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Case Reports in Gastrointestinal Medicine
Volume 2016, Article ID 3801962, 4 pages
Case Report

Pancreatic Neuroendocrine Tumor in the Setting of Dorsal Agenesis of the Pancreas

1Boston University School of Medicine, 72 East Concord St., Boston, MA 02118, USA
2Department of Pathology and Laboratory Medicine, Boston University School of Medicine, 72 East Concord St., Boston, MA 02118, USA
3Boston University School of Medicine, Moakley Building, 3rd Floor, 830 Harrison Avenue, Boston, MA 02118, USA

Received 4 July 2016; Revised 29 August 2016; Accepted 30 August 2016

Academic Editor: Haruhiko Sugimura

Copyright © 2016 Samih Nassif et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Dorsal agenesis of the pancreas (DAP) is an uncommon embryological abnormality where there is absence of the distal pancreas. DAP is mostly asymptomatic, but common presenting symptoms include diabetes mellitus, abdominal pain, pancreatitis, enlarged pancreatic head, and, in a few cases, polysplenia. MRCP and ERCP are the gold standard imaging techniques to demonstrate the absence of the dorsal pancreatic duct. The literature on the association of pancreatic neoplasia and DAP is limited. We present the case of a pancreatic neuroendocrine tumor in a patient with dorsal agenesis of the pancreas, with a review of the related literature.