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 Article (Review)

Dorsal agenesis of the pancreas (DAP) is a rare congenital anomaly, with only 44 cases having been reported in the English literature since 1966.A retrospective review of our IRB-approved pancreatic surgery database was performed from November 2005 to November 2010 searching for cases of DAP.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).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.

Full Text

Duke Authors

Cited Authors

  • Rittenhouse, DW; Kennedy, EP; Mascaro, AA; Brumbaugh, JL; Stein, LH; Rosenberger, LH; Sauter, PK; Yeo, CJ; Lavu, H

Published Date

  • September 2011

Published In

Volume / Issue

  • 15 / 9

Start / End Page

  • 1643 - 1649

PubMed ID

  • 21512846

Pubmed Central ID

  • 21512846

Electronic International Standard Serial Number (EISSN)

  • 1873-4626

International Standard Serial Number (ISSN)

  • 1091-255X

Digital Object Identifier (DOI)

  • 10.1007/s11605-011-1542-6


  • eng