Background: Cholangiocarcinoma is frequently observed in patients with congenital bile duct dilatation (CBDD). Most cholangiocarcinomas are adenocarcinomas. Other types, especially neuroendocrine carcinomas (NECs), are rare. To the best of our knowledge, this is the third reported case of an NEC of the common bile duct associated with CBDD and the first to receive adjuvant chemotherapy for advanced disease.
Case presentation: A 29-year-old woman presented with upper abdominal pain. Preoperative imaging indicated marked dilatation of the common bile duct and a tumor in the middle portion of the common bile duct. She was suspected of having distal cholangiocarcinoma associated with CBDD and underwent pylorus-preserving pancreaticoduodenectomy. Pathological and immunohistological findings led to a final diagnosis of large-cell NEC (pT3aN1M0 pStageIIB). The postoperative course was uneventful, and she was administered cisplatin and irinotecan every 4 weeks (four cycles) as adjuvant chemotherapy. She has remained recurrence-free for 16 months.
Conclusions: NEC might be a differential diagnosis in cases of cholangial tumor associated with congenital bile duct dilatation. This presentation is rare and valuable, and to establish better treatment for NEC, further reports are necessary.
Keywords: Adjuvant chemotherapy; Case report; Cholangiocarcinoma; Congenital bile duct dilatation; Large cell neuroendocrine carcinoma; Neuroendocrine carcinoma.