We present a rare case of adenocarcinoma arising from a heterotopic pancreas in the duodenum, and review the associated literature. A 62-year-old woman was admitted to our hospital, complaining of vomiting and epigastralgia. Imaging studies revealed advanced gastric cancer with a gastric outlet obstruction. Whipple's operation and resection of the regional lymph node were performed because of a direct invasion to the pancreas. Histopathologic examination of the resected specimen demonstrated the malignant transformation of a hetrotopic pancreas in the duodenum. At the 12-month follow-up, there was no recurrence of symptoms. The prognosis of adenocarcinoma arising from a heterotopic pancreas is not known. Further accumulation of cases and investigation of this entity are necessary.
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