Extrahepatic Portal Research Articles

S4892 Isolated Jejunal Varices: A Hidden Source of Gastrointestinal Bleeding in Patient With Extrahepatic Portal Hypertension and Altered Anatomy

S4892 Isolated Jejunal Varices: A Hidden Source of Gastrointestinal Bleeding in Patient With Extrahepatic Portal Hypertension and Altered Anatomy

Medium-to Long-term Outcomes of Rex Shunt in 105 Children With Extrahepatic Portal Vein Obstruction in China

PurposeThe aim of our study was to analyze the medium-to long-term outcomes of Rex shunts in a large series of children with extrahepatic portal vein obstruction (EHPVO). MethodsThe clinical data of 105 children aged between 6 months and 16 years with EHPVO who underwent Rex shunt between October 2014 and June 2021 at our center were retrospectively reviewed after more than 2 years of follow-up. ResultsThe overall patency rate of the Rex shunt was 91.43% (96/105) during a median follow-up of 41 months (range, 24–98 months). Eighty-seven (82.86%) of the 105 patients underwent classical Rex shunt with internal jugular vein (IJV) bypass, and the remaining 18 patients (17.14%) underwent modified Rex shunt with intra-abdominal vein bypass. Patients with a patent shunt experienced portal hypertension resolution, which was characterized by a reduction in portal pressure, disappearance of variceal bleeding, relief of gastroesophageal varices, and relief of splenomegaly or hypersplenism. The rate of Rex shunt thrombosis in our center was 8.57% (9/105), and a repeat Rex shunt was effective for the treatment of graft thrombosis. Anastomotic stenosis occurred in 14.26% (15/105) of the children, 38.46% (5/13) of whom received successful endovascular intervention therapy and experienced remission of portal hypertension symptoms. The patency rate of the classical Rex shunt was higher than that of the modified Rex shunt (97.70% vs. 61.11%), whereas the rate of vascular complications, including anastomotic stenosis and graft thrombosis, of the classical Rex shunt was lower than that of the modified Rex shunt (11.49% vs. 77.78%). Further comparison revealed that the risk of vascular complications was substantially greater in the modified Rex shunt group than in the classical Rex shunt group in the nonadjusted model, minimally adjusted model, and fully adjusted model (RR ranged from 6.77 to 7.07, all p < 0.001). ConclusionsThe Rex shunt provides medium-to long-term benefits for children with EHPVO. The classical Rex shunt with IJV bypass provides the best patency rate and the fewest vascular complications. Levels of EvidenceⅢ Type of StudyRetrospective comparative study.

Spontaneous Non-Traumatic Splenic Rupture in an Adult Patient with Extrahepatic Portal Venous Obstruction (EHPVO) – A Case Report with Review of Literature

Spontaneous Non-Traumatic Splenic Rupture in an Adult Patient with Extrahepatic Portal Venous Obstruction (EHPVO) – A Case Report with Review of Literature

Choledochal Cyst with Pancreas Divisum – Surgical Insight into A Rare Association

&#x0D; &#x0D; &#x0D; &#x0D; Congenital pancreaticobiliary anomalies like pancreatic divisum (PD), choledochal cysts (CDC), anomalous pancreaticobiliary ductal union (APBDU) have been reported in 5.7% of patients undergoing magnetic resonance cholangiopancreatography. CDC’s are characterized by abnormal dilatations of the intrahepatic and/or extrahepatic portion of the biliary tree and can be complicated by cystolithiasis, cholangitis, pancreatitis and malignant transformation necessitating surgical managemnet. While CDCs are commonly associated with APBDU, combination of CDC with PD is rare and a potential surgical challenge. We report a case of recurrent cholangitis in a patient with CDC and coincidental classic PD and illustrate how preoperative identification, fastidious dissection technique to safeguard both the pancreatic ducts and simple intraoperative preemptive strategies may decrease consequences of distal stump blowout. PD, pancreatic divisum; CBD, common bile duct; CDC, choledochal cysts; APBDU, anomalous pancreaticobiliary ductal union; MRCP, magnetic resonance cholangiopancreatography; DOS, Duct of Santorini; DOW, Duct of Wirsung; PTBD, percutaneous transhepatic biliary drainage; RYHJ, roux en Y hepaticojejunostomy.&#x0D; &#x0D; &#x0D; &#x0D;

Phase I/II clinical trial of regorafenib plus durvalumab (MEDI4736) in patients with chemotherapy-refractory advanced biliary tract cancers.

TPS581 Background: Biliary tract cancers (BTC) are a heterogeneous group of cancers affecting the epithelial lining of the intra- and extrahepatic portions of the biliary tree, ranking as the second most prevalent primary liver cancer after hepatocellular carcinoma. A subgroup of BTC was found to have an abundant tumor specific neoantigen expression, enriched expression of immune related genes and genes regulating inhibitory immune checkpoint proteins. Recent advancements in BTC treatment include the TOPZ-1 trial, which established the survival advantage of adding durvalumab to the gemzar + cisplatin regimen with an estimated 24-month overall survival rate of 24.9% versus 10.4% for placebo. The hazard ratio for progression-free survival favored durvalumab at 0.75 (95% CI, 0.63 to 0.89; P=0.001). Objective response rates also showed improvement, with 26.7% for durvalumab and 18.7% for placebo. Furthermore, single agent regorafenib has exhibited efficacy in patients with refractory advanced metastatic cholangiocarcinoma, particularly in terms of progression-free survival, favoring regorafenib over placebo, the overall toxicity profile was as expected. Regorafenib targets multiple tyrosine kinases and has been reported to show immunomodulatory properties that may counteract tumor-induced immunosuppression, providing a rationale for combining it with checkpoint inhibitors. We believe that modulating the tumor microenvironment with small molecule inhibitors like regorafenib will have synergistic effect when combined with checkpoint-based immunotherapy like durvalumab in patients with chemo refractory BTC. Methods: This study comprises a single-arm, unblinded Phase I/II trial designed to assess the safety and efficacy of the combination therapy involving regorafenib and durvalumab in patients with chemo-refractory advanced BTC. The Phase I portion employed a 3 + 3 design with two dose levels of regorafenib (80 mg and 120 mg) administered in conjunction with 1500 mg IV durvalumab every 28 days. Phase I successfully completed, and the Data Monitoring Committee (DMC) endorsed the continuation of the trial as planned. Eligibility: Histologically confirmed unresectable or metastatic disease. Progressed on at least one line of therapy. May have received checkpoint inhibitor in the past. Inclusion criteria also include ECOG PS 0-1. Objectives:Primary Phase I: Safety of regorafenib in combination with durvalumab.Phase II: Progression-free survival (PFS).Secondary:Disease Control Rate (DCR), Overall response rate (ORR), Overall survival (OS). Statistical Plan: Interim futility testing after one year, halting if conditional power &lt; 20%. Final analysis: PFS and OS via Weibull Regression. Enrollment is currently ongoing with 8 out of 40 patients enrolled.

Nonoperative Management for Traumatic Pseudoaneurysm of the Extrahepatic Portal Vein in an Older Adult

Nonoperative Management for Traumatic Pseudoaneurysm of the Extrahepatic Portal Vein in an Older Adult

Makeshift Shunts in Extrahepatic Portal Vein Obstruction in Pediatric Population.

More than 20% of patients with extrahepatic portal vein obstruction (EHPVO) may be deemed as nonshuntable due to lack of a suitable vein. The role of "makeshift shunts" or "lesser shunts" assumes importance in such cases. In this report, the authors have shared their experience with the makeshift shunts in the management of portal hypertension in children with emphasis upon anatomic considerations, resolution of symptoms, outcomes after surgery, and shunt patency. During the period 1983-2018, 138 children with portal hypertension were managed under the care of a single surgeon (VB). Of them, 134 were EHPVO. Children with EHPVO were treated with splenectomy and proximal lienorenal shunt (n = 107), splenectomy and devascularization (n = 21), and makeshift shunts (n = 6). Makeshift shunts comprised (i) side-to-side right gastroepiploic vein (Rt-GEV) to left renal vein (LRV) shunt (n = 1), (ii) superior mesenteric vein (SMV) to inferior vena cava (IVC) shunt using a spiral saphenous venous graft (n = 1), (iii) side-to-side inferior mesenteric vein (IMV) to LRV shunt (n = 2), (iv) side-to-side IMV to IVC shunt (n = 1), (v) end-to-side IMV to IVC shunt (n = 1), and (vi) side-to-side IMV to LRV shunt (n = 1) in a case of crossed fused renal ectopia. Following the creation of portosystemic shunt, a decline in portal pressure was demonstrated in all six patients. There was resolution of symptoms including hematemesis, melena, and anorectal variceal bleed. None of the patients demonstrated the features of hepatic encephalopathy. The associated portal cavernoma cholangiopathy (n = 1) also resolved following Rt-GEV to LRV shunt. Shunt patency was documented for the entire duration of follow-up (1.5-4 years) in five of six patients; the sixth patient demonstrated shunt block at 6-month follow-up but without recurrence of symptoms. Makeshift shunts offer a viable alternative to standard portosystemic shunting in pediatric patients with a nonshuntable vein. The selection of such shunts is, however, subject to surgeon's preferences and has to be individualized to local anatomy.

CT Arterioportography for Complex Portomesenteric Venous Interventions

AbstractAuthors report the use of computed tomography arterioportography (CTAP) in the diagnosis and endovascular treatment of portomesenteric venous occlusion. Three patients (2 male and 1 female; median age: 51 years) with an extrahepatic portal or mesenteric venous occlusion and variceal bleeding were included in this institutional review boards-approved retrospective study. CTAP was performed with an angiographic catheter positioned in the proximal superior mesenteric artery, and CT was obtained during the venous phase following the intra-arterial injection of contrast material. CTAP provided excellent opacification and delineation of portomesenteric veins, including occlusion length, intravascular webs, venous collaterals, and bleeding varices. This enabled preoperative planning for complex recanalization or extra-anatomic bypass to treat extrahepatic portal and mesenteric venous obstruction. There were no complications related to CTAP.

Ectopic Variceal Bleeding from the Hepaticojejunostomy due to Extrahepatic Portal Vein Occlusion: How to Treat?

Atypical variceal bleeding, which primarily stems from extrahepatic portal vein obstruction (EHPVO), is a severe complication of pancreatic hepatobiliary surgery. This review provides insights into this condition's incidence, diagnosis, and management strategies. The treatment modalities for atypical variceal bleeding resulting from EHPVO range from endoscopic intervention to surgical procedures, including direct variceal ligation and shunt surgery. Here, we discuss the efficacy and potential limitations of each treatment approach. Additionally, we explored the utility and therapeutic advantages of the meso-Rex shunt, a particularly promising surgical technique for mitigating the hemodynamic and metabolic impacts of EHPVO.

An Audit of Extrahepatic Portal Vein Obstruction: Experience from Tertiary Referral Center.

Extrahepatic portal vein obstruction (EHPVO) is a common cause of portal hypertension in India. (1) To evaluate the clinical presentation and the natural history of EHPVO; (2) to describe the risk factors, rebleeding rates and development of portal biliopathy on follow-up; and requirement of surgery in EHPVO at a tertiary care center. Data from 318 consecutive patients with EHPVO from June 2012 to October 2020 were analyzed. All patients underwent liver biochemistry, ultrasonography (USG) abdomen, upper gastrointestinal (GI) endoscopy, and viral serology. Color Doppler, computed tomography (CT) abdomen and magnetic resonance cholangiopancreatography (MRCP) were done as indicated. Mean age of presentation was 15.08 years [standard deviation (SD) 12.74; 6 months-60 years; 210 males)]. The presenting features were upper GI bleed (n = 227) (age at first bleed 11 years; 4 months-56 years), left hypochondrium pain or lump (n = 67), and only lower GI bleed (n = 1). Incidentally detected EHPVO on USG was seen in 10.69% (n = 34) patients. Postbleed ascites were seen in 10.69% (n = 34) patients. Six patients had symptomatic portal biliopathy and 14 had hypersplenism. Around 14.77% (n = 47) of patients had a history of being delivered at home, while 3.45% (n = 11) had a history of umbilical sepsis. During follow-up, 35.3% (n = 82) of patients had rebled. On imaging, associated splenic vein (SV) collaterals and superior mesenteric vein (SMV) collaterals were seen in 35.84% (n = 114) and 11.01% (n = 35) patients, respectively. Gallbladder varices were seen in 44.3% (n = 106), while gallstones in 5.66% (n = 18). On endoscopy, 87.42% (n = 278) patients had esophageal varices, 18.86% (n = 60) had isolated fundic varices, and three had ectopic varices. Only two patients had rectal varices and colopathy. Emergency devascularization was required in 3.45% (n = 14) patients for the failure of variceal bleed control, 1.88% (n = 7) underwent splenectomy, and four patients had proximal splenorenal shunt (PSRS) surgery. Extrahepatic portal hypertension (EHPVO) is an important cause of portal hypertension (PHT) in our country. The majority of them present with GI bleed; postbleed ascites were seen only in ~10%. Rebleed occurs in one-third of cases. Gallbladder varices were common; portal biliopathy occurred in 10% and were usually asymptomatic.

Portosystemic Shunt Surgery for Extrahepatic Portal Venous Obstruction Beyond Endoscopic Variceal Eradication: Two Decades of Pediatric Surgical Experience

This exclusively surgical series on pediatric extrahepatic portal venous obstruction (EHPVO) defines surgical indications beyond endoscopic eradication of esophageal varices (EEEV), the selection of an appropriate surgical procedure, and the long-term post-surgical outcome. EHPVO management protocol at the reporting institute has been endotherapy untilesophageal variceal eradication and surgery for select adverse sequelae manifesting after EEEV. One hundred and thirty-nine EHPVO cases underwent surgery for the following indications in combination: i) massive splenomegaly with severe hypersplenism (n=132, 95%); ii) growth retardation (GR, n=95, 68%); iii) isolated gastric (IGV) and ectopic varices (n=49, 35%); iv) Portal cavernoma cholangiopathy (PCC) (n=07, 5%). A portosystemic shunt (PSS) was performed in 119 (86%) cases. Types of PSS performed were as follows: central end-to-side splenorenal shunt with splenectomy (n=104); side-to-side splenorenal shunt (n=4); mesocaval shunt (n=1); inferior mesenteric vein (IMV) to left renal vein shunt (n=2); IMV to inferior vena cava shunt (n=3); H-graft interposition splenorenal shunt (n=1); spleno-adrenal shunt (n=3); makeshift shunt (n=1). Esophagogastric devascularization (n=20, 14%) was opted for only for non-shuntable anatomy. At a median follow-up (FU) of 41 (range: 6-228) months, PSS block was detected in 13 (11%) cases, with recurrent variceal bleeding in 4 cases. PCC-related cholestasis regressed in 5 of 7 cases. Issues of splenomegaly were resolved, and growth z-scores improved significantly. Endotherapy for secondary prophylaxis untilEEEV has resulted in a shift in surgical indications for EHPVO. Beyond EEEV, surgery was indicated predominantly for non-variceal sequelae, namely massive splenomegaly with severe hypersplenism, GR, and PCC. Varices warranted surgery infrequently butmore often from sites less amenable to endotherapy, i.e., IGV and ectopic varices. The selection of PSS was tailored to anatomy and surgical indications. On long-term FU post surgery, PSS block was detected in 13% of patients. PCC-related cholestasis regressed in 71%, and issues of splenomegaly resolved with significantly improved growth Z scores.

Abstract 1065: Cancer-specific molecular subtypes of cholangiocarcinoma reveal unique molecular dependency with clinical relevance

Abstract Introduction: Cholangiocarcinoma is a heterogeneous tumor entity harboring distinct tumor biology and clinical phenotype according to their unique tumor microenvironment (TME), especially anatomic location in terms of intrahepatic, peri-hilar, and distal extrahepatic portion. Therefore, there is a clear unmet need for the precision strategy based on cancer-specific genomic features rather than previous molecular subtypes from bulk RNA sequencing. Methods: Comprehensive analyses were performed using the dataset from the cancer dependency map (DepMap) project, including cancer-specific molecular characterization with multi-omics data, genome-wide loss-of-function screening with CRISPR-Cas9 system, and drug sensitivity with compound screening to uncover cancer-specific molecular subtypes showing clinical relevance. The cancer-specific molecular signatures were validated in independent translational cohorts (TCGA-CHOL; n=45, ICGC-BTC; n=181, GSE132305; n=220). Results: Integrative profiling of transcriptome from bile duct cancer cell lines (n=39) from the Cancer Cell Line Encyclopedia (CCLE) revealed a total of three cancer-specific molecular subtypes showing distinct tumor biology through all omics layer as well as clinical relevance with prognostic significance. Major molecular features of each subtype were reproducible in the validation cohorts. Subtype-specific molecular biomarkers, including mutational signature and metabolites, were identified. Finally, the target drug with subtype-specific genetic dependency was analyzed to provide precision strategy according to distinct subtypes’ molecular characterization. Conclusions: Comprehensive analysis using a multi-omics dataset revealed precision strategies based on cancer-specific molecular subtypes of cholangiocarcinoma in terms of tumor classification and discriminative therapeutic chances. Prospective translational studies companion with clinical trials based on cancer-specific molecular subtypes is mandatory to establish the precision strategy for managing cholangiocarcinoma. Citation Format: Woo Young Kwon, Sung Hwan LEE. Cancer-specific molecular subtypes of cholangiocarcinoma reveal unique molecular dependency with clinical relevance [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 1065.

Splenic Stiffness By 2D Shear Wave Elastography (SWE) and Platelet Count to Predict Varices Needing Treatment and Bleeding in Extrahepatic Portal

Splenic Stiffness By 2D Shear Wave Elastography (SWE) and Platelet Count to Predict Varices Needing Treatment and Bleeding in Extrahepatic Portal

Assessment of Rex vein patency in children with Extra hepatic Portal Venous Obstruction

Assessment of Rex vein patency in children with Extra hepatic Portal Venous Obstruction

Focal Nodular Hyperplasia–like Lesions in Patients with Extrahepatic Portal Vein Obstruction

Focal Nodular Hyperplasia–like Lesions in Patients with Extrahepatic Portal Vein Obstruction

Bibliometric Analysis and Visualization of the Extrahepatic Portal Venous Obstruction Publication Landscape.

A scientometric analysis was conducted to characterize the global research publications in extrahepatic portal venous obstruction (EHPVO), and state-of-the-art visualization graphics were generated to provide insight into specific bibliometric variables. The Web of Science database was accessed for research productivity and bibliometric variables of countries, institutions, authors, journals, and content analysis of top-20 cited documents were performed. Collaborative networks and co-occurrence of keywords map were generated using VOSviewer software. Two hundred and sixteen records were retrieved with an annual growth rate of 2.53%. India is the leading country in productivity (n = 4339), followed by the USA and China. Post Graduate Institute of Medical Education and Research, Chandigarh, was the top productive institute. Sarin SK was the most prolific author, having the highest citations received and h-index. The hotspot topics were "portal hypertension," "cirrhosis," "children," "biliopathy/cholangiopathy," "liver fibrosis," and "liver transplantation" as per keyword co-occurrence networking. J Gastroenterol Hepatol had the most publications of EHPVO research as well the h-index. Regarding collaborative network mapping, the USA and Primignani M were the significant nodes among country and author, respectively. EHPVO research publication volume is low but is gradually progressing with dominant contributions from Indian institutes and authors. Most highly cited articles are of low level of evidence, and multi-institutional collaborative research can be the way forward.

Pathology of Cholangiocarcinoma.

The liver excretes bile through the biliary system, which has a complicated anatomical structure. Cholangiocarcinoma, a malignant bile duct epithelial tumor, is separated into intrahepatic and extrahepatic portions depending on the structure of the bile duct and exhibits both similarities and varieties in patient presentations and staging. The three main macroscopic characteristics of cholangiocarcinoma-mass formating, intraductal growth, and periductal infiltrating types-allow pathologists and surgeons to see and analyze the cancerous tissue. The majority of cholangiocarcinoma patients are in advanced stages and poor prognosis. Although surgery is the main treatment option, target therapy based on molecular pathology background offers hope for improving patient's prognosis.

Single center real-world treatment and outcomes in patients with hepatocellular carcinoma receiving immunotherapy.

e16134 Background: Immune checkpoint inhibitor (ICI) based therapy has emerged as a therapeutic option in hepatocellular carcinoma (HCC), both in combination (IC-C) and as single agent (IC-SA). Approvals were based on clinical trials with strict eligibility criteria limiting generalizability to the entire spectrum of clinical practice. Survival outcomes have not been evaluated in a real-world population and there is no established post ICI treatment standard. Methods: Patients (pts) with advanced HCC treated with ICI across lines of therapy were included in this retrospective study. Data regarding demographics, comorbidities, HCC etiology, liver function (Child Pugh Score [CPS] and ALBI Grade [G]), tumor burden, AFP, treatments, reason for discontinuation (dc) and outcomes were collected. Descriptive statistics and survival analysis were performed using Stata with cox regression analysis. Results: The cohort consisted of 138 pts: median age 63 years (24,95); 83% male; 38% Hispanic/Latino, 27% Asian, 17% Non-Hispanic White, 4% Black and 14% other/unknown. Etiology of cirrhosis: 16% Hepatitis B, 38% Hepatitis C, 12% alcohol liver disease, 9% NAFLD and 24 % mixed/other; Baseline CPS were 74% CPA, 23% CPB, and 2% CPC; ALBI Scores were G1 in 35%, G2 in 53%, and G3 in 12% pts; 62% had extrahepatic disease and/or portal invasion; AFP was ≥ 400ng/mL in 30% of pts. 88% of pts had prior local therapy. For the entire cohort, first line systemic therapy consisted of 52% ICI (18% IC-C and 34% IC-SA) and 48% TKI. mOS was 12 months (mo) (0,74) for first line ICI group and 19.5 mo (3,78) in those with first line TKI. In CPA pts, first line therapy was 51% ICI (24% IC-C and 27% IC-SA) and 39% TKI. 79% of CPA patients received ≥ 2 lines of therapy with second line consisting of 65% ICI and 20% TKI. First line therapy for pts with CPB cirrhosis was 58% ICI (2% IC-C and 55% IC-SA) and 42% TKI. 55% of CPB patients received ≥ 2 lines of therapy and second line therapy for CPB pts included 71% ICI and 18% TKI. mOS was 18 mo in CPA pts and 10 mo in CPB pts. Cirrhosis related complications resulted in treatment discontinuation in 2% of CPA pts vs. 14% of CPB pts. On multivariable analysis, Asian ethnicity (HR 0.41 p = 0.006), CPS (HR 1.64 p = 0.027), and number of treatment lines (HR 0.74 p = 0.005) were associated with OS. Conclusions: This single institution real-world cohort highlights the reality of sequential therapy in pts with advanced HCC. Survival outcomes in our CPA cohort are comparable to data from recent phase 3 trials. The survival in our CPB cohort in a tertiary care setting compares favorably with available data from clinical trials and suggests the feasibility of sequential and effective anti-cancer therapy in this population. Liver function, Asian ethnicity, and number of treatment lines are independent predictors of OS in this cohort of HCC patients receiving ICI. Prospective studies related to optimal treatment sequence and to CPB pts are needed.

Benign Hepatic Nodules in Patients With Primary Extrahepatic Portal Vein Obstruction: Clinical and Magnetic Resonance Imaging Features

To retrospectively evaluate the magnetic resonance imaging (MRI) features of benign hepatic nodules in patients with extrahepatic portal vein obstruction (EHPVO) and assess predictable features for their development. This retrospective observational study included 18 diagnosed patients of EHPVO who underwent contrast enhanced abdominal MRI at our Institute between June 2016 and May 2017, and who could be followed up for at least two years. The patients with liver nodules formed the study group (n= 8; 4 males, 4 females; mean age: 26.1±10.9 years) and patients without liver nodules were controls (n= 10; 3 males, 7 females; mean age: 24.2±15.1 years). Liver nodules were confirmed as benign by either biopsy or stability on follow up imaging. MRI features of liver nodules were assessed. Clinical details and imaging data of the study group were compared with controls to assess predictable features. There was no statistically significant difference in age, gender, clinical characteristics and upper gastrointestinal endoscopic findings between the study and control groups. The size of the lienorenal collateral,left renal vein and superior mesenteric vein were significantly larger in the study group (P < 0.05). In the study group, the majority had multiple hepatic nodules with most of them being isointense on T1 (18/35; 51.4%) and T2-weighted images (16/35; 45.7%) and showing restriction of diffusion (21/35; 60%). All (n= 35) lesions showed arterial phase hyperenhancement and none showed washout in the venous phase. The patients in the control group did not develop any liver nodules during the follow-up period. Liver nodules in patients with EHPVO are likely to be benign and have characteristic MRI features. Significantly larger lienorenal collateral,left renal vein and superior mesenteric vein were associated with the development of these nodules.

Single-center real-world treatment and outcomes in patients with hepatocellular carcinoma receiving immunotherapy.

401 Background: Immune checkpoint inhibitor (ICI) based therapy has emerged as a therapeutic option in hepatocellular carcinoma (HCC). Approvals were based on clinical trials with strict eligibility criteria limiting generalizability to clinical practice. Further, there is no established post ICI treatment standard. Methods: Patients (pts) with advanced HCC treated with ICI as single agent (ICI-SA) or in combination (ICI-C) across lines of therapy were included in this retrospective study. Results: The cohort consisted of 118 pts: median age 63 years (24, 88); 84% male; 35% Hispanic/Latino, 26% Asian, 19% Non-Hispanic White, 5% Black and 15% unknown. Etiology of cirrhosis: 13% Hepatitis B, 45% Hepatitis C, 16 % alcohol liver disease, 10% NAFLD and 16 % mixed/other; 73% had baseline Child Pugh (CP) A, 25% had CP-B, and 2% had CP-C; ALBI Scores were ≤ -2.60 in 32%, 2.60 &lt; and ≤-1.39 in 48%, and ≥-1.39 in 19% pts; 62% had extrahepatic disease and/or portal invasion; AFP was ≥ 400 ng/mL in 25% of pts. 81% of pts had prior local therapy. First line systemic therapies were: TKIs in 52 pts (44%), ICI-SA in 42 (36%), ICI-C in 15 (13%), clinical trial agent (CTA) in 8 (7%) and chemotherapy in 1 (1%), with a median duration (dur) of 4 months (95% CI 2.3 to 4). Ninety one (77%) and 61 pts (52%) received ≥ 2 or 3 lines of therapy, respectively. Fifty seven pts had ICI-SA and 14 ICI-C in second line or beyond. Median dur of ICI therapy was 4 months (95% CI 3-5) for all lines. Post-ICI therapies included 11% ICI, 30% CTA and 59% TKIs. TKIs included sorafenib (46%), cabozantinib (27%), lenvatinib (15%), and regorafenib (27%). For the 118 pt cohort, mOS was 14 months (95% CI 12-19). For pts treated with ICI in first line, mOS was 11 months (0, 74); post-ICI mOS was 6 months (95% CI 3-9) and mPFS was 3 months (95% CI 2-3). Thirty one pts received a TKI post ICI; mOS for this subset was 19 months (15, 22); mOS from start of TKI post ICI was 6.5 months (4, 12). On multivariable regression analysis, ALBI score was associated with OS (HR 1.63, p=0.02, CI: 1.08-2.27). Conclusions: Pts with advanced HCC and CP-A or B cirrhosis are able to receive sequential systemic therapy including ICI. Survival outcomes in this cohort are impacted by the inclusion of patients with more compromised liver function and less restrictive pt selection compared to clinical trials. Usage of TKIs post ICI is feasible with suggestion of clinical activity but this is an area in need of prospective studies.