Introduction : Agenesis of the dorsal pancreas (ADP) is very rare anomaly, and less than 70 cases have been reported worldwide. A few reports have been reported that the patients who had neoplasia in the pancreas with this anomaly. Here we report the first case of pancreatic ductal adenocarcinoma in a setting of agenesis of the dorsal pancreas, in Korea.

Methods : A 49-year-old female visited local hospital due to indigestion and jaundice. After internal bile duct drainage and biopsy, she was referred to my institution, Korea Cancer Center Hospital. Computed tomography demonstrated that body and tail part of the pancreas was absent, and that diffuse low attenuation mass was present at the head of pancreas. She underwent pancreaticoduodenectomy (total pancreatectomy, as a result) in department of Surgery. Pathologic report confirmed the patient’s diagnosis as ductal adenocarcinoma of pancreas, with lymph node metastases. She is under adjuvant treatment and DM control now.

Results : A few studies were reported that ADP was associated with the high frequency of occurrence of pancreatic neoplasia. ADP is mostly asymptomatic, but common presenting symptoms include diabetes mellitus, pancreatitis, etc. The diagnosis of ADP is mostly accidental finding, and is dependent on the image studies such as CT, ultrasound or MRI.

Conclusions : When ADP is diagnosed, careful follow-up is necessary for the surveillance of occurrence of pancreatic tumors.