2018 Volume 32 Issue 2 Pages 295-306
This case is of a 42-years-old man with a history of surgery for congenital biliary dilatation. Two years after surgery, a 25-mm mass was indicated at the intrapancreatic bile duct or residual cyst on computed tomography (CT) and magnetic resonance imaging (MRI). After confirming a diagnosis of residual bile duct or cyst carcinoma, we performed surgical resection. Pathological examination indicated the presence of neuroendocrine carcinoma (NEC) and tubular adenocarcinoma components in the mass, each occupying 70% and 30% of the mass, respectively. Based on these findings, the final diagnosis was MANEC at the residual cyst. The patient remains alive more than 5 months after diagnosis, without adjuvant therapy. According to previous reports, MANEC of the bile duct is a disease with poor prognosis. Moreover, carcinoma of the residual bile duct/cyst also exhibits poor prognosis. Therefore, surgical treatment for congenital biliary tract dilatation should not leave a cyst. This is the rare case report of MANEC with congenital biliary dilatation.
