Document Detail

The novel triad of dorsal agenesis of the pancreas with concurrent pancreatic ductal adenocarcinoma and nonalcoholic chronic calcific pancreatitis: a case series and review of the literature.
MedLine Citation:
PMID:  21512846     Owner:  NLM     Status:  MEDLINE    
INTRODUCTION: Dorsal agenesis of the pancreas (DAP) is a rare congenital anomaly, with only 44 cases having been reported in the English literature since 1966.
MATERIALS AND METHODS: A retrospective review of our IRB-approved pancreatic surgery database was performed from November 2005 to November 2010 searching for cases of DAP.
DISCUSSION: Disorders in the retinoic acid (Raldh) and hedgehog (Hh) signaling pathways, which appear to play a role in the development of DAP, have been implicated in other diseases of the pancreas such as pancreatic ductal adenocarcinoma (PDA) and nonalcoholic chronic calcific pancreatitis (NCCP).
CONCLUSION: In this report, we describe three cases of DAP in the setting of PDA, two of which include the third component of NCCP. We provide a discussion of the clinical features of this novel triad and address the molecular pathways that relate to these respective diseases.
David W Rittenhouse; Eugene P Kennedy; Andres A Mascaro; Jennifer L Brumbaugh; Louis H Stein; Laura H Rosenberger; Patricia K Sauter; Charles J Yeo; Harish Lavu
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2011-04-22
Journal Detail:
Title:  Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract     Volume:  15     ISSN:  1873-4626     ISO Abbreviation:  J. Gastrointest. Surg.     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-08-23     Completed Date:  2012-01-23     Revised Date:  2014-09-22    
Medline Journal Info:
Nlm Unique ID:  9706084     Medline TA:  J Gastrointest Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1643-9     Citation Subset:  IM    
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MeSH Terms
Adenocarcinoma / complications*,  diagnosis,  genetics,  surgery
Congenital Abnormalities / diagnosis*,  genetics
Hedgehog Proteins / genetics
Middle Aged
Pancreas / abnormalities
Pancreatic Neoplasms / complications*,  diagnosis,  genetics,  surgery
Pancreatitis, Chronic / complications*,  diagnosis,  genetics,  surgery
Retinal Dehydrogenase / genetics
Signal Transduction / genetics
Grant Support
Reg. No./Substance:
0/Hedgehog Proteins; EC protein, human; EC Dehydrogenase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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