Abstract
A 60-year-old man was admitted to our hospital with a diagnosis of duodenal tumor was found, on endoscopic examination to have an ulcer with surrounding elevation in the second portion of the duodenum. Biopsy indicated adenocarcinoma. Diagnosing duodenal cancer, we conducted a pancreatoduodenectomy with regional lymph node dissection. The resected specimen showed a submucosal tumor-like lesion in the second portion of the duodenum. Histopathological and immnohistochemical examination revealed endocrine carcinoma, which has a dismal prognosis due to rapid metastasis. Only 14 cases of endocrine carcinoma of the duodenum have been reported in the Japanese literature, so this case was very rare.