National survey of hepatobiliary and pancreatic surgery in hemophilia patients in Japan.

BACKGROUND: Approximately 8300 hemophiliacs are registered in Japan, but no comprehensive reports on hepatobiliary and pancreatic surgery (HBPS) have been conducted. This report investigates the current status of HPBS in hemophilia patients in Japan. METHODS: The subjects were hemophiliac patients seen between January 1 2007, and December 31 2017, at facilities participating in this study among the facilities for performing high-difficulty cases nationwide designated by the Japanese Society for HBPS. A retrospective examination of short-term outcomes in 49 cases was conducted to assess patient background, disease, surgical procedure, and complications. RESULTS: The types of hemophilia were A: 43 cases, B: four cases, and von Willebrand disease: two cases (hemophilia severity: mild 32, moderate seven, severe 10). The target malignant diseases for surgery were hepatocellular carcinoma (HCC) in 20 cases, intrahepatic cholangiocellular carcinoma (CCC) in four cases, combined HCC-CCC in two cases, hilar CCC in two cases, and pancreatic cancer in four cases. As for the surgical procedure, limited resection (subsegmentectomy and partial hepatectomy) was performed in 16 cases of HCC even with normal liver function tests. Pancreaticoduodenectomy and distal pacreatectomy were performed for pancreatic cancers as in the standard procedure. Postoperative complications were postoperative bleeding in two cases after hepatectomy and one after pancreatectomy in one case. When compared with Japanese National Clinical Data base, the complication rates after hepatectomy and pancreatectomy were not conspicuous in hemophilic patients. CONCLUSIONS: As long as they are performed in qualified centers, complication rate is not increased in hemophilic patients undergoing HBPS.

Cytoglobin-expressing cells in the splenic cords contribute to splenic fibrosis in cirrhotic patients.

BACKGROUND AND AIM: Among several noninvasive evaluation methods of portal hypertension (PH), the measurement of spleen stiffness is a reliable method for predicting esophageal variceal bleeding; however, the underlying mechanisms for increased stiffness remain unclear. We attempted to elucidate the pathological changes to the spleen and the underlying mechanisms in patients with PH. METHODS: Histological examination was performed using splenic tissues from 42 patients with PH who underwent laparoscopic splenectomy, and the results were compared with those from patients without PH. RESULTS: In addition to splenic sinus congestion, diffuse fibrosis was detected in the splenic cords in the red pulp of patients with PH. The degree of the fibrosis was well correlated with severity in thrombocytopenia and splenomegaly. Cells expressing α-smooth muscle actin dramatically increased in the splenic cord. Cytoglobin (Cygb) expression was detected in human splenic cords as reported in animal reticular cells, and fluorescent double immunostaining revealed that these cells expressed α-smooth muscle actin in patients with PH, suggesting transformation of Cygb-expressing cells to myofibroblastic cells. Expression levels of nicotinamide adenine dinucleotide phosphate oxidase (NOX) 2, nitrotyrosine, and transforming growth factor-ß were markedly upregulated in the red pulp of patients with PH, implying a significant role of oxidative stress in the mechanism for splenic fibrosis. CONCLUSION: Splenic fibrosis progresses along with advancement of PH. Cygb-expressing cells in the splenic cord possibly participate in this process through mechanisms including oxidative stress.

Intraoperative Ultrasound Elastography Is Useful for Determining the Pancreatic Texture and Predicting Pancreatic Fistula After Pancreaticoduodenectomy.

OBJECTIVES: Pancreatic fistula (PF) is one of the most common complications after pancreaticoduodenectomy (PD). The soft pancreatic texture is known to be an important predictive factor for PF after PD. However, its evaluation is dependent on the sense of touch by the operator during operation, thus not objective. The aim of this study was to investigate the relationship between mean elasticity via intraoperative ultrasound elastography and histological pancreatic hardness, as well as predictive factor of PF after PD. METHODS: Forty-eight patients who underwent ultrasound elastography during PD and had pancreatic parenchyma histologically evaluated were included. RESULTS: Pancreatic fistula was noted in 20 patients. There were significant differences in the histological pancreatic fibrosis rate between soft pancreas group (8.2%) and hard pancreas group (28.4%, P < 0.05) and in the mean elasticity between soft pancreas group (1.94 m/s) and hard pancreas group (3.17 m/s, P < 0.05). The mean elasticity was significantly correlated with pancreatic fibrosis rate (P < 0.05). A multivariate analysis revealed that the mean elasticity of less than 2.2 m/s was a significant predictor of PF after PD (P = 0.003). CONCLUSIONS: Intraoperative ultrasound elastography could predict pancreatic texture objectively. The mean elasticity of less than 2.2 m/s was a significant predictor of PF after PD.

Immunohistochemical characterization of cancer-associated fibroblasts at the primary sites and in the metastatic lymph nodes of human intrahepatic cholangiocarcinoma.

Cancer-associated fibroblasts (CAFs) are an important constituent of the cancer stroma. In intrahepatic cholangiocarcinoma (ICC), the features of CAFs at the primary site and in the metastatic lymph nodes (Met-LNs) and their origin have been unclear. In the present study, we characterized CAFs at the primary site (n = 42) and in the Met-LNs (n = 10) of human ICC by immunohistochemistry using potential molecular markers of CAFs, portal fibroblasts (PFs), hepatic stellate cells (HSCs), and bone marrow-derived fibrocytes (BMDFs). At the primary site, the stroma was strongly positive for α-smooth muscle actin (α-SMA; marker for CAFs), platelet-derived growth factor receptor-ß (PDGFR-ß) (common marker for HSCs and PFs), fibulin-2, and thymus cell antigen-1 (Thy-1; PF marker), whereas immunoreactivity for fascin (HSC marker) was scarce. Most of the α-SMA-positive cells were found to express PDGFR-ß, Thy-1, and fibulin-2 by double immunostaining. A small population of BMDF marker-positive (α-SMA+CD45+CD34+) cells was found by triple immunostaining. In the micro-Met-LNs, α-SMA-positive cells were absent in cancer aggregates of the LN sinus, whereas they were present in the invasion area of cancer cells from the LN sinus to the LN parenchyma. In the macro-Met-LNs, there were abundant α-SMA-positive cells that were also positive for PDGFR-ß and Thy-1 but negative for fibulin-2 and fascin. Thus, regarding the expression of molecular markers, CAFs at the primary site of ICC are similar to PFs and different from those of HSCs or CAFs in the Met-LNs. CAFs at the primary sites and in the Met-LN are thought to be derived from PFs/BMDFs and resident cells of LNs, respectively.

Difficulty Achieving a Preoperative Diagnosis of IgG4-Related Sclerosing Cholangitis.

A 75-year-old male was admitted to our hospital because of bile duct stenosis. He had no medical history of autoimmune disease. The level of tumor markers, serum IgG, and IgG4 were within normal ranges. Computed tomography showed perihilar and distal bile duct stenosis and wall thickening without swelling or abnormal enhancement of the pancreas. Endoscopic retrograde cholangiopancreatography showed perihilar and distal bile duct stenosis. A biopsy and cytology from the distal bile duct stenosis suggested adenocarcinoma, and cytology from the perihilar bile duct also suggested adenocarcinoma. A preoperative diagnosis of perihilar and distal bile duct cancer was made, and the patient underwent left hepatectomy and pancreaticoduodenectomy. Resected specimens showed wall thickening in the perihilar and distal bile duct; however, tumors were unclear. A histopathological examination revealed lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis in the perihilar and distal bile ducts. Immunohistochemistry revealed diffuse infiltration of IgG4-positive plasma cells in the perihilar and distal bile ducts. Lymphoplasmacytic infiltration, inflammatory change, storiform fibrosis, and obliterative phlebitis were shown in the pancreas. A final diagnosis of IgG4-related sclerosing cholangitis (IgG4-SC) with autoimmune pancreatitis was made. We herein report a case in which a preoperative diagnosis of IgG4-SC was difficult due to normal serum IgG4 levels and no obvious pancreatic lesion.

Is Noncurative Hepatic Resection Justified for Advanced Hepatocellular Carcinoma?

It has been obscure whether or not noncurative hepatic resection (Hx) has a favorable impact on the clinical course in patients with advanced hepatocellular carcinoma (HCC). The aim of this study is to clarify the significance of noncurative Hx for advanced HCC. Among 666 consecutive patients undergoing Hx for HCC in our department, 79 patients underwent noncurative Hx. These patients were classified as Group A (presence of macrovascular invasion [MVI]; n = 29), Group B (residual tumors in the remnant liver; n = 37), Group C (residual tumors in the remnant liver with MVI; n = 7), or Group D (residual tumors in the remnant liver with distant metastasis [with or without MVI]; n = 6). The three-year survival rates were 49.6 per cent in Group A, 30.3 per cent in Group B, 14.3 per cent in Group C, and 0.0 per cent in Group D, respectively (Groups A and B vs Group D, P < 0.05). Moreover, the survival rate was significantly higher in patients with ≤3 tumors than in those with ≥4 tumors (P < 0.05), when Group B was divided into subgroups according to the number of residual tumors in the remnant liver. In conclusion, noncurative Hx might be acceptable for advanced HCC with MVI or ≤3 residual tumors in the remnant liver.

Short- and long-term outcomes of the Frey procedure for chronic pancreatitis: a single-center experience and summary of outcomes in Japan.

PURPOSE: To evaluate the short- and long-term outcomes of the Frey procedure for chronic pancreatitis (CP). METHODS: The subjects of this study were 12 patients who underwent the Frey procedure for CP between January, 2000 and December, 2016. We assessed pain relief, weight gain, and exocrine/endocrine insufficiency during follow-up. RESULTS: The study population comprised 11 men and 1 woman (91.7% vs. 8.3%; mean age, 50.3 ± 6.8 years; range 39-61 years). Pancreatitis was caused by alcohol in 9 (75%) patients and was idiopathic in 3 (25%) patients. The mean follow-up period was 82.5 ± 46.5 months (range 16.9-152.1 months). There was no operative mortality, but three patients (25%) suffered postoperative morbidity. All patients were pain-free at the time of discharge. There was no case of new-onset diabetes mellitus after surgery, although one patient (8.3%) suffered exocrine insufficiency. The body weight and body mass index of all patients improved during follow-up. Only one patient continued to suffer pain in the long term. CONCLUSION: The findings of this long-term follow-up of patients who underwent the Frey procedure suggest that it offers effective pain relief and is a safe technique for the management of CP.

Multifocal Mass Lesions in Autoimmune Pancreatitis.

A 59-year-old male patient with jaundice was referred to our hospital because of mass lesions in the pancreatic head and tail. An immunological examination revealed an elevated serum IgG4 level. Computed tomography showed two clear boundary mass lesions in the pancreatic head and tail. Magnetic resonance imaging showed that the mass lesions exhibited low intensity on T1-weighted images and iso-intensity on T2-weighted images. Magnetic resonance cholangiopancreatography showed an obstruction of the main pancreatic duct in the pancreatic head and tail. The possibility of malignant tumors could not be ruled out; therefore, we performed total pancreatectomy. A histopathological examination of the nodular lesions revealed severe lymphoplasmacytic infiltration and inflammatory change around the pancreatic ducts. Immunohistochemistry revealed diffuse infiltration of IgG4-positive plasma cells in the nodules. According to these pathological findings, we diagnosed the patient with IgG4-related multifocal mass lesions of autoimmune pancreatitis (AIP). It is difficult to distinguish between focal type AIP and pancreatic cancer. We herein report a rare case of multifocal mass lesions in AIP and include bibliographical comments.

Hepatectomy for Hilar Cholangiocarcinoma with Right-Sided Ligamentum Teres Using a Hepatectomy Simulation System.

Right-sided ligamentum teres (RSLT) is a rare congenital anomaly often accompanied by variation of the hepatic vasculature. We herein report a surgical case of a hilar cholangiocarcinoma with RSLT in whom preoperative hepatectomy simulation proved useful for understanding the anatomical structure of the liver. A 78-year-old male with obstructive jaundice was referred to our department for further examination. The patient was suspected of having a hilar cholangiocarcinoma originating from the left hepatic bile duct by contrast-enhanced computed tomography (CT), and CT also showed right umbilical portion (RUP). Three-dimensional images of the hepatic vasculature and biliary system reconstructed using a hepatectomy simulation system suggested that all portal branches ramified from RUP were right paramedian branches, and three leftward portal branches from these ran parallel to the peripheral bile ducts confluent with the left hepatic bile duct, where the tumor was present. Hepatic resection of part of the ventral area of the right paramedian sector and left hemiliver was performed along the demarcation line drawn after clamping the portal branches; the ratio of estimated liver resection volume was 28.9%. After the operation, bile leakage occurred. However, the leakage was treated with percutaneous drainage alone, and the patient was discharged 77 days after the operation. The patient is doing well without any signs of recurrence 21 months after the operation. The vascular and biliary anatomy in patients with RSLT is complicated and should be evaluated in detail preoperatively using a hepatectomy simulation system.

Perioperative and Long-term Outcome of the Warshaw Technique in Laparoscopic Spleen-preserving Distal Pancreatectomy.

PURPOSE: The purpose of this study was to compare the safety and outcomes of laparoscopic splenic vessel preservation (Lap-SVP) to those of laparoscopic Warshaw technique (Lap-WT). MATERIALS AND METHODS: Fourteen patients underwent Lap-SVP and 5 patients underwent Lap-WT. Their clinical data and outcomes of the 2 procedures were compared. RESULTS: The operative time, intraoperative blood loss, clinically relevant pancreatic fistula, and the rate of splenic infarction were significantly higher in the patients who underwent Lap-WT than in those who underwent Lap-SVP. In both groups, the platelet count ratio and splenic volume ratio at 1 month after the operation did not differ from the preoperative values. Two patients in the Lap-WT group experienced splenic infarction, and could be observed without any treatment. CONCLUSIONS: The long-term outcome of Lap-WT was relevant. Therefore, Lap-WT could be a treatment option for patients who may not have undergone Lap-SVP.

11C-Choline positive but 18F-FDG negative pancreatic metastasis from renal cell carcinoma on PET.

Choline is a new PET tracer, which uptake may occur via a choline-specific transporter protein and be accelerated during the proliferation of tumor cells. We report a 61-year-old woman with a metastatic pancreatic tumor from renal cell carcinoma, measuring 35×40 mm. PET scans demonstrated accumulation of 11C-choline in the metastatic pancreatic tumor, but no accumulation of 18F-FDG. Choline PET/CT may play a useful and complementary imaging modality, especially when FDG-PET/CT does not show expected findings or when the evaluation of tumor viability is needed, in patients with renal cell carcinoma.

Laparoscopic Resection of an Epithelial Cyst in an Intrapancreatic Accessory Spleen.

An epithelial cyst in an intrapancreatic accessory spleen (ECIAS) is rare. We herein report a case of a patient with ECIAS who underwent laparoscopic surgery. A 57-year-old woman was referred to our hospital because of a pancreatic tail tumor. She was asymptomatic, and a physical examination revealed no remarkable abnormalities. The levels of the tumor marker carbohydrate antigen 19-9 (CA19-9) and s-pancreas-1 antigen (SPan-1) were elevated. Ultrasonography showed a well-defined homogeneous cystic tumor. Computed tomography showed a well-demarcated cystic tumor in the pancreatic tail. Magnetic resonance imaging showed that the cystic tumor exhibited low intensity on T1-weighted images and high intensity on T2-weighted images. The cystic tumor was diagnosed as mucinous cystic neoplasm preoperatively. The patient underwent laparoscopic spleen-preserving distal pancreatectomy. A histopathological examination revealed the cyst wall to be lined by stratified squamous epithelium within splenic parenchyma, and the ultimate diagnosis was ECIAS. The postoperative course was uneventful, and the patient was discharged on postoperative day 12. ECIAS is very difficult to diagnose preoperatively. Laparoscopic surgery is a safe and minimally invasive procedure for patients with difficult-to-diagnose pancreatic tail tumor suspected of having low-grade malignancy.

Bronchobiliary fistula caused after hepatectomy for hepatocellular carcinoma: a case report.

BACKGROUND: A bronchobiliary fistula, an intercommunication between the biliary tract and bronchial trees, is an extremely rare complication after hepatectomy. CASE PRESENTATION: A 70-year-old male underwent partial resection of the liver for recurrent hepatocellular carcinoma under a thoracoabdominal approach. The immediate postoperative clinical course was uneventful, but the patient was febrile and laboratory examinations revealed leukocytosis on the 15th postoperative day. An intraabdominal abscess was suspected based on the computed tomography findings, and percutaneous drainage was performed. Bile was drained, and fluoroscopy using a contrast medium from the drainage tube revealed a communication between the cavity and the common hepatic duct. Two weeks after drainage, bilioptysis was seen. Fistulography demonstrated the presence of the bronchus in the right lower lobe of the lung via the subphrenic space. Therefore, the patient was diagnosed to have a bronchobiliary fistula. Fistulography revealed closure of the communication with the bronchus about a month after drainage. However, the bile leakage and bilioptysis did not stop even after endoscopic nasogastric biliary drainage, and ethanol injection therapy were performed. Eventually, residual right bisectionectomy without resection of the fistulous tract and involved lung was performed to remedy the intractable bile leakage. The clinical course after the reoperation was good without bile leakage, bilioptysis, or pulmonary disorders, and the patient was discharged 40 days after reoperation. CONCLUSIONS: We experienced a rare case of bronchobiliary fistula that occurred after hepatectomy for hepatocellular carcinoma. Careful attention should be paid to prevent bile leakage during hepatectomy, since bile leakage has the potential to cause a bronchobiliary fistula.

Synchronous double cancers of primary hepatocellular carcinoma and cholangiolocellular carcinoma: a case report.

Synchronous double cancers consisting of hepatocellular carcinoma (HCC) and cholangiolocellular carcinoma (CoCC) are extremely rare. We herein report a surgical case of synchronous double cancers in a patient with primary HCC and CoCC. A 45-year-old man with hepatitis B was admitted to our hospital with hepatic tumors. The level of protein induced by vitamin K antagonist (PIVKA-II) was found to be elevated. Computed tomography (CT) revealed a 23-mm tumor with early-phase enhancement and late-phase washout in the 6th segment of the liver, and a 10-mm tumor with slight early-phase enhancement and late-phase washout in the 7th segment of the liver. Magnetic resonance imaging (MRI) revealed that the two tumors in the 6th and 7th segments showed low intensity on T1-weighted images and high intensity on T2-weighted images. Based on those preoperative examinations, the liver tumors were diagnosed as multiple primary hepatocellular carcinomas. The patient underwent a posterior segmentectomy. A histopathological examination revealed that the tumor of the 6th segment of the liver was moderately differentiated HCC, and that the tumor of the 7th segment of the liver was CoCC. The postoperative course was uneventful. However, lymph node recurrence was observed 6 months later and the patient died 20 months after surgery. There are only six reported surgical cases of synchronous double primary liver cancers consisting of HCC and CoCC. We are of the opinion that curative resection may be an effective treatment for double cancer consisting of HCC and CoCC, and that it may provide long-term survival.

Hepatic Pseudolymphoma with Fluorodeoxyglucose Uptake on Positron Emission Tomography.

A 69-year-old woman with chronic hepatitis B was admitted to our hospital with a hepatic tumor. The levels of 2 tumor markers, carcinoembryonic antigen and carbohydrate antigen 19-9, were slightly elevated; however, the α-fetoprotein and protein levels induced by vitamin K antagonist II were within the normal limits. Abdominal ultrasonography showed a well-defined peripheral hypoechoic mass that was isoechoic and homogeneous on the inside. Computed tomography showed a poorly enhanced tumor of 13 mm in diameter in the 5th segment of the liver. Fluorodeoxyglucose positron emission tomography showed a slight uptake (maximum standard uptake value 3.4) by the hepatic tumor. These findings suggested cholangiocellular carcinoma, and we performed anterior segmentectomy of the liver. A histopathological examination showed a hepatic pseudolymphoma. The patient's postoperative course was uneventful, and she remains alive without recurrence 5 months after undergoing surgery. In most cases, hepatic pseudolymphoma is preoperatively diagnosed as a malignant tumor and a definite diagnosis is made after resection. It is therefore necessary to consider hepatic pseudolymphoma as a differential diagnosis in patients with hepatic tumors.

Randomized Controlled Trial of Pancreaticojejunostomy versus Stapler Closure of the Pancreatic Stump During Distal Pancreatectomy to Reduce Pancreatic Fistula.

OBJECTIVES: The aim of this study was to evaluate in a multicenter randomized controlled trial (RCT) whether pancreaticojejunostomy (PJ) of pancreatic stump decreases the incidence of pancreatic fistula after distal pancreatectomy (DP) compared with stapler closure. BACKGROUND: Several studies reported that PJ of pancreatic stump reduces the incidence of pancreatic fistula after DP. However, no RCT has confirmed the efficacy of PJ of pancreatic stump. METHODS: One hundred thirty-six patients scheduled for DP were enrolled in this study between June 2011 and March 2014 at 6 high-volume surgical centers in Japan. Enrolled patients were randomized to either stapler closure or PJ. The primary endpoint was the incidence of pancreatic fistula based on the International Study Group on Pancreatic Fistula criteria. This RCT was registered with ClinicalTrials.gov (NCT01384617). RESULTS: Sixty-one patients randomized to stapler and 62 patients randomized to PJ were analyzed by intention-to-treat. Pancreatic fistula occurred in 23 patients (37.7%) in the stapler closure group and 24 (38.7%) in the PJ group (P = 0.332) in intention-to-treat analysis. The incidence of clinically relevant pancreatic fistula (grade B or C) was 16.4% for stapler closure and 9.7% for PJ (P = 0.201). Mortality was zero in both groups. In a subgroup analysis for thickness of pancreas greater than 12 mm, the incidence of clinically relevant pancreatic fistula occurred in 22.2% of the patients in the stapler closure group and in 6.2% of the PJ group (P = 0.080). CONCLUSIONS: PJ of the pancreatic stump during DP does not reduce pancreatic fistula compared with stapler closure.

Successful laparoscopic extirpation of peritoneal dissemination after hepatectomy for ruptured hepatocellular carcinoma.

Clinically, peritoneal dissemination of hepatocellular carcinoma (HCC) rarely occurs. We herein report a case that had a good outcome following laparoscopic extirpation of peritoneal dissemination after hepatectomy for ruptured HCC. A 66-year-old man underwent central bisectionectomy 12 days after emergency transcatheter arterial embolization for a ruptured HCC. Thereafter, pulmonary resection was performed twice for lung metastasis. About 8 months after the second pulmonary resection, a mass lesion was detected at the left subphrenic space on CT and (18) F-fluorodeoxyglucose PET scans. We made a diagnosis of peritoneal dissemination of HCC, and laparoscopic extirpation was performed. The patient is now doing well without any signs of recurrence 2 years after the last operation. Laparoscopic surgical resection for peritoneal dissemination that develops after hepatectomy for HCC may have a beneficial effect as a less-invasive approach and may improve the prognosis in select patients.

Development of hepatocellular carcinoma in cardiac congestive liver fibrosis: report of a case.

We report an extremely rare case of the development of hepatocellular carcinoma (HCC) in cardiac congestive liver fibrosis. A 62-year-old female presented to our hospital with a complaint of right upper quadrant pain. The patient had undergone cardiac surgery for pulmonary valve insufficiency, pulmonary stenosis and atrial septal defect when she was fifteen years of age. During the subsequent 47 years, she had occasionally suffered from various symptoms associated with right-sided heart failure due to pulmonary stenosis. Computed tomography revealed a liver tumor measuring 63 mm in diameter in segment 5 and other liver tumors in segments 5 (18 mm), 8 (17 mm) and 4 (12 mm), which were diagnosed as HCCs. There was no evidence of stenosis in any hepatic veins or inferior vena cava, and no infectious hepatitis or alcoholic liver damage. Anterior sectionectomy and partial resection of segment 4 was performed, and histological examination showed that these tumors were HCC accompanied by congestive liver fibrosis. Nine months later, multiple recurrent HCCs were detected in segment 6, and transcatheter arterial chemoembolization was employed thereafter. The patient died 40 months after surgery due to advanced recurrence.

Granulocyte colony-stimulating factor-producing cholangiocellular carcinoma.

A 61-year-old female was admitted to our hospital with epigastric pain and fever. The laboratory data showed severe inflammatory reactions. Computed tomography revealed an irregular tumor in the left hepatic lobe and swelling of lymph nodes. (18)F-fluorodeoxy-glucose positron emission tomography (FDG-PET) showed high uptake by the tumor, with diffuse uptake in the spine. Based on the elevated leukocyte count and FDG-PET findings, the patient was diagnosed with a granulocyte colony-stimulating factor (G-CSF)-producing tumor (G-CSF, 213 pg/mL). We performed left trisegmentectomy of the liver, bile duct resection, and lymph node dissection. Histologically, the tumor was a poorly differentiated adenocarcinoma with some lymph nodes metastasis. Immunohistochemical staining of the tumor cells was positive for G-CSF. Therefore, the tumor was diagnosed as G-CSF-producing cholangiocellular carcinoma. The inflammatory reactions and serum G-CSF level transiently improved immediately after surgery. However, 1 month later, the leukocyte count and serum G-CSF level increased again, and recurrence was observed in the remnant liver. The patient died 3 months after the operation. G-CSF-producing cholangiocellular carcinoma is rare. This tumor progresses rapidly, and surgical treatment for advanced condition should be carefully selected.