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Fibrolamellar carcinoma (FLC) is a rare primary liver tumor affecting predominantly younger and otherwise healthy patients. Typically, FLC presents with advanced disease due to the paucity of typical symptoms and no history of underlying liver disease. Depending on tumor characteristics and the patient's general condition, surgical treatment is the most promising treatment modality. Aggressive resection and liver transplantation have been utilized and are presently indispensable curative treatment options. Under certain circumstances surgical resection is also possible for metachronous metastases or local recurrence. Recent tumor biology discoveries have contributed to improved diagnostic specificity and systemic treatment options.
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http://dx.doi.org/10.2147/OTT.S296127 | DOI Listing |
Anticancer Res
September 2025
Department of Surgery, Yamaga City Medical Center, Yamaga, Japan;
Background/aim: Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare histological variant of hepatocellular carcinoma (HCC) that typically affects adolescents and young adults without underlying liver disease. In elderly patients, particularly those with a history of chronic hepatitis C virus (HCV) infection, FLHCC is extremely rare and presents diagnostic challenges.
Case Report: A 67-year-old woman with a history of chronic HCV infection and sustained virologic response (SVR) was incidentally found to have two hepatic lesions during routine follow-up imaging.
Ann Saudi Med
August 2025
From the Division of Medical Oncology, Department of Internal Medicine, Medipol University School of Medicine, Istanbul, Türkiye.
Background: Fibrolamellar Hepatocellular Carcinoma (FLHCC) is a rare primary liver tumour that is distinct from conventional HCC in both histopathological and clinical features. There is no clear consensus on its treatment.
Objective: Assess the demographics, prognosis, and treatment- particularly systemic therapies-of FLHCC patients across multiple centers in Türkiye.
Mol Cancer Res
July 2025
Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States.
The pediatric and adolescent liver cancers Hepatoblastoma (HBL) and Fibrolamellar hepatocellular carcinoma (FLC) are dangerous diseases requiring aggressive surgery, when feasible, and non-targeted toxic chemotherapy for a chance of cure, due to insufficient knowledge of underlying molecular mechanisms. We've previously reported the essential role of ph-S675-β-catenin in the reorganization of genomic structure in HBL and FLC by oncogenic activation via chromosomal regions called Cancer Enhancing Genomic Regions or Aggressive Liver Cancer Domains (CEGRs/ALCDs). In FLC, the fusion J-PKAc oncoprotein phosphorylates β-catenin at Ser675, triggering such CEGRs/ALCDs-mediated activation of oncogenes.
View Article and Find Full Text PDFJ Clin Exp Hepatol
June 2025
Division of Hematology, Oncology, and Blood and Marrow Transplantation, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.
Sci Rep
July 2025
Department of Surgery, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA.
Fibrolamellar carcinoma (FLC) is a type of primary liver cancer that predominantly affects healthy adolescents and young adults in a background of normal liver. The DNAJB1-PRKACA fusion gene is an oncogenic driver in FLC tumors. To investigate the oncogenic mechanisms of this fusion gene, we developed a model using human liver progenitor organoids engineered to express DNAJB1-PRKACA.
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