TEMPORARY REMOVAL: A novel scoring system to predict short-term mortality after living donor liver transplantation for acute liver failure.

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Laparoscopic left hepatectomy in a goat as a training model for laparoscopic anatomic liver resection: results of training courses with a total of 70 goats.

BACKGROUND: To create a suitable animal model for the training of laparoscopic anatomic liver resection, we performed left hepatectomy using a goat and found its suitability. We have since started using goats for wet-lab training and have gradually standardized the relevant procedures. Herein, we report our standardized training procedures using a goat and discuss its feasibility as a novel training model. METHODS: The standardized wet-lab training courses of laparoscopic liver resection conducted on 62 tables with a total of 70 goats were reviewed. The training course began by encircling the hepatoduodenal ligament for the Pringle maneuver, which was repeated during the parenchymal dissection. Following partial liver resection of the left lateral section, left hepatectomy was performed by a standardized procedure for humans in which the liver was split, exposing the entire length of the middle hepatic vein trunk from the dorsal side after extrahepatic transection of the left Glissonean pedicle. If a goat deceased before initiating left hepatectomy, the training was restarted with a new goat. The surgical procedures were performed by surgeons of varying skill levels. RESULTS: A total of 184 surgeons including 10 surgical residents participated in the training. Partial liver resection was initiated in 62 tables, with 8 (13%) dying during or after the procedure of partial liver resection. Subsequently, left hepatectomy was initiated in 61 and completed in 59 tables (98%), regardless of whether the goat survived or deceased, and was not completed in 2 tables (3%) due to time limitation. In 14 tables (23%), the goats deceased during the procedure, however, the procedure was completed. The causes of death were multifactorial, including massive bleeding, reperfusion injury after the Pringle maneuver, and carbon dioxide gas embolism. CONCLUSIONS: Left hepatectomy in a goat is useful as a training model for laparoscopic anatomic liver resection.

Improved Mortality, Morbidity, and Long-Term Outcome After Anatomical Hepatectomy With the Glissonean Pedicle Approach in Patients With Hepatocellular Carcinoma: 30 Years' Experience at a Single Institute.

OBJECTIVE: We evaluated the morbidity and mortality after anatomical hepatectomy with the Glissonean pedicle approach, and long-term outcomes in relation to the morbidity in patients with hepatocellular carcinoma (HCC). SUMMARY BACKGROUND DATA: The mortality, morbidity, and long-term outcomes were evaluated retrospectively. METHODS: A total of 1953 patients with HCC underwent various anatomical hepatectomies with the Glissonean pedicle approach between 1985 and 2014. The mortality (30-day and 90-day) and morbidity (Clavien-Dindo class Ilia or higher) were evaluated among six 5-year eras (1985-1989, 1990-1994, 1995-1999, 2000-2004, 2005-2009, 2010-2014). RESULTS: A total of 460 patients (24%) showed morbidity, and the overall 30-day and 90-day mortality rates were 1.8% and 3.3%, respectively. The 30-day (3.9%, 3.0%, 1.8%, 1.3%, 0.3%, 0.5%: P = 0.0074) and 90-day mortality (6.0%, 4.3%, 3.8%, 2.8%, 2.2%, 1.4%: P = 0.0445) significantly improved over the eras. Blood loss >2 L (odds ratio: 11.808, P = 0.0244) was an independent risk factor for 30-day mortality, and blood loss >2 L (odds ratio: 4.046, P = 0.0271) and bile leakage (odds ratio: 2.122, P = 0.0078) were independent risk factors for 90-day mortality on multivariate analysis. Morbidity was significant independent prognostic factors for overall survival (relative risk: 2.129, P < 0.0001) and recurrence-free survival (relative risk: 1.299, P < 0.0001) in patients with HCC. CONCLUSIONS: Anatomical hepatectomy with the Glissonean pedicle approach was achieved safely in patients with HCC. For more safety and longer survival, blood loss, bile leakage, and morbidity should be reduced. Longterm outcomes after anatomical hepatectomy with the Glissonean pedicle approach in patients with HCC have been improved over 30 years with gradually less mortality and morbidity due to decreases in blood loss >2 L and bile leakage.

Primary hyperoxaluria complicated with liver cirrhosis: A case report.

Primary hyperoxaluria (PH) is a rare, autosomal recessive disorder characterized by overproduction of oxalate caused by a deficiency in a hepatic enzyme. The excess oxalate combines with calcium in the kidneys to form deposits of calcium oxalate, which can lead to nephrocalcinosis and renal failure. PH type 1 (PH1), the most common form of this disease, is caused by a deficiency of the liver-specific enzyme alanine/glyoxylate aminotransferase (AGT). Liver transplantation is performed as a definitive therapy for PH to correct the enzyme defect. Usually, liver depositions are limited and liver function is normal without fibrosis. Here, we report an adult case of liver cirrhosis caused by PH1. A 28-year-old woman was admitted to our hospital under suspicion of PH1 and the presence of nephrocalcinosis. The patient had suffered from kidney stone recurrences from 17 years of age, and was initiated on hemodialysis due to renal failure at the age of 27 years. The serum level of oxalic acid was high, whereas the AGT level in the liver tissue was decreased. Thus, the patient was definitively diagnosed with PH1. Although she had normal liver function, surface nodularity and splenomegaly were detected by computed tomography, suggesting liver cirrhosis. The native liver showed micronodular cirrhosis and portal fibrosis. Several arterioles were filled with rhomboid and polyhedral refractile oxalate crystals and various portal tracts showed these crystals. Our case suggests that long-term oxalosis can lead to liver cirrhosis; thus, PH should be considered one of the causes of liver cirrhosis.

Phase I clinical trial of multiple-peptide vaccination for patients with advanced biliary tract cancer.

BACKGROUND: The prognosis of patients with advanced biliary tract cancer (BTC) is extremely poor and only a few standard treatments are available for this condition. We performed a phase I trial to investigate the safety, immune response and anti-tumor effect of vaccination with three peptides derived from cancer-testis antigens. METHODS: This study was conducted as a phase I trial. Nine patients with advanced BTC who had unresectable tumors and were refractory to standard chemotherapy were enrolled. Three HLA-A*2402 restricted epitope peptides-cell division cycle associated 1 (CDCA1), cadherin 3 (CDH3) and kinesin family member 20A (KIF20A)-were administered subcutaneously, and the adverse events and immune response were assessed. The clinical effects observed were the tumor response, progression-free survival (PFS) and overall survival (OS). RESULTS: The three-peptide vaccination was well-tolerated up to a dose of 3 mg per peptide (9 mg total). No grade 3 or 4 adverse events were observed after vaccination. Peptide-specific T cell immune responses were observed in all patients and stable disease was observed in 5 of 9 patients. The median PFS and OS were 3.4 and 9.7 months. The Grade 2 injection site reaction and continuous vaccination after PD judgment appeared to be prognostic of OS. CONCLUSIONS: Multiple-peptide vaccination was well tolerated and induced peptide-specific T-cell responses. TRIAL REGISTRATION: This study was registered with the University Hospital Medical Information Network Clinical Trials Registry (UMIN-CTR000003229).

Long-term survival of patients with cholangiolocellular carcinoma after curative hepatectomy.

BACKGROUND: Cholangiolocellular carcinoma (CoCC) has distinct pathological characteristics, and CoCC is considered to originate from hepatic progenitor or stem cells. However, the surgical outcome of CoCC has not been clarified in detail. METHODS: We retrospectively studied 275 patients with intrahepatic cholangiocarcinoma (ICC) who underwent hepatectomy between 1990 and 2011. Surgical outcomes were compared between 29 patients with CoCC and 130 patients with mass-forming (MF) type ICC since all patients with CoCC showed MF type on macroscopic findings. RESULTS: The number of patients with chronic liver disease was significantly higher in the CoCC group than in the ICC group. The number of patients with abnormal levels of CA19-9 was significantly lower in the CoCC group than in the ICC group. Portal vein invasion and intrahepatic metastasis were significantly lower in patients with CoCC group than in the ICC group. In the CoCC group, 15 of 28 patients survived for more than 5 years after curative surgery whereas 15 of 102 patients with ICC survived for more than 5 years after curative surgery. The 5-year survival rate was significantly higher in patients with CoCC (75 %) than in patients with ICC (33 %, p = 0.0005). Multivariate analysis showed CoCC, absence of portal vein invasion or hepatic vein invasion, and absence of intrahepatic metastasis to be significant independent prognostic factors for overall survival in patients with MF-type ICC and CoCC. CONCLUSIONS: CoCC is rare, but patients with CoCC had special characteristics with favorable long-term survival due to its less invasive histopathologic characteristics.

[Multiple large hepatocellular adenomas in a patient with glycogen storage disease type Ia].

A man diagnosed at birth with glycogen storage disease type Ia was found to have multiple hepatocellular adenomas at 15 years of age. At 18 years of age, he underwent transarterial tumor embolization in segments 4 and 5. At 27 years of age, the tumor in segment 4 had increased in size on follow-up computed tomography, and he was referred to our hospital. Because the tumor was large, increasing in size, and we could not exclude malignancy, we performed resection of segments 4 and 8 of the liver and partial resection of segment 5 for excisional biopsy. The pathological diagnosis was multiple inflammatory hepatocellular adenomas.

Evaluation of two-stage hepatectomy using portal vein embolization for colorectal liver metastasis: a retrospective nationwide cohort survey in Japan.

INTRODUCTION: Two-stage hepatectomy (TSH) enables patients to undergo surgery for colorectal liver metastasis (CRLM) which one-stage hepatectomy cannot remove. Although the outcome of TSH has been reported, there is no original report from Japan. The aim of this retrospective study was to evaluate the outcome of TSH in Japanese patients with CRLM. METHODS: We conducted a retrospective cohort study using the nationwide database that included clinical information of 12,519 patients treated with CRLM between 2005 and 2017 in Japan. The primary outcome measure was overall survival. The second outcome measure was progression-free survival. Fisher's exact test, chi-squared test and Mann-Whitney U test were conducted to examine an intergroup difference. Univariate and multivariate analyses were performed using Cox regression model. Survival analysis was performed by Kaplan-Meier method and log-rank test. RESULTS: Of the database, 53 patients undergoing TSH using portal vein embolization (PVE) were identified and analyzed. Their morbidity and in-hospital mortality rate at the second hepatectomy were 26.4% and 0.0%. The mean observation period was 21.8 months. The estimated 1-, 3- and 5-year overall survival rate were 92.5%, 70.8% and 34.7%. Multivariate analyses showed that more than 10 liver nodules significantly increased the mortality risk by 4.2-fold (95%CI 1.224-14.99, P= 0.023). Survival analysis revealed that repeat hepatectomy for disease progression after TSH was superior to chemotherapy in overall survival (mean: 49.6 vs. 18.7, months, P= 0.004). CONCLUSION: In the Japanese cohort, TSH was confirmed to be a safety procedure with acceptable survival outcome. More than 10 liver nodules may be a predictor for unfavorable outcome of patients with CRLM undergoing TSH. Furthermore, repeat hepatectomy can be a salvage treatment for resectable intrahepatic recurrence after TSH.

Impact of hepatectomy for huge solitary hepatocellular carcinoma.

BACKGROUND: To evaluate the surgical outcome of patients with huge solitary hepatocellular carcinoma (HCC, ≥10 cm in diameter) without major vascular invasion. METHODS: We retrospectively studied 177 patients with huge HCC (≥10 cm in diameter) who underwent hepatectomy from 1990 to 2008. Surgical outcomes and clinicopathological characteristics were compared among 54 patients with solitary HCC without a major portal vein tumor thrombus (MPVT), 53 patients with multiple HCCs without MPVT, and 70 patients who have HCC with MPVT. RESULTS: The mean tumor size and indocyanine green retention rate at 15 min showed no significant difference among patients groups. The overall 5-year survival rate was significantly higher in patients with solitary HCC (79%) than in patients with multiple HCCs (31%, P < 0.0001) and MPVT (17%, P < 0.0001). In patients who underwent curative surgery, the 5-year disease-free survival rate was significantly higher in patients with solitary HCC (48%) than in patients with multiple HCCs (12%, P = 0.0008) and MPVT (12%, P = 0.0003). Multivariate analysis showed solitary HCC to be a significant independent prognostic factor for overall survival and disease-free survival. CONCLUSIONS: Patients with solitary HCC without MPVT show a favorable surgical outcome even for tumor size ≥10 cm in diameter.

The Outcome of Salvage Liver Transplantation and Liver Resection for Recurrent Hepatocellular Carcinoma Using the 5-5-500 Rule, Japanese Extended Liver Transplantation Criteria for Hepatocellular Carcinoma.

BACKGROUND: Liver transplantation (LT) for hepatocellular carcinoma (HCC) is limited to Child-Pugh class C patients according to the Japanese HCC treatment algorithm. However, extended criteria of LT for HCC, known as the 5-5-500 rule, were published in 2019. Hepatocellular carcinoma reportedly has a high recurrence rate after primary treatment. We hypothesized that the outcome of recurrent HCC would be improved if the 5-5-500 rule were adopted for patients with recurrent HCC. We, therefore, analyzed the outcomes of surgical treatment (liver resection [LR] and LT) for recurrent HCC using the 5-5-500 rule in our institute. METHODS: Fifty-two patients younger than 70 years of age received surgical treatment for recurrent HCC using our institute's 5-5-500 rule from 2010 to 2019. We divided these patients into the LR and LT groups in the first study. The 10-year overall survival and re-recurrence-free survival were analyzed. The second study analyzed the risk factors of re-recurrence after surgical treatment for recurrent HCC. RESULTS: In the first study, the background characteristics of the 2 groups (LR and LT) showed no significant difference, except for age and Child-Pugh classification. There was no significant difference in the overall survival between groups (P = .35), but the re-recurrence-free survival in the LR group was significantly shorter than that in the LT group (P < .01). In the second study, the male sex and LR were risk factors of re-recurrence after surgical treatment for recurrent HCC. Child-Pugh's class did not contribute to re-recurrence. CONCLUSIONS: To improve the outcomes of recurrent HCC, LT is the better choice, regardless of the Child-Pugh class.

Comprehensive data of 4502 patients newly diagnosed with colorectal liver metastasis between 2015 and 2017, and prognostic data of 2427 patients newly diagnosed with colorectal liver metastasis in 2013 and 2014: Third report of a nationwide survey in Japan.

To improve treatment outcomes in patients with colorectal liver metastasis (CRLM), the Joint Committee for Nationwide Survey on CRLM was established by the Japanese Society for Cancer of the Colon and Rectum and the Japanese Society of Hepato-Biliary-Pancreatic Surgery. The aim of the study was to evaluate transition in the characteristics and treatment strategy in CRLM patients and analyze prognostic factors using large-scale data. The present study summarizes the data of patients newly diagnosed between 2015 and 2017 and presents prognostic data of patients newly diagnosed in 2013 and 2014. Survival curves were generated by the Kaplan-Meier method and compared by log-rank test. Multivariate analyses were carried out using Cox proportional hazard modeling. The data of 4502 patients newly diagnosed with CRLM between 2015 and 2017 and the prognostic data of 2427 patients diagnosed in 2013 and 2014 are included. Regarding the 2013 and 2014 prognostic data, the 5-year overall survival (OS) rates of patients who underwent hepatectomy alone was 59.8%. Multivariate analyses identified age at diagnosis of CRLM ≥70 years, concomitant extrahepatic metastasis at diagnosis of CRLM, tumor depth of primary lesion ≥subserosa/pericolic or perirectal tissue, mutant KRAS status, number of CRLM ≥5, maximum diameter of CRLM >5 cm, and surgical curability R1/R2 as independent predictors of OS. Analysis of the latest nationwide database of patients diagnosed with CRLM revealed changes in patients and oncological characteristics, a transition in treatment strategy, and different independent prognosticators to those reported previously.

Validation study of the JSHBPS nomogram for patients with colorectal liver metastases who underwent hepatic resection in the recent era - a nationwide survey in Japan.

BACKGROUND: The Japanese Society of Hepato-Biliary-Pancreatic Surgery (JSHBPS) nomogram was developed to predict disease-free survival in patients with colorectal liver metastases (CRLM) undergoing upfront hepatectomy. However, the utility of the nomogram in patients with resected CRLM remains unknown in the current situation in which treatment strategies are changing with advances in drugs. METHODS: Patients in the initial nomogram cohort (n = 727) and validation cohort (n = 2225) were divided into the upfront hepatectomy and preoperative chemotherapy groups. The nomogram was validated by measuring calibration and discrimination in the two cohorts. Calibration curves were plotted, and survival probabilities were compared. Finally, to quantify the discrimination power, we estimated the concordance index (C-index). RESULTS: In the upfront hepatectomy group, the C-index was 0.63, the suitable cutoff value of the Beppu score was 7, and adjuvant chemotherapy was significantly effective limited to high-risk patients (Beppu score ≥7). The C-index was 0.56 in the preoperative chemotherapy group. CONCLUSIONS: The JSHBPS nomogram remains beneficial for patients undergoing upfront hepatectomy in the recent era but is less effective for patients undergoing hepatectomy after chemotherapy. Patients with a Beppu score ≥7 showed high-risk recurrence, and adjuvant chemotherapy should be recommended for these patients.

Combined hepatocellular-cholangiocarcinoma had poor outcomes after hepatectomy regardless of Allen and Lisa class or the predominance of intrahepatic cholangiocarcinoma cells within the tumor.

BACKGROUND: We evaluated the surgical outcomes of combined hepatocellular-cholangiocarcinoma (CHC) in relation to Allen and Lisa class and the predominance of intrahepatic cholangiocarcinoma (ICC) cells within the tumor. METHODS: We retrospectively studied 44 patients with CHC who underwent hepatectomy between 1987 and 2010. Clinicopathologic characteristics and surgical outcomes were compared in relation to Allen and Lisa class (33 combined type, 11 mixed type) and the predominance of ICC cells within the tumor (cases in which ICC cells accounted for 50% or less of all cells within the tumor, n = 22; cases in which ICC cells accounted for more than 50%, n = 22). RESULTS: The survival rate for 42 patients with CHC who underwent curative surgery was 24% at 5 years, and median survival was 15.4 months. According to Allen and Lisa class, the number of cases with portal invasion was far higher in patients with the mixed type than in patients with the combined type. The 5-year survival rates of patients with the combined type and mixed type who underwent curative surgery were 27% and 13%, respectively, showing no statistically significant difference. According to the predominance of ICC cells, there was no difference in clinicopathologic findings between the groups. The 5-year survival rates of patients with ≤50% and >50% ICC cells within the tumor who underwent curative surgery were 21% and 26%, respectively, showing no statistically significant difference. CONCLUSIONS: CHC had poor outcomes after hepatectomy regardless of Allen and Lisa class or the predominance of ICC cells within the tumor.

[Induction of human dendritic cells from peripheral blood mononuclear cells by using a HydroCellTM].

Cancer immunotherapy using dendritic cells (DCs) is widely known to produce an anticancer effects. However, there is no approved DC vaccine in Japan, possibly because the culture and harvesting of DC is complicated. To further enable DC vaccine therapy, an easier, safer, and inexpensive culture system is needed, and we therefore aimed to build an automated DC culture system. In a previous study using a HydroCellTM, we successfully cultured and harvested DCs from peripheral blood monocytes without using enzymes, and showed that HydroCellTM is a useful tool for automated DC culture. In this study, we performed a similar process of DC culture from peripheral blood mononuclear cells and demonstrated the safe and easy culturing and harvesting of DCs. Furthermore, CD83-positive mature DCs were increased compared to the usual plate method. These results demonstrate the utility of HydroCellTM as an automated DC culture system.

Left hepatectomy with suprahepatic inferior vena cava resection and reconstruction under veno-arterial extracorporeal membrane oxygenation for intrahepatic cholangiocarcinoma: a case report.

BACKGROUND: Curative surgery is the most effective treatment for intrahepatic cholangiocarcinoma (ICC). When an ICC involves the suprahepatic inferior vena cava (IVC), hepatectomy with suprahepatic IVC resection and reconstruction is challenging. For reconstruction of the suprahepatic IVC, total hepatic vascular exclusion (THVE), veno-venous bypass, and/or in situ hypothermic portal perfusion are required, but mortality and morbidity remain high. CASE PRESENTATION: We present a 73-year-old woman with mass-forming ICC which invaded the suprahepatic IVC and middle hepatic vein. Left hepatectomy, suprahepatic IVC resection, and reconstruction with an artificial graft were successfully performed during veno-arterial extracorporeal membrane oxygenation (V-A ECMO) to maintain blood pressure. While clamping the IVC diagonally, the right hepatic vein confluence could be preserved. No congestion in the right liver was seen; therefore, there was no requirement for the Pringle maneuver or THVE during reconstruction. No morbidity or mortality was seen after surgery. CONCLUSIONS: Hepatectomy with suprahepatic IVC resection and reconstruction under V-A ECMO can be performed safely. When an ICC invades the suprahepatic IVC, V-A ECMO during resection and reconstruction of the suprahepatic IVC with an artificial graft is recommended as one of the options.

Intrahepatic Cholangiocarcinoma With Neither Intrahepatic Metastasis Nor Lymph Node Metastasis Is the Optimal Indication for Hepatectomy With Adjuvant Therapy.

Background/Aim: The optimal indication of hepatectomy with adjuvant therapy for intrahepatic cholangiocarcinoma (ICC) has not been evaluated in detail. Patients and Methods: We retrospectively studied 224 patients with ICC who underwent hepatectomy between 2000 and 2019. Prognostic factors for overall survival (OS) were evaluated by univariate and multivariate analysis. A total of 127 patients were treated with adjuvant therapy (62 patients with chemotherapy and 65 patients with immunotherapy) after hepatectomy, and 97 patients were treated with hepatectomy alone. Results: Intrahepatic metastasis (IM), lymph node metastasis (LNM) of ICC, adjuvant chemotherapy, and adjuvant immunotherapy were significant prognostic factors for OS on multivariate analysis. In 127 patients with neither IM nor LNM, the 5-year OS rate was significantly higher in 36 patients with adjuvant chemotherapy (81%) and in 34 patients with adjuvant immunotherapy (68%) than in 57 patients with hepatectomy alone (45%). Conclusion: The absence of IM or LNM is the optimal indication for hepatectomy with adjuvant therapy in patients with ICC.

Mass-forming intrahepatic cholangiocarcinoma with marked enhancement on arterial-phase computed tomography reflects favorable surgical outcomes.

BACKGROUND: Outcomes after hepatectomy in patients with mass-forming (MF) type intrahepatic cholangiocarcinoma (ICC) with marked enhancement within the tumor on arterial-phase computed tomography (CT) scans have not been clarified in detail. METHODS: We retrospectively studied 140 patients with MF type ICC who underwent hepatectomy from 1989 through 2008. Surgical outcomes were compared between 25 patients with MF type ICC with marked enhancement within the tumor (hypervascular ICC) and 109 patients without enhancement within the tumor (hypovascular ICC) on arterial-phase CT scans. RESULTS: Portal invasion and intrahepatic metastasis were significantly lower in patients with hypervascular ICC than in those with hypovascular ICC. The 5-year survival rate was significantly higher in patients with hypervascular ICC (86%) than in patients with hypovascular ICC (27%, P < 0.0001). Multivariate analysis showed hypervascular ICC on arterial-phase CT scans, normal level of cancer-associated carbohydrate antigen 19-9, absence of portal invasion, and absence of intrahepatic metastasis of ICC to be significant independent prognostic factors for overall survival in patients with MF type ICC. CONCLUSIONS: MF type ICC with marked enhancement within the tumor on arterial CT scans showed a favorable surgical outcome due to its less invasive histopathologic characteristics in patients with MF type ICC.

[In vitro culture of human dendritic cells by using a HydroCell™].

Cancer Immunotherapy using dendritic cells would be a feasible and useful tool for cancer treatment. However, no immunotherapy has been approved in Japan because of a lack of any randomized clinical studies. We are now trying to develop an automatic dendritic cell culture system in order to perform a large-scale randomized clinical trial. In this study, we investigated the utility of a HydroCell™ for in vitro culture of human dendritic cells induced from peripheral blood monocytes. The dendritic cells grew one and a half times when they were cultured in a HydroCell™. All the cells were floating and harvested easily without any enzymes. The cells expressed the CD80 and CD83 molecules on their surface and still had strong phagocytosis. This results demonstrated that a HydroCell™ was a useful tool for in vitro culture of dendritic cells.

A case of hemorrhage of hepatocellular carcinoma resembling a hepatic cyst arising from non-cirrhotic steatohepatitis.

A 70-year-old female was found to have multiple hepatic cysts at her annual checkup. In the posterior segment of the right lobe of the liver, an 81 × 67 mm circular cystic lesion was detected by contrast-enhanced computed tomography (CT). Magnetic resonance imaging (MRI) of the cyst revealed a solid component. The cyst had a capsule-like structure and non-uniform fluid accumulation suggested bleeding. Since the lesion was enlarged and malignancy could not be ruled out, it was surgically resected. Histopathologically, reticular fibers of the liver were seen in necrotic tissue and the lesion was diagnosed as a bleeding hepatocellular carcinoma (HCC). The non-cancerous liver tissue showed non-cirrhotic steatohepatitis. This was an unusual presentation of HCC.

The utility of liver transplantation to treat acute liver failure caused by adult-onset Still's disease: case reports.

PURPOSE: Adult-onset Still's disease (AOSD) is an inflammatory condition commonly complicated by mild liver dysfunction. However, severe liver failure is rarely reported. We report three cases of severe acute hepatic failure (ALF) associated with AOSD. We encountered three cases of acute liver failure (ALF) with encephalopathy. RESULTS: Case 1 was a 75-year-old female, who was started on a steroid (prednisolone, PSL) to treat AOSD; this was gradually tapered. Two months later, severe ALF developed. She died despite an increase in the PSL dose and artificial liver support. Case 2 was a 26-year-old-female taking PSL 30 mg/day to treat subacute thyroiditis. PSL was tapered, and she received methyl PSL pulse therapy and artificial liver support, but this did not cure the ALF. Liver transplantation (LT) was performed 25 days later. Three years later, the same symptoms were observed and we diagnosed AOSD. Case 3 was a 56-year-old-female who met the AOSD criteria. PSL 50 mg/day was started and then tapered. Methyl PSL pulse therapy was prescribed to treat hemophagocytic syndrome, but she required LT on hospital day 13. CONCLUSION: In AOSD cases, ALF is rarely complicated; urgent LT should be considered only for patients with AOSD-related severe ALF.