Published online Mar 27, 2014. doi: 10.4240/wjgs.v6.i3.42
Revised: January 15, 2014
Accepted: February 16, 2014
Published online: March 27, 2014
Processing time: 108 Days and 0.1 Hours
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.
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.