A case of adenoendocrine cell carcinoma of the papilla of vater

Abstract

A 64-year-old woman was admitted to the hospital with a diagnosis of obstructive jaundice. Abdominal ultrasonography, CT scan, upper gastrointestinal endoscopy, and ERCP provided the diagnosis of obstructive jaundice due to a tumorous lesion of the papilla of Vater. As a result of a biopsy, carcinoma of the papilla of Vater was diagnosed. After relief of obstructive jaundice by EBD, subtotal stomach-preserving pancreatoduodenectomy was performed. The histopathological diagnosis was adenoendocrine cell carcinoma.
A total of 10 cases of adenoendocrine cell carcinoma of the duodenum, including our case, have been reported in the Japanese literature. The disease has a high grade malignant potential and carries an extremely poor prognosis even curative resection followed by adjuvant chemotherapy is performed. In this case hepatic metastasis has already been confirmed at the time of operation. Oral administration of S-1 as postoperative adjuvant chemotherapy failed to suppress the gradual progress of the disease, and the patient died 14 months after the operation. We here present this case of adenoendocrine cell carcinoma of the papilla of Vater, together with a review of the Japanese literature.