A Resected Case of Endocrine Cell Carcinoma at the Papilla Vater

Abstract

Recent clinical studies have shown that endocrine cell carcinoma should be designated malignant carcinoid tumor to distinguish it from classical carcinoid tumor. We report a case of endocrine cell carcinoma of the papilla of Vater. A 66-year-old woman admitted for fever and abdominal pain was diagnosed with undifferentiated adenocarcinoma or endocrine cell carcinoma of the papilla of Vater, necessitating pylorus-preserving pancreaticoduodenectomy with lymph node dissection. The resected specimen showed a submucosal tumor with ulceration at the second portion of the duodenum. Histological and immunohistochemical examination showed endocrine cell carcinoma, The Ki-67 index was noted in 50% of the specimen. The postoperative course was very poor due to rapid metastasis. Endocrine cell carcinoma of the papilla of Vater is rare, with our case being the 18th to be reported in Japan.