Early-onset diabetes with low utilization of lipid as an energy source carrying a rare missense mutation in the CEL gene.

Summary: Carboxyl ester lipase (CEL) is a major component of pancreatic juice and is responsible for the duodenal hydrolysis of cholesteryl esters. Maturity-onset diabetes of the young (MODY) is a form of diabetes mellitus characterized by early onset and dominant inheritance of beta-cell dysfunction. CEL gene mutations cause the type of MODY denoted as MODY8.

Association of Metabolic-Associated Steatotic Liver Disease With Clinical Outcomes of Durvalumab and Tremelimumab in Advanced Hepatocellular Carcinoma Patients: A Multicenter Study.

Aim: This study aimed to evaluate the association between the efficacy of durvalumab plus tremelimumab (Dur/Tre) and metabolic dysfunction-associated steatotic liver disease (MASLD) in patients with hepatocellular carcinoma (HCC).

Methods: A total of 239 patients with HCC who received Dur/Tre between March 2023 and October 2024 at multiple institutions in Japan were retrospectively analyzed. Patients were categorized into three groups based on underlying liver disease: viral-related HCC (n = 126), MASLD-related HCC (n = 30), and nonviral, non-MASLD HCC (n = 83).

A novel approach to evaluate the therapeutic efficacy of durvalumab and tremelimumab combination therapy in hepatocellular carcinoma.

Aim: This study aimed to evaluate the efficacy of durvalumab + tremelimumab (Dur + Tre) in real-world clinical practice, characterize "stable disease (SD)," and identify SD responders (SD-R) who benefit from Dur + Tre treatment.

Methods: This multicenter observational study included 212 patients with unresectable hepatocellular carcinoma (u-HCC) treated with Dur + Tre between March 2023 and November 2024. The patients were categorized into 95 first-line and 117 later-line cases, respectively.

Induction hepatic arterial infusion chemotherapy followed by surgery for hepatocellular carcinoma with massive portal vein tumor thrombosis: a case series of 20 patients.

Background: The prognosis of hepatocellular carcinoma with portal vein tumor thrombosis is very poor, and the optimal treatment remains controversial. The aim of this study is to examine the safety and feasibility of our multimodal treatment.

Methods: This was a single-institution, retrospective case series.

Antitumor effects and immune-mediated adverse events of durvalumab plus tremelimumab treatment for unresectable hepatocellular carcinoma.

Aim: Durvalumab plus tremelimumab (Dur/Tre) is a first-line systemic treatment option for unresectable hepatocellular carcinoma (uHCC). However, the management of severe immune-mediated adverse events (imAEs) is challenging. Therefore, we investigated the relationship between severe imAEs and antitumor responses in patients with uHCC treated with Dur/Tre.

Efficacy of Lenvatinib and Atezolizumab Bevacizumab Combination Therapy in Patients With Combined Hepatocellular-cholangiocarcinoma.

Background/aim: Surgical resection remains the only curative treatment for combined hepatocellular-cholangiocarcinoma; however, systemic therapy is the primary treatment option for unresectable cases. This multicenter retrospective study aimed to assess the efficacy of systemic therapy for unresectable combined hepatocellular-cholangiocarcinoma.

Patients And Methods: Twenty-one patients with histologically confirmed or clinically diagnosed combined hepatocellular-cholangiocarcinoma who received systemic therapy were included.

The benefit of conversion therapy for patients with unresectable hepatocellular carcinoma receiving atezolizumab plus bevacizumab using newly established oncological resectability criteria in Japan.

Background: This study aimed to investigate the changes in oncological resection status in patients with unresectable hepatocellular carcinoma (u-HCC) receiving atezolizumab plus bevacizumab (Atez/Bev) and the impact of conversion therapy following Atez/Bev treatment.

Methods: This cohort included 631 patients with u-HCC treated with Atez/Bev. Tumors were assessed using oncological resectability criteria and categorized as borderline resectable 1 (BR1, n=166) or borderline resectable 2 (BR2, n=465).

Combination Assay of Methylated with Tumor Markers Shows High Sensitivity for Detection of Early-Stage Hepatocellular Carcinoma.

Introduction: Patients with hepatitis virus-related hepatocellular carcinoma (viral HCC) are decreasing as hepatitis control improves, but those with non-viral-related HCC (non-viral HCC) are increasing in Japan. No established surveillance system exists for patients with non-viral HCC, so they are often diagnosed at an advanced stage. To address this, we performed this study.

Varying Impact of Lenvatinib or Sorafenib Therapy on Skeletal Muscle Loss in Patients with Hepatocellular Carcinoma.

Introduction: Lenvatinib and sorafenib are key therapeutic agents for hepatocellular carcinoma (HCC). However, there are no useful biomarkers for selecting molecular-targeted agents (MTAs). Skeletal muscle volume is associated with the clinical outcomes in these patients.

Methylated SEPT9 assay-based liquid biopsy as a biomarker in molecular targeted agent-treated hepatocellular carcinoma.

Background: The development of molecular targeted agents (MTAs) has changed the treatment strategy for hepatocellular carcinoma (HCC). However, currently, there are no established predictive biomarkers for the treatment efficacy of MTAs. Previously, we developed a novel liquid biopsy test for HCC screening using sensitive methylated DNA testing of septin 9 gene (SEPT9).

Impact of skeletal muscle volume on patients with BCLC stage-B hepatocellular carcinoma undergoing sorafenib therapy.

Aim: Skeletal muscle volume has been reported to be an important factor that determines overall survival (OS) and post-progression survival (PPS) in patients with hepatocellular carcinoma (HCC). However, the impact of skeletal muscle volume on HCC with Barcelona Clinic Liver Cancer (BCLC) stage B (BCLC-B) remains unclear. We conducted sub-analyses of a previous study on BCLC-B and compared our findings with data on HCC with BCLC stage C (BCLC-C).

Double cancer of primary hepatic angiosarcoma and hepatocellular carcinoma treated with atezolizumab plus bevacizumab.

Aim: Primary hepatic angiosarcoma (PHA) is extremely rare, and its imaging findings are similar to those of other liver tumors including hepatocellular carcinoma (HCC). Here, we report a case of hepatitis C virus (HCV)-related HCC followed by PHA that showed remarkable clinical response to atezolizumab plus bevacizumab (Atezo/Bev) therapy.

Case Presentation: A 78-year-old man with recurrent HCC had a liver tumor with lymphadenopathy.

A combination of liver stiffness and international normalized ratio is an ideal prognostic predictor of portosystemic shunt occlusion in patients with portal hypertension.

Background: We previously reported liver stiffness (LS) as a prognostic predictor of portosystemic shunt (PSS) occlusion. This study aims to reinvestigate the predictive factors of the model for end-stage liver disease-sodium (MELD-Na) score amelioration following balloon-occluded retrograde transvenous obliteration (BRTO) and to evaluate the postoperative prognoses of patients with portal hypertension by using newly identified factors.

Methods: Seventy-five patients who underwent BRTO between 2008 and 2021 were retrospectively enrolled.

Management of Systemic Therapies and Hepatic Arterial Infusion Chemotherapy in Patients with Advanced Hepatocellular Carcinoma Based on Sarcopenia Assessment.

Background: Sarcopenia, defined as the loss of skeletal muscle mass (MM), physical performance, and strength, has been associated with poor clinical outcomes in hepatocellular carcinoma (HCC) patients treated with several therapies. As systemic therapies, including molecular targeted agents, have a strong impact on sarcopenia, we aimed to review the impact of sarcopenia in patients receiving systemic therapies, especially sorafenib and hepatic arterial infusion chemotherapy (HAIC).

Summary: Several studies have demonstrated that sarcopenia is associated with poor clinical outcomes in patients receiving sorafenib or lenvatinib, while HAIC has no association with overall survival (OS) and sarcopenia.

Screening for portopulmonary hypertension using computed tomography-based measurements of the main pulmonary artery and ascending aorta diameters in patients with portal hypertension.

Aim: This study aimed to demonstrate the feasibility of identifying candidates of portopulmonary hypertension (PoPH) from general portal hypertension patients based on chest computed tomography (CT) results.

Methods: One hundred and thirty patients with portal hypertension who had undergone interventional radiology therapies at our hospital between August 2011 and July 2021 were included, and preoperative clinical data were collected. Suspicious PoPH was defined as main pulmonary artery diameter (mPA-D) ≥ 29 mm or the ratio of mPA-D to ascending aorta diameter (mPA-D/aAo-D) ≥ 1.

Skeletal Muscle Volume Is an Independent Predictor of Survival after Sorafenib Treatment Failure for Hepatocellular Carcinoma.

Few studies exist on the relationship between post-progression survival (PPS) and skeletal muscle volume in hepatocellular carcinoma (HCC) patients receiving sorafenib. This study aimed to analyze the effects of muscle volume on clinical outcomes. We retrospectively enrolled 356 HCC patients.

Splenic non-infarction volume determines a clinically significant hepatic venous pressure gradient response to partial splenic embolization in patients with cirrhosis and hypersplenism.

Background: This study aimed to investigate changes in the hepatic venous pressure gradient (HVPG) by partial splenic embolization (PSE) and to identify the determinants of a clinically meaningful postoperative HVPG reduction.

Methods: Sixty-eight patients with cirrhosis and hypersplenism who underwent PSE at our department between September 2007 and June 2020 were included. The HVPG was evaluated pre- and immediately post-PSE.

Albumin-bilirubin score as a useful predictor of energy malnutrition in patients with hepatocellular carcinoma.

Background & Aims: Chronic liver diseases, including hepatocellular carcinoma (HCC), lead to an imbalance in energy metabolism. The non-protein respiratory quotient (npRQ), which estimates energy malnutrition, can be evaluated using an indirect calorimeter; however, npRQ measurement is limited in routine work. This study aimed to investigate the relationship between the albumin-bilirubin (ALBI) score and npRQ in patients with HCC.

Short-term Effects of Hepatic Arterial Buffer Responses Induced by Partial Splenic Embolization on the Hepatic Function of Patients with Cirrhosis According to the Child-Pugh Classification.

Objective This study primarily aimed to investigate the short-term effects of partial splenic embolization (PSE) on the Child-Pugh score and identify predictive factors for changes in the score caused by PSE. The secondary aim was to analyze changes in various parameters at one month postoperatively using these identified factors. Methods Between September 2007 and December 2019, 118 patients with cirrhosis and hypersplenism underwent PSE at our hospital.