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.
Publication/Presentation Date
9-1-2011
Abstract
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.
Volume
15
Issue
9
First Page
1643
Last Page
1649
ISSN
1873-4626
Published In/Presented At
Rittenhouse, D. W., Kennedy, E. P., Mascaro, A. A., Brumbaugh, J. L., Stein, L. H., Rosenberger, L. H., Sauter, P. K., Yeo, C. J., & Lavu, H. (2011). 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. Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract, 15(9), 1643–1649. https://doi.org/10.1007/s11605-011-1542-6
Disciplines
Business Administration, Management, and Operations | Health and Medical Administration | Management Sciences and Quantitative Methods
PubMedID
21512846
Department(s)
Administration and Leadership
Document Type
Article