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

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.

Agenesis of the dorsal pancreas: systematic review of a clinical challenge

“…In mouse models, the development of PDA with a ductal phenotype has been confirmed. This is consistent with the clinical findings of Rittenhouse (5,50).…”

“…Overall 30 articles met the inclusion criteria and these reported on 53 patients; 7 children and 46 adults. The disorders associated with the 53 cases reported since 2008 are summarized in table I (5)(6)(7)(8)(9).…”

“…The association between cystic lesions and non-alcoholic chronic pancreatitis with pancreatic ductal adenocarcinoma (PDA) has recently been highlighted by Rittenhouse et al (5), in 3 patients who also presented dorsal pancreas agenesia. Other authors have reported an association with ampullary adenocarcinoma and intraductal papillary mucinous neoplasm (IPMN) (6,26).…”

“…The clinical presentation of DPA varies greatly ranging from incidental detection on X-ray, surgery or autopsy through to the development of a ductal adenocarcinoma of the pancreas (4,5). Diagnosis requires confirmation of the absence of the neck, body and tail of the pancreas and duct of Wirsung using endoscopic retrograde cholangiopancreatography (ERCP) or MRCP (Figs.…”

“…This anomaly was first described in 1911, and 53 cases were reported up to 2008 (3,4). Several authors have reported an association between DPA and acinar-ductal metaplasia (ADM), and neoplasia of the remnant pancreas (ventral pancreas) and diabetes (5)(6)(7)(8)(9).…”