Combined cholecystotomy, retrograde hydropulsion, and choledochal stenting to treat extrahepatic biliary tract obstruction in 3 cats.

Extrahepatic biliary tract obstruction (EHBO) is uncommonly encountered in cats. Surgical treatment aims to decompress the biliary tract and insure bile duct patency. In veterinary medicine, cholecystotomy is not widely used in practice. The objective was to describe the use of cholecystotomy, retrograde hydropulsion of choleliths, and choledochal stenting to remove choleliths from the extrahepatic biliary tract back in the gallbladder. Three adult domestic shorthair cats were presented with anorexia, lethargy, and vomiting. Serum biochemistry revealed hyperbilirubinemia and increased hepatic enzymes. Abdominal ultrasonography showed evidence of EHBO requiring surgical intervention. Choleliths were localized in the proximal and middle portions of the common bile duct (CBD) in the first case, in the distal portion of the CBD and within the major duodenal papilla in the second case, and in the middle and distal portions of the CBD in the third case. Cholecystotomy was followed by retrograde hydropulsion of the choleliths into the gallbladder, after which choledochal stenting was performed. Complications were defined as major when requiring additional medical or surgical treatment, or minor when not. Three major complications were reported. In 2 cases, severe anemia requiring blood transfusion occurred 24 h postoperatively; in 1 case, EHBO recurrence was encountered 41 d postoperatively. All cats were discharged within 4 d following surgery. Two cats were still alive at 12 and 14 mo after surgery, respectively. In the last case, owners refused revision surgery and the cat was euthanized. Key clinical message: Cholecystotomy combined with retrograde hydropulsion of choleliths permitted removal of choleliths and decompression of the biliary tract in 3 cats. Major complications included severe anemia and EHBO recurrence.

Cholécystotomie combinée, hydropulsion rétrograde et pose de stent cholédocien pour traiter l'obstruction des voies biliaires extra-hépatiques chez 3 chats. Les obstructions biliaires extra-hépatiques (OBEH) sont peu fréquentes chez le chat. Le traitement chirurgical vise à lever l'obstruction et s'assurer de la perméabilité des voies biliaires. En médecine vétérinaire, la cholécystotomie est une technique peu pratiquée. L'objectif de ce rapport de cas était de décrire l'utilisation de la cholécystotomie, de l'hydropulsion rétrograde des cholélithes et d'une prothèse endoluminale cholédoquale (PEC) pour repousser les cholélithes présents dans les voies biliaires extrahépatiques dans la vésicule biliaire (VB).Trois chats européens adultes ont été présentés pour anorexie, léthargie et vomissements. La biochimie sérique a révélé une hyperbilirubinémie et une augmentation des enzymes hépatiques. L'échographie abdominale a mis en évidence une OBEH nécessitant une intervention chirurgicale. Les cholélithes étaient situés dans la portion proximale et moyenne du canal cholédoque pour le premier cas; dans la portion distale et la papille duodénale majeure dans le second cas; dans la portion moyenne et distale pour le troisième cas. Une cholécystotomie a été suivie d'une rétro-hydropulsion des cholélithes dans la VB, puis une PEC a été placée. Les complications ont été définies comme majeures lorsqu'elles nécessitaient un traitement médical ou chirurgical supplémentaire, ou mineures lorsqu'elles n'en nécessitaient pas.Trois complications majeures ont été rapportées : chez 2 cas, une anémie sévère a été observée 24 h après l'intervention, nécessitant une transfusion sanguine; chez un cas, une récidive d'obstruction biliaire a eu lieu à 41 jours postopératoire. Tous les patients sont sortis de l'hôpital dans les 4 jours suivant l'opération. Deux cas étaient encore en vie 12 et 14 mois après l'intervention. Pour le dernier cas, la seconde chirurgie a été refusée par les propriétaires et le chat a été euthanasié.Message clinique clé :La cholécystotomie combinée à l'hydropulsion rétrograde des cholélithes a permis le retrait de cholélithes obstructives (dont certaines distales) et la décompression du tractus biliaire chez 3 chats. Les complications majeures incluaient une anémie sévère et une récidive d'obstruction biliaire.(Traduit par les auteurs).

[Double extrahepatic bile duct with pancreaticobiliary maljunction:a case report].

A 76-year-old woman with a suspected double extrahepatic bile duct was referred to our hospital. MRCP revealed that the left hepatic and posterior ducts combined to form the ventral bile duct and that the anterior duct formed the dorsal bile duct. ERCP demonstrated that the ventral bile duct was linked with the Wirsung duct. Amylase levels in the bile were unusually high. Based on these findings, we diagnosed a double extrahepatic bile duct with pancreaticobiliary maljunction and choledocholithiasis. Duplicate bile duct resection and bile duct jejunal anastomosis were performed considering the risk of biliary cancer due to pancreaticobiliary maljunction. The resected bile duct epithelium demonstrated no atypia or hyperplastic changes.

A case of spindle and giant cell-type undifferentiated carcinoma of the extrahepatic bile duct.

Spindle and giant cell type undifferentiated carcinoma of the extrahepatic bile duct is an uncommon malignancy. We report a case involving the common bile duct in a 72-year-old male with jaundice who was admitted to our hospital. Diagnostic imaging, including abdominal computed tomography and magnetic resonance imaging, revealed a mass in the distal common bile duct, accompanied by dilatation of both intra- and extrahepatic bile ducts and regional lymph node enlargement. Endoscopic retrograde cholangiography demonstrated stenosis in the distal common bile duct, with a biopsy confirming adenocarcinoma. The patient underwent endoscopic retrograde biliary drainage followed by a subtotal stomach-preserving pancreaticoduodenectomy with regional lymphadenectomy. Microscopic examination revealed that the tumor predominantly comprised spindle and giant atypical cells within the stroma. Immunohistochemical analysis showed the tumor cells expressing cytokeratins and mesenchymal markers, confirming the diagnosis of spindle and giant cell type undifferentiated carcinoma of the common bile duct. Ki-67 labeling index was observed to be above 80%. Postoperatively, intra-abdominal lymph node recurrence was noted at two months, and multiple liver metastases were identified at three months. The patient died seven months post-surgery. The literature pertaining to this rare disease is reviewed and discussed.

Osteosarcopenia is a significant predictor of recurrence and the prognosis after resection for extrahepatic bile duct cancer.

PURPOSE: This study examined the impact of osteosarcopenia on recurrence and the prognosis after resection for extrahepatic biliary tract cancer (EBTC). METHODS: We retrospectively analyzed 138 patients after resection for perihilar cholangiocarcinoma (11), distal cholangiocarcinoma (54), gallbladder carcinoma (30), or ampullary carcinoma (43). Osteosarcopenia is defined as the concomitant occurrence of osteopenia and sarcopenia. We investigated the relationship between osteosarcopenia and the overall survival (OS) and disease-free survival (DFS) in univariate and multivariate analyses. RESULTS: Osteosarcopenia was identified in 38 patients (27.5%) before propensity score (PS) matching. In the multivariate analysis, the independent recurrence factors were the prognostic nutrition index (p = 0.015), osteosarcopenia (p < 0.001), poorly differentiated adenocarcinoma (p = 0.004), perineural invasion (p = 0.002), and non-curability (p = 0.008), whereas the independent prognostic factors were prognostic nutrition index (p = 0.030), osteosarcopenia (p < 0.001), poorly differentiated adenocarcinoma (p = 0.007), lymphatic invasion (p = 0.018), and non-curability (p = 0.004). After PS matching, there was no significant difference in the variables between the patients with and without osteosarcopenia (n = 34 each). The 5-year DFS and OS after PS matching in patients with osteosarcopenia were significantly worse than in patients without osteosarcopenia (17.6% vs. 38.8%, p = 0.013 and 20.6% vs. 57.4%, p = 0.0005, respectively). CONCLUSIONS: Preoperative osteosarcopenia could predict the DFS and OS of patients after resection for EBTC.

Risk Factors for Distant Metastasis in Extrahepatic Bile Duct Cancer after Curative Resection (KROG 1814).

PURPOSE: Risk factors predicting distant metastasis (DM) in extrahepatic bile duct cancer (EHBDC) patients treated with curative resection were investigated. MATERIALS AND METHODS: Medical records of 1,418 EHBDC patients undergoing curative resection between Jan 2000 and Dec 2015 from 14 institutions were reviewed. After resection, 924 patients (67.6%) were surveilled without adjuvant therapy, 297 (21.7%) were treated with concurrent chemoradiotherapy (CCRT) and 148 (10.8%) with CCRT followed by chemotherapy. To exclude the treatment effect from innate confounders, patients not treated with adjuvant therapy were evaluated. RESULTS: After a median follow-up of 36.7 months (range, 2.7 to 213.2 months), the 5-year distant metastasis-free survival (DMFS) rate was 57.7%. On multivariate analysis, perihilar or diffuse tumor (hazard ratio [HR], 1.391; p=0.004), poorly differentiated histology (HR, 2.014; p < 0.001), presence of perineural invasion (HR, 1.768; p < 0.001), positive nodal metastasis (HR, 2.670; p < 0.001) and preoperative carbohydrate antigen (CA) 19-9 ≥ 37 U/mL (HR, 1.353; p < 0.001) were significantly associated with inferior DMFS. The DMFS rates significantly differed according to the number of these risk factors. For validation, patients who underwent adjuvant therapy were evaluated. In patients with ≥ 3 factors, additional chemotherapy after CCRT resulted in a superior DMFS compared with CCRT alone (5-year rate, 47.6% vs. 27.7%; p=0.001), but the benefit of additional chemotherapy was not observed in patients with 0-2 risk factors. CONCLUSION: Tumor location, histologic differentiation, perineural invasion, lymph node metastasis, and preoperative CA 19-9 level predicted DM risk in resected EHBDC. These risk factors might help identifying a subset of patients who could benefit from additional chemotherapy after resection.

Laparoscopic Resection of the Middle Bile Duct for Cholangiocarcinoma (with Video).

BACKGROUND: Surgical resection remains the sole approach to achieving long-term survival in cholangiocarcinoma cases. The universally recognised standard procedures for such cases include pancreaticoduodenectomy (PD) or hemihepatectomy accompanied by bile duct reconstruction. Nevertheless, some patients may still attain curative intent through bile duct segmental resection (BDR). However, these procedures are still in the experimental stage and should only be recommended for carefully chosen patients. METHODS: A 57-year-old male patient was admitted to our department after two weeks of escalating jaundice and abdominal discomfort. Upon admission, his total bilirubin was recorded at 102 µmol/L, and his direct bilirubin was 87 µmol/L. His carbohydrate antigen 19-9 (CA 19-9), carcinoembryonic antigen (CEA) and alpha fetoprotein (AFP) levels were normal. Enhanced computed tomography (CT) and magnetic resonance imaging (MRI) scans revealed a thickened and enhanced biliary tree extending from the cystic duct junction to the common hepatic duct no vascular invasion indicated by three-dimensional reconstruction. RESULTS: The patient underwent laparoscopic resection of the extrahepatic bile duct, accompanied by radical lymphadenectomy with skeletonisation and biliary reconstruction, was successfully conducted within 320 min, with a minimal blood loss of only 50 ml. The histological grading of the procedure was T2bN0M0 (stage II). The patient was discharged on the sixth postoperative day without complications. Following this, he underwent a regimen of single-agent capecitabine chemotherapy. After an 18-month follow-up period, no recurrence was observed. CONCLUSIONS: Our experience suggests that in selected patients diagnosed with middle bile duct cholangiocarcinoma, laparoscopic resection could potentially reach the standard of lymphadenectomy through skeletonisation.

Difficulty of adjuvant chemotherapy administration in patients with biliary tract cancer.

PURPOSE: This study aimed to elucidate the difficulty of adjuvant chemotherapy administration in patients with biliary tract carcinoma (BTC). METHODS: Clinical data of patients with BTC who underwent curative-intent surgery were retrospectively analyzed. The eligible patients were stratified into two groups according to the presence or absence of adjuvant chemotherapy administration (adjuvant and non-adjuvant groups), and the clinicopathological features were compared between the two groups. The ratios of adjuvant chemotherapy administration were investigated in each surgical procedure. Independent factors associated with no administration of adjuvant chemotherapy were analyzed using multivariate analyses. RESULTS: Among 168 eligible patients, 141 (83.9%) received adjuvant chemotherapy (adjuvant group), while 27 (16.1%) did not (non-adjuvant group). The most common surgical procedure was pancreaticoduodenectomy in the adjuvant group, and it was hepatectomy with extrahepatic bile duct resection (BDR) in the non-adjuvant group, respectively. The rate of no adjuvant chemotherapy was significantly higher in patients who underwent hepatectomy with BDR than in those who underwent other surgeries (p < 0.001). The most common cause of no adjuvant chemotherapy was bile leak in 12 patients, which occurred after hepatectomy with BDR in ten patients. Multivariate analyses revealed that hepatectomy with BDR and preoperative anemia were independently associated with no adjuvant chemotherapy (p < 0.001 and p < 0.001, respectively). CONCLUSIONS: Hepatectomy with BDR and subsequent refractory bile leak can be the obstacle to adjuvant chemotherapy administration in patients with BTC.

Clinical significance of extrahepatic bile duct resection for T2 gallbladder cancer using data from the Japanese Biliary Tract Cancer Registry between 2014 and 2018.

PURPOSE: The present study aimed to determine whether concomitant extrahepatic bile duct resection (EHBDR) improves the prognosis of patients with T2 gallbladder cancer (GBC). METHODS: Between 2014 and 2018, 4947 patients with GBC were registered in the National Biliary Tract Cancer Registry in Japan. This included 3804 patients (76.9%) who underwent curative-intent surgical resection; 1609 of these patients had pT2 GBC with no distant metastasis. Of the 1609 patients with GBC, 520 underwent EHBDR and 1089 did not. We compared the patients' backgrounds and disease-specific survival rates between the groups. RESULTS: The frequency of lymph node metastasis was significantly higher in the EHBDR group than in the non-EHBDR group (38.2% vs. 20.7%, p < .001). In the entire cohort, however, there was no significant difference in disease-specific survival between the two groups (76% vs. 79%, p = .410). The EHBDR group had a significantly higher incidence of postoperative complications (Clavien-Dindo classification grade = 3) (32.4% vs. 11.7%, p < .001). When we focused on the survival of only T2N1 patients who underwent gallbladder bed resection, the prognosis was significantly improved for the EHBDR group (5-year survival rate: 64% vs. 54%, p = .017). The non-EHBDR group was subcategorized into two groups: D2 dissection and D1 dissection or sampling, and survival curves were compared between these subgroups. Although the EHBDR group tended to have a favorable prognosis compared to the D2 group, this difference was not significant (p = .167). However, the EHBDR group had a significantly greater prognosis than the D1 dissection or sampling group (5 year-survival rate: 64 vs. 49%, p = .027). CONCLUSIONS: The EHBDR may improve the prognosis of patients with T2 gall bladder cancer with lymph node metastases; however, its indication should be carefully determined because of the increased risk of postoperative complications.

Impact of AI system on recognition for anatomical landmarks related to reducing bile duct injury during laparoscopic cholecystectomy.

BACKGROUND: According to the National Clinical Database of Japan, the incidence of bile duct injury (BDI) during laparoscopic cholecystectomy has hovered around 0.4% for the last 10 years and has not declined. On the other hand, it has been found that about 60% of BDI occurrences are due to misidentifying anatomical landmarks. However, the authors developed an artificial intelligence (AI) system that gave intraoperative data to recognize the extrahepatic bile duct (EHBD), cystic duct (CD), inferior border of liver S4 (S4), and Rouviere sulcus (RS). The purpose of this research was to evaluate how the AI system affects landmark identification. METHODS: We prepared a 20-s intraoperative video before the serosal incision of Calot's triangle dissection and created a short video with landmarks overwritten by AI. The landmarks were defined as landmark (LM)-EHBD, LM-CD, LM-RS, and LM-S4. Four beginners and four experts were recruited as subjects. After viewing a 20-s intraoperative video, subjects annotated the LM-EHBD and LM-CD. Then, a short video is shown with the AI overwriting landmark instructions; if there is a change in each perspective, the annotation is changed. The subjects answered a three-point scale questionnaire to clarify whether the AI teaching data advanced their confidence in verifying the LM-RS and LM-S4. Four external evaluation committee members investigated the clinical importance. RESULTS: In 43 of 160 (26.9%) images, the subjects transformed their annotations. Annotation changes were primarily observed in the gallbladder line of the LM-EHBD and LM-CD, and 70% of these shifts were considered safer changes. The AI-based teaching data encouraged both beginners and experts to affirm the LM-RS and LM-S4. CONCLUSION: The AI system provided significant awareness to beginners and experts and prompted them to identify anatomical landmarks linked to reducing BDI.

Detection of the communicating accessory bile duct in laparoscopic resection of residual gallbladder by the combination of the indocyanine green fluorescence cholangiography and the intraoperative cholangiography: A case report.

BACKGROUND: The partial cholecystectomy may be performed while in complicated laparoscopic cholecystectomy (LC). Biliary anomalies especially the accessory bile duct are established high risk of bile duct injury (BDI) in LC. Laparoscopic resection of residual gallbladder is a challenging procedure and extremely vulnerable to BDI. We report the execution of a laparoscopic resection of residual gallbladder with a communicating accessory bile duct using indocyanine green (ICG) fluorescence cholangiography and the intraoperative cholangiography (IOC). A case that has not been reported previously. PRESENTATION OF CASE: A 29-year-old female with history of laparoscopic partial cholecystectomy was admitted in our hospital. Magnetic resonance cholangiopancreatography (MRCP) revealed the residual gallbladder with an accessory bile duct. Considering the complexity of this patient, we performed a laparoscopic surgery using ICG fluorescence cholangiography. ICG was injected intravenously 1 h before the surgery, the residual gallbladder and the extrahepatic biliary structures including the accessory bile duct were imaged in green in fluorescence imaging that could be recognized clearly. IOC revealed that residual gallbladder communicated with intrahepatic bile duct through the accessory bile duct and drained into the common bile duct (CBD). The entire procedure was performed smoothly and successfully without bile duct injuries. DISCUSSION: Laparoscopic resection of residual gallbladder is a challenging procedure. Fluorescence cholangiography using ICG is regarded as a novel technique that could provide a real-time imaging intraoperative, which allowed to recognize and identify the residual gallbladder and the extrahepatic bile duct. IOC is also important in identifying a communicating accessory bile duct. Under the guidance of them, we completed this laparoscopic surgery. CONCLUSIONS: The combination of fluorescence cholangiography using ICG and IOC have profound significance in complicated LC.

Small-cell Neuroendocrine Carcinoma of the Extrahepatic Bile Duct: A Rare Case Report.

Neuroendocrine carcinoma (NEC) arising from the extrahepatic bile duct is extremely rare and commonly mistaken for cholangiocarcinoma. Therefore, NEC of the bile duct is difficult to diagnose preoperatively. Previously reported cases were resected with a diagnosis of cholangiocarcinoma and diagnosed with NEC after surgery. This paper reports an 84-year-old female with small-cell NEC of the extrahepatic bile duct, confirmed by a biopsy from an ERCP, with a review of the relevant literature. Contrast-enhanced abdomen computed tomography and magnetic resonance cholangiopancreatography revealed an approximately 1.7 cm enhancing intraductal mass in the proximal common bile duct with dilatation of the upstream bile duct. ERCP showed a long strictured segment in the proximal common bile duct with bile duct dilatation. A biopsy was performed at the site of the stricture. Histological examinations and hematoxylin-eosin staining showed the solid proliferation of small tumor cells with irregularly shaped hyperchromatic nuclei. Immunohistochemical examinations showed that the tumor cells were positive for CD56 and synaptophysin. Small-cell NEC of the extrahepatic bile duct was confirmed based on the histology and immunohistochemistry findings. The patient and their family denied treatment because of the patient's old age.

Mixed neuroendocrine-non-neuroendocrine neoplasm with mucinous adenocarcinoma and amphicrine carcinoma components in the bile duct: an autopsy case.

We report the first case of bile duct mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) that had a mucinous carcinoma component. An 88-year-old man with biliary obstruction was diagnosed as having distal bile duct cancer using imaging examinations and endoscopic biopsy. The patient received the best supportive care without surgical resection for 13 months until death. An autopsy revealed a bulky mass involving the distal bile duct and multiple metastases in intra-abdominal lymph nodes, the liver, and the lungs. The primary cancer was microscopically diagnosed as a MiNEN, which consisted of mucinous adenocarcinoma and large cell-type neuroendocrine carcinoma (NEC) components. Metastatic lesions in the liver and lungs were composed of only NEC with rich extracellular mucin without adenocarcinoma cells. Using electron microscopy and immunohistochemistry, it was proved that all NEC cells in both primary and metastatic lesions had amphicrine features. On the basis of pathological findings, we thought that the MiNEN was initially derived from a mucinous adenocarcinoma that dedifferentiated to amphicrine NEC cells with mucin production.

[Pre-surgical diagnosis of neuroendocrine carcinoma of the extrahepatic bile duct: a case study].

A 70-year-old woman presented to our hospital with jaundice. Abdominal ultrasonography showed biliary duct dilatation. Blood tests revealed elevated total bilirubin and hepatobiliary enzyme levels. A contrast-enhanced computed tomography of the abdomen showed bile duct thickening with wall enhancement. Transpapillary bile duct biopsy showed an invasive carcinoma proliferating in a follicular pattern. Pathology revealed positive synaptophysin and chromogranin A and a Ki67 index >40%, consistent with a diagnosis of neuroendocrine carcinoma (NEC). After confirming the absence of distant metastases, a subtotal stomach-preserving pancreaticoduodenectomy was performed. The result of the postoperative pathology was the same as the preoperative biopsy. According to previous reports, 7 out of 28 cases with NEC/mixed adenoneuroendocrine carcinoma could be diagnosed as NEC before surgery. However, biliary cytology and bile duct scraping cytology were used in many cases;only 11 cases underwent bile duct biopsy. For the latter, 5 out of 11 cases could be diagnosed preoperatively. NEC of the extrahepatic duct often exhibits a submucosal tumor-like morphology, which may result in a false negative result with biliary cytology or bile duct scraping cytology. In our case, the transpapillary bile duct biopsy sample was sufficient to diagnose NEC. This method could be an attractive option for the diagnosis of these tumors.

Endoscopic ultrasound-assisted rendezvous by transgastric approach in malignant extrahepatic bile duct stricture.

Endoscopic ultrasonography rendezvous (EUS-RV) is useful in cases of difficult deep biliary cannulation. However, in malignant extrahepatic bile duct strictures (MEHBDS), transduodenal routes are challenging and the transgastric approach often requires fistula dilation. We report a case of resectable MEHBDS in which transgastric puncture from the antrum of the stomach as EUS-RV was useful.

Risk factors for postoperative pneumonia after hepatectomy with extrahepatic bile duct resection.

BACKGROUND: The present study investigated the incidence of postoperative pneumonia (POP) after hepatectomy with extrahepatic bile duct resection (BDR) and elucidated risk factors for POP. METHODS: A total of 257 patients undergoing hepatectomy with BDR between 2011 and 2019 were retrospectively reviewed to evaluate the incidence of POP, defined by the Clavien-Dindo grade ≥ II. The predictive factors for POP were identified using logistic regression analysis of the pre- and intraoperative evaluable factors. RESULTS: Postoperative pneumonia developed in 15 patients (5.8%), diagnosed as grade IV or V in five patients. In a multivariate analysis, a vital capacity ratio (%VC) <80% (odds ratio [OR] 7.30, P = .005), age ≥ 74 years old (OR 5.39, P = .026), and future liver volume remnant (FLR) ratio ≤ 40% (OR 4.97, P = .045) were independent risk factors for POP. Among patients with performing spirometry, the incidence of developing POP was 1.0%, 8.8%, and 60% in patients with 0, 1-2, and 3 risk factors, respectively. CONCLUSIONS: The incidence of POP in patients undergoing hepatectomy with BDR was 5.8%. A %VC <80%, age ≥74 years old, and FLR ratio ≤40% were identified as preoperative predictive factors of developing POP.

[A Case of Neuroendocrine Carcinoma of the Extrahepatic Bile Duct].

A 77-year-old man visited a clinic because of nausea and chest discomfort. On blood test, hepatobiliary enzymes were elevated, and he referred to our hospital. Contrast-enhanced CT revealed stenosis of the extrahepatic bile duct and brush cytology of the bile duct showed adenocarcinoma. We therefore performed pancreatoduodenectomy for extrahepatic bile duct cancer. Pathological diagnosis was small cell neuroendocrine carcinoma, pT3N2M0, Stage ⅢA. The patient did not receive adjuvant chemotherapy and 3 months later contrast-enhanced CT and MRI showed multiple liver metastases. The patient was treated with cisplatin plus irinotecan in the first-line, cisplatin plus etoposide in the second-line, and amrubicin in the third-line and accordingly he died 1 year and 3 months after the surgery. Chemotherapy for neuroendocrine carcinoma of the bile duct is recommended as in small cell lung cancer, but the prognosis is extremely poor. We report this case with a review of some of the literature.