[Successful R0 Resection of Hilar Cholangiocarcinoma Invading the Inferior Vena Cava through Left Trisegmentectomy with Combined Resection of the Portal Vein and Inferior Vena Cava after Gemcitabine/Cisplatin Combination Chemotherapy-A Case Report].

A 65-year-old woman was admitted to our institution with sonography results indicating a caudate lobe mass. CT showed a large low-density mass in the caudate lobe, extensively involving the inferior vena cava and main portal vein. Moderately differentiated adenocarcinoma was found on transcutaneous biopsy. We therefore regarded this tumor as a severe locally advanced hilar cholangiocarcinoma and initiated gemcitabine/cisplatin combined chemotherapy. The tumor gradually reduced in size. However, after 28 courses of treatment, CT showed persistent tumor invasion in the left trunk of the portal vein and inferior vena cava invasion in succession in the middle; the tumor had not yet invaded the left hepatic vein. Owing to myelosuppression and general malaise, it was difficult to continue chemotherapy. After 32 courses of treatment, the patient underwent a left trisegmentectomy with combined resection of the portal vein and inferior vena cava. Postoperative microscopic findings revealed no apparent invasion of the tumor in the inferior vena cava, thus suggesting successful R0 resection. The patient is alive without recurrence 18 months postoperatively.

Metastatic primary pulmonary leiomyosarcoma to the pancreas diagnosed by endoscopic ultrasound-guided fine-needle aspiration.

A 77-year-old female patient consulted our hospital for an abnormal shadow observed on chest X-ray. Computed tomography revealed the shadow of a mass in the right lower lung lobe and two shadows of masses in the pancreatic head and body. 18F-fluorodeoxyglucose-positron emission tomography showed an intense uptake only in the fields corresponding to these three masses. Each mass was diagnosed as leiomyosarcoma by transcutaneous needle biopsy of the pulmonary mass and endoscopic ultrasound-guided fine-needle aspiration of the pancreatic masses. The primary site was the lung because the pulmonary lesion was solitary, and no tumor was found in other organs. In English language literature, a case of primary pulmonary leiomyosarcoma with metastasis solely to the pancreas has not yet been reported to the best of our knowledge.

T1b primary remnant cystic duct cancer following cholecystectomy: A case report.

INTRODUCTION: Although primary cystic duct cancer is a rare entity, remnant cystic duct cancer is even more rare. We report a case of early cystic duct cancer following cholecystectomy. PRESENTATION OF THE CASE: A 81 year-old man complained temporary loss of appetite. He had underwent cholecystectomy for acute cholecystitis 5 years prior. Contrast enhanced computed tomography, magnetic resonance image and endoscopic ultrasonography showed remnant cystic duct tumor with protrusion to common bile duct. Endoscopic retrograde cholangiography revealed defect of contrast medium around confluence of the remnant cystic duct and common bile duct. We performed step biopsy by using forceps which revealed adenocarcinoma. Based on these findings, extrahepatic bile duct and remnant cystic duct resection were performed. The histopathology showed adenocarcinoma, pap > tub2, filling in remnant cystic duct, 30 mm in size but showed no lymphovascular or perineural invasion, no lymph node metastasis and negative surgical margin, and was classified as pT1bN0M0. CONCLUSION: This is a rare case of primary carcinoma of remnant cystic duct cancer which is detected during computed tomography follow up for hepatic cell carcinoma recurrence. We confirmed remnant cystic duct cancer and its superficial extension to common bile duct with endoscopic ultrasonography and intraductal ultrasonography. Proper curative surgery was performed.

Small Cystic Pancreatic Neuroendocrine Neoplasm with Huge Liver and Bone Metastases.

Pancreatic neuroendocrine neoplasms occasionally have a cystic component. We herein report a case of multiple hepatic tumors, including a huge one and a 24-mm sized pancreatic cystic lesion. The hepatic tumor showed an enhancement pattern consistent with hepatic adenoma. The pancreatic cystic lesion revealed a thickened outside border and a solid inside component, which was enhanced following a contrast study, suggesting cystic pancreatic neuroendocrine neoplasm. Surgical resection was performed. After the surgery, somatostatin receptor scintigraphy detected an occult lumbar spine metastasis. Huge multiple liver and bone metastases of the neuroendocrine neoplasm G2 component were seen, with a G1 small primary lesion.

Diagnostic performance of a new endoscopic scraper for malignant biliary strictures: a multicenter prospective study.

BACKGROUND AND AIMS: The efficacy of ERCP for histologic diagnosis of malignant biliary strictures is disappointingly low. The aim of this study was to investigate the diagnostic performance of a newly developed endoscopic device with scraping loops in combination with conventional biopsy forceps. METHODS: We performed a multicenter single-arm prospective study. Between February 2013 and December 2014, 123 patients with suspected malignant biliary strictures were enrolled in the study. The new device and conventional biopsy forceps were applied for histologic diagnosis by ERCP. The primary outcome was to evaluate cancer detectability by biopsy forceps, the new device, and their combined use. RESULTS: Of the 123 patients, 119 were diagnosed with a malignant stricture. Sufficient samples were collected in 83.7% (103/123), 93.5% (115/123), and 95.9% (118/123) of patients using biopsy forceps, the new device, and their combination, respectively. Cancer detectability of forceps biopsy, the new device, and their combination were 51.3% (61/119), 64.7% (77/119), and 74.8% (89/119), respectively. The new device had a significantly higher sample yield and cancer detectability than biopsy forceps (P < .01 and P = .018, respectively, McNemar test). Complementary use of the new device with biopsy forceps demonstrated a significantly additive effect in both sample yield and cancer detection (P < .01 each, McNemar test). The new device detected 48.3% (28/58) of cancers that were not diagnosed as malignant by biopsy forceps. CONCLUSIONS: The new endoscopic scraper demonstrated a large sample yield and high cancer detectability. It could be a first-line tissue-sampling device for biliary strictures. (University Hospital Medical Information Network Clinical Trial Registry [UMIN-CTR] (http://www.umin.ac.jp/ctr/index.htm) registration number: UMIN000009895.).

Tolvaptan for improvement of hepatic edema: A phase 3, multicenter, randomized, double-blind, placebo-controlled trial.

AIM: Hepatic edema is manifested by ascites, lower limb edema and intolerable symptoms. Some patients insufficiently respond to the conventional diuretic therapy. Therefore, a novel therapeutic option is required. We conducted a phase 3 study to confirm therapeutic effect of tolvaptan on hepatic edema associated with liver cirrhosis. METHODS: In our multicenter, randomized, double-blind, placebo-controlled trial, liver cirrhosis patients who showed insufficient response to conventional diuretics were randomly assigned to 7-day administration of either tolvaptan at 7.5 mg/day or placebo as an add-on therapy to conventional diuretics. The primary outcome was change in bodyweight from baseline. RESULTS: Of 164 eligible patients, 84 were assigned to tolvaptan and 80 to placebo. Change in bodyweight from baseline on the final dosing day was -0.44 kg (standard deviation [SD], 1.93) in the placebo group and -1.95 kg (SD, 1.77) in the tolvaptan group (P < 0.0001). Improvement rates for lower limb edema and ascites-related clinical symptoms were higher with tolvaptan than with placebo. Even in patients with low serum albumin (<2.5 g/dL), decrease in bodyweight was greater with tolvaptan than with placebo (P = 0.0163). In addition, tolvaptan significantly increased serum sodium concentration from baseline. CONCLUSION: Add-on therapy with tolvaptan was effective for the treatment of hepatic edema and ascites-related clinical symptoms. Furthermore, tolvaptan is expected to improve low serum sodium concentration and to exert its effect regardless of serum albumin level. Add-on therapy with tolvaptan is therefore considered to be a novel therapeutic option for hepatic edema.

Dose-finding trial of tolvaptan in liver cirrhosis patients with hepatic edema: A randomized, double-blind, placebo-controlled trial.

AIM: Liver cirrhosis represents the end stage of any chronic liver disease, and it is associated with hepatic edema such as ascites. Many patients with ascites do not respond to diuretic therapy or require administration of diuretics at high doses that can cause adverse events. This 7-day, multicenter, double-blind trial of tolvaptan was designed to determine the optimal dose of tolvaptan for producing the intended pharmacological effect in hepatic edema. METHODS: Liver cirrhosis patients with inadequate diuretic response despite having received a conventional diuretic therapy were enrolled in the trial. Participants were stratified randomly to four groups receiving tolvaptan at 7.5, 15 or 30 mg/day, or placebo as an add-on to conventional diuretics once daily for 7 days. Changes in bodyweight and abdominal circumference were analyzed. Serum sodium concentrations were measured. Safety assessment was performed. RESULTS: Tolvaptan at 7.5-30 mg/day reduced bodyweight and abdominal circumference compared with placebo. Serum sodium concentrations remained within the normal range in all tolvaptan groups. Serious adverse events were not observed, and most common adverse event was thirst. Tolvaptan at 7.5 mg/day showed the maximum change in bodyweight and abdominal circumference together with preferable tolerability. CONCLUSION: Tolvaptan at 7.5 mg/day was considered the optimal dose in liver cirrhosis patients with hepatic edema who showed inadequate response to conventional diuretics.

Solitary metastasis to the intercostal muscle from hepatocellular carcinoma: A case report.

INTRODUCTION: Skeletal muscle metastases from carcinomas, especially to intercostal muscles, are rare. Most metastatic chest wall tumors from hepatocellular carcinoma (HCC) result from disseminations through needle tracts of intrahepatic HCC treatments. PRESENTATION OF CASE: We report the case of a 65-year-old man with chronic viral hepatitis B whose intrahepatic lesions were stabilized by repeated radiofrequency ablations and transcatheter arterial chemoembolization. Follow-up computed tomography demonstrated a well-enhanced mass in the right chest wall. Because α-fetoprotein and des-γ-carboxy prothrombin levels were elevated and no other tumors were detected, we diagnosed the mass as an extrahepatic metastasis from the HCC and resected it along with the surrounding ribs. There was no involvement of the bone, pleura, and lung. DISCUSSION: The tumor was microscopically diagnosed as an intercostal muscle tumor metastasized from HCC, which has not been documented previously. The resection rate of extrahepatic tumors of HCC is low in literature. No other apparent extrahepatic recurrence has been observed for more than 20 months after the surgery. CONCLUSION: We report the case of HCC patient who underwent surgical resection of an intercostal muscle tumor that had metastasized from HCC. Pathological examination of the tumor revealed the tumor cells in the blood vessels, and we speculate it hematogeneous metastasis.

Clinical significance of the genotype and core promoter/pre-core mutations in hepatitis B virus carriers.

It has been shown that clinical and virological characteristics vary among hepatitis B virus (HBV) genotypes. In this study, we measured the virus level, disease severity, and presence or absence of core promoter (CP)/pre-core (PC) mutations in 241 HBV carriers, and investigated the clinical significance of measuring the HBV genotype. In genotype C HBV carriers, the proportion of hepatitis B e antigen (HBeAg)-positive patients was significantly higher than that in genotype B HBV carriers (0 vs. 34.4%, p < 0.05), and the virus level was higher (4.9 vs. 4.05 LGE/ml). In the genotype B HBV carriers, the incidence of PC mutation was significantly higher (69 vs. 34%, p < 0.05). In the genotype C HBV carriers, the incidence of CP mutation was significantly higher (13 vs. 78%, p < 0.05). We compared patients with the wild (W)/mutant (M) pattern in the CP/PC regions to those with the M/W pattern in the CP/PC regions among the genotype C HBV carriers. Both the proportion of HBeAg-positive patients (65.8 vs. 15.4%, p < 0.05) and the alanine aminotransferase (ALT) level (48 vs. 21.5 IU, p < 0.05) were higher in the patients with the M/W pattern in the CP/PC regions, and the disease severity was deteriorated. In conclusion, genotype B HBV may more frequently induce HBe seroconversion via PC mutation compared to genotype C HBV. Among the genotype C HBV carriers, hepatitis activity and the deterioration of the disease severity were significantly inhibited in the group in which PC mutation initially occurred, in comparison to the group in which CP mutation initially occurred.