ArticleJournal of gastrointestinal oncology2019
Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma.
Article in Journal of gastrointestinal oncology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 2 of them syntheses that pooled it.
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Who cites it
23 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- Treatment and Prognosis of Fibrolamellar Hepatocellular Carcinoma: a Systematic Review of the Recent Literature and Meta-analysis.Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract · 2023Pooled it
- The Role of Immunotherapy in Hepatocellular Carcinoma: A Systematic Review and Pooled Analysis of 2,402 Patients.The oncologist · 2021Pooled it
- Fibrolamellar carcinoma: The role of imaging in diagnosis and management.Radiology case reports · 2026Article
- Metastatic Fibrolamellar Hepatocellular Carcinoma in a Young Adult: A Case Report and Narrative Review.Journal of hepatocellular carcinoma · 2026Article
- Fibrolamellar hepatocellular carcinoma: Advances, challenges and opportunities in a rare malignancy.World journal of gastrointestinal surgery · 2025Review
- Identification of AKTIP as a biomarker for fibrolamellar carcinoma using WGCNA and machine learning.3 Biotech · 2025Article
- A preclinical "magic bullet" against fibrolamellar hepatocellular carcinoma?Molecular therapy : the journal of the American Society of Gene Therapy · 2024Article
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- Treatment Outcomes in Patients with Advanced Fibrolamellar Hepatocellular Carcinoma Under Systemic Treatment: Analysis of Clinical Characteristics, Management, and Radiomics.Journal of hepatocellular carcinoma · 2023Article
- Evaluation of Protein Kinase cAMP-Activated Catalytic Subunit Alpha as a Therapeutic Target for Fibrolamellar Carcinoma.Gastro hep advances · 2023Article
- Current Advances in the Treatment of Fibrolamellar Carcinoma of Liver.Journal of hepatocellular carcinoma · 2023Review
- Fibrolamellar hepatocellular carcinoma: A rare but unpleasant event.World journal of gastrointestinal oncology · 2022Review
- Survival characteristics of fibrolamellar hepatocellular carcinoma: A Surveillance, Epidemiology, and End Results database study.World journal of clinical oncology · 2022Article
- A framework for fibrolamellar carcinoma research and clinical trials.Nature reviews. Gastroenterology & hepatology · 2022Review
- A Multidisciplinary Approach to the Management of Fibrolamellar Carcinoma: Current Perspectives and Future Prospects.OncoTargets and therapy · 2022Review
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- New insights into the pathophysiology and clinical care of rare primary liver cancers.JHEP reports : innovation in hepatology · 2021Review
- Fibrolamellar Hepatocellular Carcinoma: A Population-Based Observational Study.Digestive diseases and sciences · 2021Observational
- Hyperammonemic Encephalopathy Mimicking Ornithine Transcarbamylase Deficiency in Fibrolamellar Hepatocellular Carcinoma: Successful Treatment with Continuous Venovenous Hemofiltration and Ammonia Scavengers.Cancer research and treatment · 2021Article
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Authors and funding
12 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundClinicopathological features and the outcomes of patients with fibrolamellar hepatocellular carcinoma (FLHCC) are not clearly defined.
methodsData were collected by retrospective chart review on 42 patients with FLHCC treated between 1990 and 2017 at Mayo Clinic.
resultsOf 42 patients (median age at diagnosis 22 years), 10 patients (23.8%) had stage I disease and 32 patients (76.2%) had stage II to IVB disease. All 10 patients with stage I disease and 21 of 32 patients with stage II-IVB disease underwent resection at presentation. In stage I patient group, 6 patients experienced recurrence with a median time to recurrence of 30.5 months and a 5-year overall survival (OS) of 86%. Patients with stage II to IVB disease who underwent resection (n=21) upfront had a median OS of 32.5 months and 5-year OS of 44%. In the upfront surgery group, 71% of patients experienced recurrence. The median OS of patients with unresectable disease (n=11) was 10 months. Four out of nine patients treated with sorafenib had stable disease and one patient with programmed cell death ligand-1 (PD-L1) expressing tumor had a near complete response after 2 months of therapy with nivolumab.
conclusionsIn FLHCC, surgical resection was associated with prolonged OS; although most patients had a disease recurrence regardless of disease stage and resection margin status. The response to kinase inhibitor, sorafenib, was variable. In select cases, therapy with a checkpoint inhibitor may provide a viable treatment option.
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