghers syndrome: presentation of four cases and review of the literature. Deutsche Peutz-Jeghers-Studiengruppe.

Authors:  T Vogel; V Schumacher; A Saleh; J Trojan; G Möslein
Journal:  Int J Colorectal Dis       Date:  2000-04       Impact factor: 2.571

4.  Peutz-Jeghers syndrome: 78-year follow-up of the original family.

Authors:  A M Westerman; M M Entius; E de Baar; P P Boor; R Koole; M L van Velthuysen; G J Offerhaus; D Lindhout; F W de Rooij; J H Wilson
Journal:  Lancet       Date:  1999-04-10       Impact factor: 79.321

5.  Nasal polyposis in Peutz-Jeghers syndrome: a distinct histopathological and molecular genetic entity.

Authors:  Wendy W J de Leng; Anne Marie Westerman; Marian A J Weterman; Marnix Jansen; Herman van Dekken; Francis M Giardiello; Felix W M de Rooij; J H Paul Wilson; G Johan A Offerhaus; Josbert J Keller
Journal:  J Clin Pathol       Date:  2006-06-14       Impact factor: 3.411

6.  Molecular and clinical characteristics in 46 families affected with Peutz-Jeghers syndrome.

Authors:  Hamid Mehenni; Nicoletta Resta; Ginevra Guanti; Louisa Mota-Vieira; Aaron Lerner; Mohammed Peyman; Kim A Chong; Larbi Aissa; Ali Ince; Angel Cosme; Michael C Costanza; Colette Rossier; Uppala Radhakrishna; Randall W Burt; Didier Picard
Journal:  Dig Dis Sci       Date:  2007-04-03       Impact factor: 3.199

7.  Bronchioloalveolar carcinoma: a new cancer in Peutz-Jeghers syndrome.

Authors:  A von Herbay; N Arens; W Friedl; I Vogt-Moykopf; K Kayser; K M Müller; W Back
Journal:  Lung Cancer       Date:  2005-02       Impact factor: 5.705

8.  [Rare manifestation of nasal polyposis in the Peutz-Jeghers syndrome].

Authors:  N Cerqua; L R D'Ottavi; V Perrotti; G Coen Tirelli; E Piccirillo; G Spaziani
Journal:  Acta Otorhinolaryngol Ital       Date:  1993 Jul-Aug       Impact factor: 2.124

9.  Hereditary gynecological tumors associated with Peutz-Jeghers syndrome (Review).

Authors:  Kouji Banno; Iori Kisu; Megumi Yanokura; Kenta Masuda; Arisa Ueki; Yusuke Kobayashi; Akira Hirasawa; Daisuke Aoki
Journal:  Oncol Lett       Date:  2013-08-16       Impact factor: 2.967

10.  Further observations on LKB1/STK11 status and cancer risk in Peutz-Jeghers syndrome.

Authors:  W Lim; N Hearle; B Shah; V Murday; S V Hodgson; A Lucassen; D Eccles; I Talbot; K Neale; A G Lim; J O'Donohue; A Donaldson; R C Macdonald; I D Young; M H Robinson; P W R Lee; B J Stoodley; I Tomlinson; D Alderson; A G Holbrook; S Vyas; E T Swarbrick; A A M Lewis; R K S Phillips; R S Houlston
Journal:  Br J Cancer       Date:  2003-07-21       Impact factor: 7.640
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