Randomized controlled Phase III study comparing hepatic arterial infusion with systemic chemotherapy after curative resection for liver metastasis of colorectal carcinoma: JFMC 29-0003.

BACKGROUND: The feasibility and efficacy of adjuvant hepatic arterial infusion (HAI) in preventing the development of liver metastases in patients with advanced colon carcinoma have not been validated. The aim of this randomized controlled study was to compare the feasibility of HAI and the protective effect against liver metastasis after curative resection to those of systemic chemotherapy. METHODS: Between July 2000 and June 2003, 91 patients were enrolled. Patients were randomly assigned to receive 5-fluorouracil (5-FU) via continuous venous infusion (CVI) or intra-hepatic arterial weekly high-dose 5-FU (WHF). The primary endpoint was overall survival (OS). RESULTS: In the WHF group, the cumulative failure rate of hepatic arterial catheterization was 16.7% at 6 months. The occurrence of grade 3 adverse events was comparable between the groups. The 5-year OS rates were 59.0% in the CVI group and 34.9% in the WHF group (P = 0.164). CVI tended to show a protective effect against liver metastasis regarding the 5-year liver-specific cumulative recurrence rate: CVI, 45.0% vs. WHF, 68.3%; P = 0.037). CONCLUSION: HAI therapy has a certain protective effect against liver metastasis after curative resection in patients with colorectal cancer. However, this therapy did not contribute to any marked improvement in their overall survival.

Interventional Treatment of Severe Portal Vein Thrombosis after Living-Donor Liver Transplantation.

Portal vein thrombosis (PVT) is a rare complication of liver transplantation which can lead to graft failure and patient death. Treatment can be difficult, especially in cases of PVT from the intrahepatic portal vein to the proximal jejunal veins. A 55-year-old woman had undergone living-donor liver transplantation with splenectomy for end-stage liver cirrhosis due to hepatitis C with hepatocellular carcinoma. Ten months after transplantation, massive ascites and slight abdominal pain developed, and computed tomography revealed a PVT between the intrahepatic portal vein and the superior mesenteric vein. Repeated interventional radiology procedures were used in combination with thrombolysis, thrombectomy, and metallic stent replacement to obtain favorable portal flow to the graft. Five years after being treated, the patient is well, with favorable portal flow having been confirmed. In conclusion, repeated and assiduous interventional radiological treatment combined with thrombolytic therapy, thrombectomy, and metallic stent replacement could be important for severe PVT.

Enterovesical fistula caused by non-Hodgkin's lymphoma of the ileum: report of a case.

We herein present a rare case of enterovesical fistula caused by ileal non-Hodgkin's lymphoma. A 75-year-old Japanese male presented with macrohematuria at Kosei General Hospital in December 2010. An egg-sized mass was palpable in his right lower abdominal region, and computed tomography (CT) revealed that the ileal tumor had invaded the right posterior wall of the urinary bladder (UB). A histopathological examination of a CT-guided needle biopsy specimen revealed diffuse large B-cell lymphoma involving the ileum and the UB. Thereafter, fecaluria appeared. A transurethral catheter was put in place, and there were no symptoms of cystitis. The patient received chemotherapy for the lymphoma, which produced a partial response. However, the fecaluria continued, and an examination of the small intestine with contrast revealed a thick and irregular wall of the ileum and a fistula between the ileum and UB. A partial resection of the ileum and a partial cystectomy were carried out in April 2011. The surgical specimen demonstrated two tumors 5 cm apart in the ileum, measuring 4.5 × 7 and 4 × 3 cm in size. The proximal tumor had directly invaded the UB and formed an ileovesical fistula. The patient made a good recovery and was doing well 5 months after the surgery without any evidence of recurrence.

Silicon drain with channels along the sides for internal biliary stenting of hepaticojejunostomy in hepatic hilar malignancies.

BACKGROUND: We compared two types of stents in patients who underwent surgery for hepatic hilar malignancies. METHODS: Twenty-one patients with hepatic hilar malignancies who underwent hepatectomy were randomly assigned to one of two groups. A 5-Fr silicon drain with an internal lumen and side holes was used for the hepaticojejunostomy in one group (intraluminal stent group), and a 10-Fr silicon drain with channels along the sides was used in the other (channel stent group). RESULTS: Leakage developed in four patients (36.4%) in the intraluminal stent group versus two (20.0%) in the channel stent group. Cholangitis developed in three patients with leakage (27.3%) in the intraluminal stent group versus no patient in the channel stent group. After operation, the times required for the serum alkaline phosphatase and total bilirubin levels to return to the normal range were significantly shorter in the channel stent group (5.3 +/- 2.9, 3.8 +/- 2.2 days) than in the intraluminal stent group (17.0 +/- 5.8, 9.4 +/- 5.7 days) (P < 0.0001, P = 0.0093). CONCLUSION: A 10-Fr silicon drain with channels is superior to a 5-Fr silicon drain with an internal lumen for internal biliary stenting of hepaticojejunostomy in patients with hepatic hilar malignancies.

Quantitative analysis of fluorouracil-related genes in chronic viral hepatitis using microdissection.

BACKGROUND/AIMS: Dihydropyrimidine dehydrogenase is the initial and rate-limiting enzyme in the catabolism of 5-fluorouracil. The aim of this study was to determine the levels of messenger RNA for 5-fluorouracil-related metabolic enzymes in cirrhotic liver and to assess the correlation between these mRNA levels and clinicopathological features. METHODOLOGY: The study material consisted of 33 liver samples. The levels of mRNA for the 5- fluorouracil-related metabolic enzymes were quantified by real-time reverse transcription polymerase chain reaction combined with laser-captured microdissection. RESULTS: The Dihydropyrimidine dehydrogenase mRNA level in patients with grade B liver damage was significantly lower than that in patients with grade A liver damage (p=0.009). The Dihydropyrimidine dehydrogenase and orotate phosphoribosyl transferase mRNA level in al samples was higher than that in a2 and a3 samples (p= 0.01 and 0.013, respectively). Statistically significant correlations were found between the hyaluronic acid and the thymidylate phosphorylase mRNA level (p= 0.0001), and the T-BIL and the dihydropyrimidine dehydrogenase mRNA level (p=0.01). CONCLUSIONS: The level of Dihydropyrimidine dehydrogenase mRNA may be affected by the clinicopathological status of patients with cirrhosis.

Surgical disconnection of patent paraumbilical vein in refractory hepatic encephalopathy.

BACKGROUND: Refractory hepatic encephalopathy (HE) frequently develops in patients with cirrhosis and portal-systemic shunt. Recently, patients with refractory HE associated with portal-systemic shunt have been treated with interventional radiology. We describe a promising new treatment for portal-systemic shunt, ligation of the patent paraumbilical vein (PUV) after partial splenic embolization, in patients with refractory HE. PATIENTS: Four patients with cirrhosis (3 women and 1 man; mean age, 56 years) and refractory HE due to a patent PUV were studied. Patency of the PUV had recurred in 1 patient after primary occlusion by interventional radiological procedures. The Child-Pugh class was B in 2 patients and C in 2. Before the present treatment, all patients had been hospitalized at least 3 times because of recurrent HE. SURGICAL PROCEDURE: Partial splenic embolization was performed in all patients to decrease portal venous pressure before surgery. Surgical ligation of the patent PUV was performed under epidural anesthesia. The patent PUV was carefully skeletonized and doubly ligated. Esophageal varices were evaluated with upper gastrointestinal endoscopy before and after surgery. RESULT: The mean follow-up duration was 15.8 months. After ligation, there were no clinically significant complications. Esophageal varices were unchanged. The serum ammonia level was higher before surgery (162.3 +/- 56.4 mug/dL, mean +/- SD) than after surgery (41.8 +/- 20.2 mug/dL; p=0.0299). No patient had symptoms of HE. CONCLUSION: Ligation of the patent PUV is an effective treatment for patients with refractory HE.

Eversion and ligation of a diverticulum: report of an inspirational case and subsequent animal study.

Although most patients with colonic diverticula have no symptoms, perforation or bleeding can be troublesome. Recently, we treated an elderly patient with a diverticular perforation of the sigmoid colon who required a loop colostomy and later resection of the diseased colon. Between the operations, the colostomy resembled a sea slug with horns, which represented eversions of the diverticula. This interesting phenomenon suggests that diverticula may be everted as pseudopolyps and ligated endoscopically. Therefore, the procedure was tested on a Landrace pig, of which the large bowel wall was pulled with an endoscope at 9 different sites using forceps (n=6) or suction (n=3) and ligated. Four days later, reexamination revealed total necrosis at 8 sites and ulceration at 1. The animal was well. The bowel removed 2 weeks later showed mucosal fold convergences without perforations. Our procedure can be used for prophylactic or therapeutic treatment of patients with colonic diverticula.

Hepatotoxicity caused by both tacrolimus and cyclosporine after living donor liver transplantation.

We present a case report of a posttransplant patient who had hepatotoxicity due to both tacrolimus and cyclosporine and cholestatic jaundice due to tacrolimus. The patient did not show sustained improvement in enzyme and bilirubin abnormalities after an initial change from tacrolimus to cyclosporine or with a change back to tacrolimus, but he ultimately showed improvement when the blood concentration of tacrolimus was lowered. A 56-year-old man with subacute fulminant hepatitis induced by acarbose was admitted to our hospital for living donor liver transplantation. The liver graft consisted of the left lobe from his ABO-identical son. The early posttransplant course was uneventful. The serum levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT), and total bilirubin improved initially, but the ALT and AST levels later increased. A liver biopsy suggested a presumptive diagnosis of drug reaction. All drugs were discontinued, the immunosuppressive agent was changed from tacrolimus to cyclosporine. After initial improvement, the ALT and AST levels increased again. Assuming a reaction to cyclosporine, we decreased the concentration of cyclosporine in the blood. The enzyme levels improved temporarily but again began to rise. We changed the immunosuppressive agent to tacrolimus, which resulted in improvements in the ALT and AST levels; however, the total bilirubin level increased. We interpreted this increase as tacrolimus-induced cholestasis; in response, we decreased the blood concentration of tacrolimus to between 3 and 5 ng/dL and added 1,000 mg of mycophenolate mofetil to the drug regimen. The patient recovered without further complications. Repeated liver biopsies throughout the hospital course suggested that the mild mononuclear cell infiltration observed in a few triads had not been caused by acute rejection but had possibly been drug-induced.

Long-term results of elective hepatectomy for the treatment of ruptured hepatocellular carcinoma.

BACKGROUND/PURPOSE: According to the General rules for the clinical and pathological study of primary liver cancer, compiled by the Liver Cancer Study Group of Japan, ruptured hepatocellular carcinoma (HCC) is classified as stage 4, even if the tumor is small and solitary. We examined the long-term results of elective hepatectomy for the treatment of ruptured HCC. METHODS: A first hepatectomy was performed without operative death in 193 patients with HCC. Ten patients had ruptured HCC (ruptured group) and 183 patients had nonruptured HCC (nonruptured group). The extension of HCC was macroscopically classified as stage 1 in 23 patients, stage 2 in 71, stage 3 in 53, and stage 4 in 46. RESULTS: Cumulative survival rates in the ruptured group at 1, 5, and 10 years were 90.0%, 67.5%, and 20.3%, respectively. The cumulative survival rate was lower in patients with stage 4 disease in the nonruptured group than that in patients in the ruptured group (P < 0.05). Cumulative survival rates did not differ significantly between patients in the ruptured group and those with stage 2 or stage 3 disease. CONCLUSIONS: Survival rates after elective hepatectomy in patients with ruptured HCC are good, even if the disease is classified as stage 4.

Curative resection of primary hepatic gastrinoma.

Primary hepatic gastrinoma is very rare, with fewer than 20 cases reported. We describe a 44-year-old woman in whom primary hepatic gastrinoma was strongly suspected clinically. The patient was referred to our hospital because of intractable diarrhea. She had elevated serum levels of alanine aminotransferase, aspartate aminotransferase, and fasting gastrin. A calcium provocative test showed a marked elevated serum gastrin level (17,000 pg/ml). Abdominal ultrasonography, computed tomography, and magnetic resonance imaging revealed a tumor in the right lobe of the liver, measuring 38 x 33 mm. No other tumor was detected in the pancreas, duodenum, or local lymph nodes on preoperative radiological imaging or endoscopic ultrasonography. The hepatic tumor was resected. Total intraoperative ultra-sonography and intraoperative exploratory palpation of the duodenum, pancreas, and lymph nodes showed no evidence of an extrahepatic tumor. Pathological findings and immunohistochemical studies revealed a neuroendocrine tumor with increased production of gastrin. Postoperatively, the serum gastrin level returned to normal.

Profiling of fluorouracil-related genes by microdissection technique in hepatocellular carcinoma.

BACKGROUND/AIMS: 5-fluorouracil (5-FU)-related metabolic enzymes, including dihydropyrimidine dehydrogenase (DPD), thymidylate synthase (TS), thymidylate phosphorylase (TP), and orotate phosphoribosyl transferase (OPRT) are initial, rate-limiting enzymes in the metabolism of 5-FU. The therapeutic implications of these enzymes in hepatocellular carcinoma (HCC) remain poorly understood. We used a newly developed laser-captured microdissection technique combined with RNA extraction to examine the mRNA levels of 5-FU-related metabolic enzymes in HCC and adjacent liver tissue. METHODOLOGY: The study material comprised 43 paired specimens of HCC and adjacent liver tissue. The mRNA levels of 5-FU-related metabolic enzymes were quantified by real-time reverse-transcriptase polymerase chain reaction combined with laser-captured microdissection. RESULTS: The DPD mRNA level in HCC (4.31 +/- 4.21) was lower than that in adjacent liver (6.53 +/- 2.93) (p < 0.001). The TS mRNA level in HCC (3.55 +/- 2.54) was higher than that in adjacent liver (1.90 +/- 0.11) p < 0.001). The TP and the OPRT mRNA levels did not differ significantly between HCC and adjacent liver. The TS mRNA level of HCC with portal invasion (4.47 +/- 2.76) was higher than that of HCC without portal invasion (2.71 +/- 1.96) (p = 0.015). The DPD mRNA level of HCC with septum formation (4.89 +/- 4.82) was significantly higher than that of HCC without septum formation (2.12 +/- 0.61) (p < 0.027). The OPRT mRNA level of poorly differentiated HCC (1.18 +/- 0.49) was lower than that of moderately or well-differentiated HCC (2.42 +/- 1.82) (p = 0.037). CONCLUSIONS: The DPD mRNA level was lower and the TS mRNA level was higher in HCC than in adjacent liver. Our results will hopefully stimulate further investigations designed to optimize the use of 5-FU in patients with HCC.

Surgical treatment for better prognosis of patients with liver metastases from colorectal cancer.

BACKGROUND/AIMS: Hepatic resection is established as the best available treatment for colorectal cancer metastases to the liver, but the optimal procedure for the resection is now controversial. In this study we retrospectively evaluated the influence of the selection of surgical procedures for hepatic resection in determining the prognostic factors. METHODOLOGY: A population of 111 patients with liver metastases from colorectal cancer underwent initial and radical hepatic resection. Survival rates as a function of clinical, primary tumor pathologic TNM classification and surgical determinates were examined retrospectively with univariate and multivariate analyses. RESULTS: The overall, 1-, 3-, 5- and 10-year survival rates were 85.5%, 51.4%, 41.6% and 30.9%, respectively. Patient survival was uncorrelated with any of the following factors: sex, age, temporal relationship, primary tumor site, tumor stage, pathological depth, pathological vascular invasion, pathological lymphatic invasion and pathological lymph node metastases of the primary tumor, maximum diameter of liver lesions, intrahepatic distribution patterns, type of hepatectomy, and number of hepatectomies. However, the number of liver tumors and the tumor-free margins were significantly associated with good prognosis. CONCLUSIONS: Safe hepatectomy with adequate tumor-free margins leads to a better prognosis.

Successful transjugular balloon dilatation of the hepatic vein stenosis causing hypoalbuminemia after pediatric living-donor liver transplantation.

Hepatic vein (HV) stenosis after liver transplantation is a rare but life-threatening complication that leads to graft loss. The incidence of HV stenosis after living-donor liver transplantation (LDLT) and split liver transplantation is not so rare, and is reported to range from 2 to 8.6%. Recently, the minimal invasiveness of radiologic interventions and the innovative techniques have led to their widespread adoption for the treatment of various complications after liver transplantation. We report a five-year-old boy with HV stenosis post-LDLT with only scarcity of clinical information including edema with hypoalbuminemia and elevated hyaluronic acid value. Doppler ultrasonography was effective to suggest stenosis, and angiography confirmed its diagnosis. The stenosis was successfully treated with balloon dilatation using interventional radiologic technique through the jugular vein. In conclusion, we suggest that if liver transplanted patients manifest ascites or/and edema with hypoalbuminemia and elevated hyaluronic acid in sera, HV stenosis should be suspected even if the other laboratory values are normal.

Peritoneovenous shunts for palliation of malignant ascites.

BACKGROUND: Malignant ascites may produce a cluster of symptoms that include abdominal distention, early satiety, respiratory embarrassment, impaired mobility, and lethargy, and relief of these symptoms is often difficult to achieve. We report on the placement of peritoneovenous shunts (PVSs) in a group of patients with malignant ascites, with particular reference to the effectiveness and complications of the procedure. PATIENTS AND METHOD: PVSs were inserted in 9 patients with malignant ascites after obtaining their informed consent. The patients were 6 men and 3 women with a median age of 59 years. All had previously been treated with vigorous diuretic therapy or repeated paracentesis or both. Shunt insertion was carried out via a percutaneous approach under local anesthesia. RESULTS: The procedure was well tolerated by all patients. The abdominal distention resolved in all patients, and urine volume increased significantly, demonstrating that the PVS did not affect renal function. The platelet count was reduced, and prothrombin time was prolonged. Two patients had the complication of shunt occlusion, and both patients underwent shunt replacement. There were no lethal complications. Median survival time after PVS placement was 21 days (range, 10 approximately 90 days), and the shunt was functioning at the time of death with good control of ascites in all patients. CONCLUSIONS: Malignant ascites produces troublesome symptoms for patients, who may live for some time. Placement of a PVS is a well-tolerated, relatively minor surgical procedure that can provide excellent control of ascites in most patients selected. The selection of optimal patients requires further study.

Major arterioportal shunt caused by hepatocellular carcinoma.

A case of hepatocellular carcinoma (HCC) causing a major arterioportal (A-P) shunt is reported. The patient exhibited massive ascites and tested positive for hepatitis B surface antigen. An abdominal computed tomography (CT) examination showed a low-density lesion in the left lobe of the liver and an A-P shunt, but no tumor stain was visible. Upper gastrointestinal endoscopy revealed severe esophageal varices. Because the tumor marker level was abnormally high, an HCC causing an A-P shunt in a cirrhotic liver background with severe esophageal varices as a result of portal hypertension was diagnosed. We performed endoscopic variceal ligation to treat the severe esophageal varices and interventional radiology treatment for the A-P shunt and HCC, but the patients condition was unchanged. Because the patients liver function gradually improved, surgical treatment was selected. The patient underwent left hepatectomy. Pathological examination revealed a poorly differentiated HCC in a cirrhotic liver background. The postoperative course was uneventful, and the patient was discharged 2 weeks after the operation. The patient subsequently underwent transcatheter arterial embolization therapy for recurrent HCC in the right lobe of the liver, but the esophageal varices disappeared.

Fixation of the greater omentum for prevention of delayed gastric emptying after left hepatectomy with lymphadenectomy for cholangiocarcinoma.

BACKGROUND/PURPOSE: Delayed gastric emptying (DGE) sometimes occurs after left hepatectomy with lymphadenectomy. We performed fixation of the greater omentum to the peritoneum to prevent DGE and other complications in patients undergoing left hepatectomy with lymphadenectomy for cholangiocarcinoma. METHODS: Patients undergoing left hepatectomy with lymphadenectomy for cholangiocarcinoma were divided into a fixation group (n = 9) and a nonfixation group (n = 10). In the fixation group, the greater omentum was sutured to the peritoneum 3 cm from the greater curvature of the stomach to prevent contact between the stomach and the cut surface of the liver. RESULTS: Mean age, sex, disease, and hepatectomy procedures were similar in the two groups. DGE developed in six patients in the nonfixation group (60.0%) versus no patients in the fixation group (P = 0.011). The total number of complications was higher in the nonfixation group (16 complications) than in the fixation group (3 complications; P = 0.003). Overall morbidity was higher in the nonfixation group (60.0%) than in the fixation group (22.2%). Postoperative hospitalization was longer in the nonfixation group (67.4 days) than in the fixation group (20.2 days; P = 0.0041). CONCLUSIONS: Fixation of the greater omentum to the peritoneum decreases the occurrence of DGE and other complications after left hepatectomy with lymphadenectomy for cholangiocarcinoma.

Recurrence of colonic cancer twice at the site of stapled colorectal anastomosis.

Recurrence at the site of a stapled anastomosis is generally believed to result from the luminal implantation of viable cancer cells during stapling. We report a case in which colon cancer recurred twice at the site of a stapled anastomosis, despite povidone iodine (PVP-I) lavage consisting of an enema with 5% PVP-I solution before the operation and intraoperative lavage of the rectal remnant and the descending colon with a 10% PVP-I solution. Three months after sigmoidectomy to resect a carcinoma of the sigmoid colon, a circular anastomotic recurrence was found at the suture line after anastomosis with a stapler. However, 11 months after the subsequent resection and reanastomosis to remove the first anastomotic recurrence, another anastomotic recurrence was found. We performed abdominoperineal resection for the second recurrence at the site of the stapled anastomosis. Suture-line recurrence could not be prevented in the present case despite lavage with a PVP-I solution for prophylaxis.

Successful percutaneous ultrasound-guided drainage for treatment of a splenic abscess.

We report a case of splenic abscess that was successfully treated with percutaneous ultrasound-guided drainage and without splenectomy. A 40-year-old woman was admitted to Nippon Medical School Hospital because of pyrexia and left upper quadrant pain, which had persisted despite antibiotic treatment. On admission, computed tomography demonstrated a low-density area in the spleen, which had been been seen on computed tomography 3 months earlier. Ultrasonography demonstrated a hypoechoic area in the spleen. Initial laboratory tests revealed a serum C-reactive protein concentration of 19.7 mg/dl and a white blood cell count of 15,800 /microl. The serum glucose concentration was 267 mg/dl, and the glycolated hemoglobin value was 7.7%. A splenic abscess was diagnosed and was treated with percutaneous drainage. Milky yellow fluid was obtained, and the patients left upper quadrant abdominal pain and pyrexia resolved. A culture of the drainage fluid yielded Escherichia coli. The drainage catheter was removed 12 days after insertion. The patient was discharged 6 days later. The splenic abscess has not recurred during 3 months of follow-up. Our results suggest that ultrasound-guided percutaneous drainage is a safe and effective alternative to surgery for the treatment of splenic abscess and allows preservation of the spleen.

Usefulness of laparoscopic hepatectomy.

BACKGROUND: The aim of this study was to investigate the advantages of laparoscopic hepatectomy over open surgery for liver tumors. PATIENTS AND METHOD: A retrospective study was performed of 10 patients with liver tumors (9 with hepatocellular carcinoma and 1 with focal nodular hyperplasia) at our hospital. Five patients who had received laparoscopic hepatectomy (Lap-Hx group) were compared with 5 patients who had undergone open hepatectomy (O-Hx group) in the same period. The operative procedure was partial hepatectomy and cholecystectomy in both groups. For liver excision, a microwave coagulation device and an ultrasonically activated scalpel were used. RESULTS: Mean patient age was 55.6 +/- 13.9 years in the Lap-Hx group and 51.8 +/- 14.1 years in the O-Hx group. Four patients in the Lap-Hx group had hepatocellular carcinoma with liver cirrhosis and 1 patient had focal nodular hyperplasia. All patients in the O-Hx group had hepatocellular carcinoma and 4 patients had associated liver cirrhosis. The mean tumor size was 2.6 +/- 1.5 cm in the Lap-Hx group and 3.0 +/- 1.8 cm in the O-Hx group. The two groups did not thus differ significantly in the preoperative background factors. Blood loss and duration of the postoperative hospital stay were significantly less in the Lap-Hx than in the O-Hx groups(213 +/- 82 vs 247 +/- 97 min; 154 +/- 128 vs 648 +/- 468 ml, p=0.05: and 10.4 +/- 2.3 vs 18.0 +/- 5.1 days, p=0.017), but operating time did not differ significantly. CONCLUSIONS: Laparoscopic hepatectomy has the advantages of reducing the amount of operative blood loss because of the magnified view afforded by the laparoscope and shortening the hospital stay. The procedure is therefore recommended for patients with appropriate liver tumors, in particular, hepatocellular carcinoma in the cirrhotic liver.

Mucin-producing bile duct carcinoma arising from primary sclerosing cholangitis: a case report.

A 60-year-old woman with primary sclerosing cholangitis (PSC) and high levels of ALP, gamma-GTP, and DUPAN-2 was admitted to our institution for examination. The patient did not have ulcerative colitis or pancreatic intraductal papillary mucinous neoplasm. Imaging studies revealed atypical dilation of bile ducts in the left lobe of the liver. Repeated cytologic examinations of the bile showed atypical cells consistent with adenocarcinoma. The patient underwent extended resection of the left lobe of the liver and was found to have intraductal papillary carcinoma with associated mucin-producing bile duct carcinoma. This carcinoma fills dilated bile duct lumens with mucin. This tumor differs morphologically from typical cholangiocarcinoma, which is usually seen in the late stages of PSC. Just one case of mucin-producing bile duct carcinoma arising from PSC has been reported worldwide. The patient has had no signs of recurrence after 27 months. Patients with mucin-producing bile duct carcinoma, as in the case of its pancreatic counterpart, may have a better prognosis and a higher survival rate than patients with typical cholangiocarcinomas.