Abstract
A 74-year-old woman who visited a hospital because of high fever and was pointed out increased levels of biliary enzymes was referred to our hospital. Following close exploration, cancer of the papilla of Vater with lymph node metastasis was diagnosed, and pylorus preserving pancreaticoduodenectomy (D2) was performed. During surgery portal vein tumor thrombus via lymph node metastasis was identified. The portal vein was incised and the tumor thrombus was removed. The histopathological diagnosis was adenoendocrine cell carcinoma of the papilla of Vater, pT4 (pDu2, pPanc3), pN1, ly2, v3, and pn2. The portal vein tumor thrombus and the metastatic lymph nodes were mainly composed of endocrine cell carcinoma component. While she had been given adjuvant chemotherapy with UFT, multiple hepatic metastases, intraperitoneal lymph node metastasis, and portal vein tumor thrombus were found 5 months after the operation. Chemotherapy with CDDP + CPT-11 was started, but she died of exacerbation of the metastatic foci 7 months after the operation.
Adenoendocrine cell carcinoma of the papilla of Vater is extremely rare, and so we present this case together with a review of the Japanese literature.