담관의 신경내분비종양은 드물며, 그 중 대세포 신경내분 비종양은 매우 드물다. 저자들은 상복부의 둔통으로 내원하여 전산화 단층촬영에서 간문부 담관암, 내시경적 세포진 검사에서 분화도 나쁜 선암 소견으로 수술 시행 후 간문부 담관의 대세포 신경내분비암종 진단되었고, 종양의 절제 변연 침범으로 방사선 치료를 시행한 1예를 경험하여 문헌고찰과 함께 보고하는 바이다.

Neuroendocrine carcinoma of the bile duct is rare, and a large cell neuroendocrine carcinoma is extremely rare. We report the first case of a large cell neuroendocrine carcinoma in the hilar bile duct. A 73-year-old woman presented with continuous dull pain in the epigastrium. We diagnosed a Klatskin`s tumor, Bismuth type II by computed tomography. An initial endoscopic cytology of the common hepatic duct showed a poorly differentiated adenocarcinoma. She underwent an extended right hepatectomy. Histologically, the tumor was diagnosed as a large cell neuroendocrine carcinoma. She received adjuvant radiotherapy for tumor involvement of the resected margin. At 12 months after surgery, lymph node metastases were present in the retroperitoneum, but she was still alive at 23 months after surgery. The role of additional therapy, such as radiotherapy, must be investigated to improve patient prognoses. (Korean J Med 2011;80:S137-S141)