2017 Volume 50 Issue 1 Pages 43-51
A 71-year-woman was admitted to our hospital because of jaundice. Abdominal US and contrast-enhanced CT suggested a distal bile duct tumor. Blushing cytology of the bile duct revealed Group V, adenocarcinoma. Pancreaticoduodenectomy with regional lymph node dissection was performed. Pathological examination indicated the presence of both neuroendocrine carcinoma (NEC) and poorly-differentiated tubular adenocarcinoma components in the mass, which was finally diagnosed as mixed adenoneuroendocrine carcinoma (MANEC). CDDP and CPT-11 was started postoperatively according to the standard regimen for the NEC. Ten months after the operation, as CT depicted multiple liver metastases, chemotherapy was changed to gemcitabine and CDDP. The patient is still alive at 13 months after operation. According to previous reports, MANEC of the distal bile duct shows an enormously poor prognosis. Although the treatment algorithm of MANEC has not yet been established, the present case suggests that MANEC could be managed in accordance with the histology of the neuroendocrine component.