Literature DB >> 7639241

A case of Peutz-Jeghers syndrome with nasal polyposis, extreme iron deficiency anemia, and hamartoma-adenoma transformation: management by combined surgical and endoscopic approach.

L De Facq1, J De Sutter, M De Man, P Van der Spek, L Lepoutre.   

Abstract

We report a rare variant of Peutz-Jeghers syndrome identified by the presence of nasal polyposis and extreme anemia. Multiple hamartomatous polyps were found throughout the upper and lower gastrointestinal tract. We conducted a combined surgical-endoscopic approach to prevent the development of a short-bowel syndrome. The polyps removed by snare and surgical polypectomy showed features typical of harmartomas. Three colonic polyps and two nasal polyps showed histological evidence of adenomatous change. This adenomatous change seems to fit into the pathogenic sequence of hamartoma-adenoma.

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Year:  1995        PMID: 7639241

Source DB:  PubMed          Journal:  Am J Gastroenterol        ISSN: 0002-9270            Impact factor:   10.864


  5 in total

1.  Total gastrointestinal endoscopy in the management of Peutz-Jeghers syndrome.

Authors:  N De Luca; Y Chia; D A Gorard
Journal:  Postgrad Med J       Date:  1998-12       Impact factor: 2.401

2.  Jan Peutz, Harold Jeghers and a remarkable combination of polyposis and pigmentation of the skin and mucous membranes.

Authors:  J J Keller; G J Offerhaus; F M Giardiello; F H Menko
Journal:  Fam Cancer       Date:  2001       Impact factor: 2.375

3.  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

4.  Cancer and Peutz-Jeghers Syndrome: A Review.

Authors:  J L Ivanovich; A J Whelan
Journal:  J Genet Couns       Date:  1997-06       Impact factor: 2.537

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

Authors:  Jy-Ming Chiang; Tse-Ching Chen
Journal:  Fam Cancer       Date:  2017-10       Impact factor: 2.375

  5 in total

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