title: 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.
abstract: BACKGROUND
  Dorsal agenesis of the pancreas (DAP) is a rare congenital anomaly, with only 44 cases having been reported in the English literature since 1966.
  
  
  METHODS
  A retrospective review of our IRB-approved pancreatic surgery database was performed from November 2005 to November 2010 searching for cases of DAP.
  
  
  CONCLUSIONS
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Surgery, Thomas Jefferson University, Jefferson Pancreas, Biliary and Related Cancer Center, Philadelphia, PA 19107, USA.
authors.forename: David W
authors.initials: DW
authors.lastname: Rittenhouse
authors.forename: Eugene P
authors.initials: EP
authors.lastname: Kennedy
authors.forename: Andres A
authors.initials: AA
authors.lastname: Mascaro
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Brumbaugh
authors.forename: Louis H
authors.initials: LH
authors.lastname: Stein
authors.forename: Laura H
authors.initials: LH
authors.lastname: Rosenberger
authors.forename: Patricia K
authors.initials: PK
authors.lastname: Sauter
authors.forename: Charles J
authors.initials: CJ
authors.lastname: Yeo
authors.forename: Harish
authors.initials: H
authors.lastname: Lavu
mesh_terms: D000230:Adenocarcinoma / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D000328:Adult; D000368:Aged; D000080924:Aldehyde Dehydrogenase 1 Family; D000818:Animals; D000013:Congenital Abnormalities / Q000175:diagnosis* / Q000235:genetics; D005260:Female; D053823:Hedgehog Proteins / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D010179:Pancreas / Q000002:abnormalities; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D050500:Pancreatitis, Chronic / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000601:surgery; D050697:Retinal Dehydrogenase / Q000235:genetics; D015398:Signal Transduction / Q000235:genetics
chemical_list: D053823:Hedgehog Proteins; D000080924:Aldehyde Dehydrogenase 1 Family; C545372:ALDH1A2 protein, human; D050697:Retinal Dehydrogenase