A Case of Neuroendocrine Cell Carcinoma in the Hilar Bile Duct: Improved Outcome after Surgery and Chemotherapy

A 69-year-old man with elevated biliary liver enzyme levels, detected by blood tests, was referred to our hospital for medical scrutiny by the primary care physician. Scrutiny and biopsy revealed a tumor that was diagnosed as a neuroendocrine carcinoma. It was 15 mm in diameter and was located in the large hilar bile duct dominating the right hepatic duct. The tumor was judged as being unresectable, because infiltration into the bile duct of the left medial segment branch (B4) was evident. Chemotherapy was given in a total of 3 cycles, commencing every 28 days, CPT-11 was given at a dose of 80 mg/m2 (day 15) and cisplatin was given at a dose of 70 mg/m2 (days 1 and 15). Following treatment the tumor shrank to 9 mm in diameter and invasion of the left hepatic duct was no longer evident. Percutaneous transhepatic portal embolization prior to surgery; radical resection of the expanded right lobe, including excision of the caudate lobe, D2 dissection and choledochojejunostomy was performed. Primary neuroendocrine cell carcinoma in the hilar region is a rare disease, seldom reported in the literature.