Document Detail


The association of adrenocortical carcinoma and thyroid cancer in a child with Peutz-Jeghers syndrome.
MedLine Citation:
PMID:  21376213     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Peutz-Jeghers syndrome (PJS) is a rare, dominantly inherited disorder characterized by gastrointestinal hamartomatous polyps, mucocutaneous hyperpigmentation, and an increased risk of cancer. We present a 16-month-old child diagnosed with PJS, who had distinguishing features compared with the previously reported cases with respect to her clinical presentation, associated malignancies, and genetic analysis. To our knowledge, this is the first report of adrenocortical carcinoma in association with PJS, as well as the first instance of associated thyroid cancer in a child with PJS. We briefly review the relevant literature and highlight the recent progress achieved in the investigation of the syndrome.
Authors:
Sule Yalçin; Elif Kirli; Arbay O Ciftci; Ibrahim Karnak; Nicoletta Resta; Rosanna Bagnulo; Zuhal Akçören; Diclehan Orhan; Mehmet Emin Senocak
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  46     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-03-07     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  570-3     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 Elsevier Inc. All rights reserved.
Affiliation:
Department of Pediatric Surgery, Hacettepe University Faculty of Medicine, Ankara 06100, Turkey.
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