19 February 2026
: Case report 
Perihilar Biliary Neuroendocrine Carcinoma Treated With Perioperative Chemotherapy and Radical Liver Surgery: A Thought-Provoking Case
Challenging differential diagnosis, Unusual setting of medical care, Rare disease
Ryo Ataka
ABCDEF 1,2*, Kazuhiko Kitaguchi AE 1, Yusuke Nakayama AE 3, Yoshinobu Ikeno AE 4, Eiji Toyoda E 1, Tetsuro Hirose E 1
DOI: 10.12659/AJCR.951108
Am J Case Rep 2026; 27:e951108
Table 1 Neuroendocrine carcinoma of the proximal extrahepatic bile duct.
| Year [ref] | Country | Age | Sex | Histology | Treatment | Operation | Chemotherapy | Rec | Prognosis |
|---|---|---|---|---|---|---|---|---|---|
| 1993 []18 | Japan | 64 | M | MiNEN (SCNEC + Ad) | Resection | N/A | − | N/A | 8 mo, alive |
| 1998 []19 | Japan | 71 | F | MiNEN (SCNEC + Ad) | Resection | Segmentectomy + BDR | − | + | 7 mo, dead |
| 2005 []20 | USA | 54 | M | SCNEC | Resection | N/A | − | + | 6 mo, alive |
| 2009 []21 | France | 73 | M | MiNEN (LCNEC + Ad) | Resection → chemo | BDR | N/A (for rec) | + | 12 mo, dead |
| 2012 []22 | Japan | 70’s | F | LCNEC | Resection | N/A | − | N/A | N/A |
| 2013 []23 | USA | 76 | M | LCNEC | Resection | RH + BDR | − | + | 21 d, dead |
| 2014 []24 | USA | 65 | M | MiNEN (LCNEC + Ad) | Resection | BDR | − | + | 5 mo, dead |
| 2015 []25 | Japan | 70 | F | SCNEC | Resection → chemo (adj) | exLH + BDR | Irinotecan + carboplatin (adj) | − | 10 mo, alive |
| 2016 []26 | India | 76 | M | MiNEN (SCNEC + Ad) | Resection | BDR | − | N/A | N/A |
| 2016 []27 | Japan | 80 | M | MiNEN (LCNEC + Ad) | Resection | BDR | − | + | 3 mo, dead |
| 2016 []28 | Japan | 72 | M | LCNEC | PTPE → resection → chemo | RH + BDR | Etoposide + cisplatin (for rec) | + | 7 mo, alive |
| 2018 []29 | China | 62 | M | NEC | Resection | LH + BDR | − | + | 6 mo, dead |
| 2018 []30 | Korea | 58 | M | LCNEC | Resection → CRT (adj) | BDR | Etoposide + cisplatin (adj) | − | 10 mo, alive |
| 2019 []31 | China | 60 | M | MiNEN (LCNEC + Ad) | Resection | BDR | − | N/A | N/A |
| 2020 []32 | China | 65 | F | MiNEN (SCNEC + Ad) | Resection | BDR | − | − | 20 mo, alive |
| 2023 []33 | USA | 42 | F | NEC | Resection | BDR | FOLFOX6 (adj) | − | 4 mo, alive |
| 2025 Our Case | Japan | 74 | M | NEC | Chemo → resection → chemo | LH + BDR + RHAR | Etoposide + cisplatin (pre and adj) | + | 18 mo, dead |
| MiNEN – mixed neuroendocrine neoplasm; SCNEC – small-cell neuroendocrine carcinoma; LCNEC – large-cell neuroendocrine carcinoma; NEC – neuroendocrine carcinoma; Ad – adenocarcinoma; PTPE – percutaneous transhepatic portal embolization; CRT – chemoradiation therapy; BDR – bile duct resection; RH – right hepatectomy; LH – left hepatectomy; exLH – extended left hepatectomy; RHAR – right hepatic artery resection; adj – adjuvant therapy; pre – preoperative therapy; rec – recurrence. | |||||||||