Malignant transformation of heterotopic pancreas is extremely rare. We describe FDG PET/CT findings in a case of pancreatic ductal adenocarcinoma arising from a heterotopic pancreas in the submucosa of the pylorus with peripyloric lymph node metastasis and elevated serum carbohydrate antigen 19-9 level. The heterotopic pancreatic adenocarcinoma showed intense FDG uptake, and the peripyloric lymph node metastasis showed mild FDG uptake. This case indicates that heterotopic pancreatic adenocarcinoma should be included in the differential diagnosis of FDG-avid gastric submucosal lesions, especially in patients with elevated serum carbohydrate antigen 19-9 level.
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