Case Report
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World J Gastrointest Surg. Mar 27, 2014; 6(3): 42-46
Published online Mar 27, 2014. doi: 10.4240/wjgs.v6.i3.42
Giant mucinous cystic adenoma with pancreatic atrophy mimicking dorsal agenesis of the pancreas
Johan Gagnière, Aurélien Dupré, David Da Ines, Lucie Tixier, Denis Pezet, Emmanuel Buc
Johan Gagnière, Aurélien Dupré, Denis Pezet, Emmanuel Buc, Department of Digestive and Hepatobiliary Surgery, Centre Hospitalier Universitaire Estaing, 63003 Clermont-Ferrand, France
David Da Ines, Lucie Tixier, Department of Radiology, Centre Hospitalier Universitaire Estaing, 63003 Clermont-Ferrand, France
Author contributions: Gagnière J and Dupré A contributed equally to this work; Gagnière J, Dupré A and Buc E designed the research; Gagniere J, Dupré A, Da Ines D and Tixier L performed the research; Da Ines D and Tixier L supervised the iconography and Gagnière J, Dupré A, Pezet D and Buc E wrote the paper.
Correspondence to: Emmanuel Buc, PhD, Department of Digestive and Hepatobiliary Surgery, Centre Hospitalier Universitaire Estaing, place Lucie et Raymond Aubrac, 63003 Clermont-Ferrand, France. ebuc@chu-clermontferrand.fr
Telephone: +33-473-750494 Fax: + 33-473-750459
Received: December 1, 2013
Revised: January 15, 2014
Accepted: February 16, 2014
Published online: March 27, 2014
Abstract

Mucinous cystic adenoma (MCA) of the pancreas is a rare benign cystic tumor with ovarian-like stroma and lack of communication with the pancreatic ductal system. The ovarian tissue is incorporated from the left gonad within the dorsal pancreas during embryogenesis. Consequently, congenital dorsal agenesis of the pancreas (DAP) cannot be associated with MCA. We report the case of a giant MCA associated with atrophy of the dorsal pancreas mimicking complete DAP. Pancreato-magnetic resonance imaging failed to identify the dorsal pancreas but the absence of diabetes mellitus and compression of the splenic vein with major tributaries rectified the diagnosis of secondary atrophy of the distal pancreas. Unusual proximal location of the cyst in the pancreas may have induced chronic obstruction of both the dorsal pancreatic duct and the splenic vein, with secondary atrophy of the distal pancreas.

Keywords: Dorsal agenesis, Pancreas, Cystic tumor, Diabetes, Surgery

Core tip: Mucinous cystic adenoma (MCA) of the pancreas is a benign tumor with ovarian-like tissue located in the body or the tail of the pancreas. We report the first case of atrophy of the distal pancreas secondary to compression by a giant MCA. We raise the question of underlying dorsal agenesis of the pancreas (DAP) but as ovarian-like tissue of MCA comes from the close migration of the left gonad and the dorsal pancreas during embryogenesis MCA can not be associated with true DAP. Finally, the absence of diabetes mellitus, and thrombosis of the splenic vein confirmed the secondary atrophy caused by a mechanism of compression.