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.
J Gastrointest Surg
; 15(9): 1643-9, 2011 Sep.
Article
en En
| MEDLINE
| ID: mdl-21512846
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 ANDMETHODS:
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.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Neoplasias Pancreáticas
/
Anomalías Congénitas
/
Adenocarcinoma
/
Pancreatitis Crónica
Tipo de estudio:
Diagnostic_studies
Límite:
Adult
/
Aged
/
Animals
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Female
/
Humans
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Male
/
Middle aged
Idioma:
En
Revista:
J Gastrointest Surg
Asunto de la revista:
GASTROENTEROLOGIA
Año:
2011
Tipo del documento:
Article
País de afiliación:
Estados Unidos