Cronkhite-Canada syndrome (CCS) is known to have a malignant potential (1); this is the first report of adenocarcinoma arising from the major duodenal papilla of a patient with CCS. The patient was a 66-year-old Japanese man who had been suffering from cacogeusia, alopecia, onychodystrophy and cutaneous hyperpigmentation for 2 years was referred to our hospital. Esophagogastroduodenoscopy and colonoscopy showed numerous reddish polyps in the stomach, the duodenum including major duodenal papilla, and the colon (Picture 1). A biopsy specimen showed inflammation with edematous stroma and a hyperplastic foveolar epithelium. He was diagnosed to have CCS based on his physical and endoscopic findings. Although most of the gastroduodenal polyps disappeared after 6 months of treatment with prednisolone (initial dose, 60 mg/day; maintenance dose, 5 mg/day), the major duodenal papilla remained enlarged (Picture 2). Pylorus-preserving pancreatoduodenectomy was performed. A histological examination revealed that the resected tumor was well-differentiated adenocarcinoma in situ without stromal invasion, and showed that the dysplastic epithelial cells had spread into the bile duct (pT1N0M0) (Picture 3).

The authors state that they have no Conflict of Interest (COI).