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How Do I Diagnose Fibrolamellar Carcinoma?
Mod Pathol
January 2025
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN. Electronic address:
Fibrolamellar carcinoma (FLC) is a unique primary carcinoma of liver that is characterized by distinct morphologic findings and a recurrent DNAJB1::PRKACA gene fusion. It typically presents in young individuals without underlying liver dysfunction. FLC is a difficult diagnosis when based only on morphology and misdiagnosis is not uncommon.
View Article and Find Full Text PDFDNAJB1-PKAc Kinase Is Expressed in Young Patients with Pediatric Liver Cancers and Enhances Carcinogenic Pathways.
Cancers (Basel)
December 2024
Division of General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
Hepatoblastoma (HBL) and fibrolamellar hepatocellular carcinoma (FLC) are the most common liver malignancies in children and young adults. FLC oncogenesis is associated with the generation of the fusion kinase, DNAJB1-PKAc (J-PKAc). J-PKAc has been found in 90% of FLC patients' tumors but not in other liver cancers.
View Article and Find Full Text PDFCytoreductive surgery and HIPEC with oxaliplatin for the treatment of peritoneal carcinomatosis from recurrent fibrolamellar carcinoma.
BMJ Case Rep
January 2025
General Surgery, Hospital Universitario HM Sanchinarro, Madrid, Spain
Fibrolamellar hepatocellular carcinoma (FHCC) is a rare variant of hepatocellular carcinoma (HCC), characterised by a poorer prognosis in later stages compared with conventional HCC due to a high rate of local recurrence, lymph node metastasis and peritoneal metastasis. Conventional chemotherapy is generally ineffective, making surgery the only potentially curative treatment. Currently, surgery is also indicated in cases of recurrence, always aiming for an R0 resection.
View Article and Find Full Text PDFFibrolamellar hepatocellular carcinoma with intrapancreatic nodal metastases masquerading as pancreatic neuroendocrine tumour with hepatic metastases.
BMJ Case Rep
January 2025
General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Fibrolamellar hepatocellular carcinoma (FL-HCC) is a primary liver tumour presenting at a young age. Aggressive surgery of FL-HCC is the mainstay of management unlike other malignancies where metastatic stage precludes curative surgery. There are limited reports of response of FL-HCC to systemic therapies predominantly owing to its rarity.
View Article and Find Full Text PDFLiver cancer multiomics reveals diverse protein kinase A disruptions convergently produce fibrolamellar hepatocellular carcinoma.
Nat Commun
December 2024
Laboratory of Cellular Biophysics, The Rockefeller University, New York, NY, USA.
Fibrolamellar Hepatocellular Carcinoma (FLC) is a rare liver cancer characterized by a fusion oncokinase of the genes DNAJB1 and PRKACA, the catalytic subunit of protein kinase A (PKA). A few FLC-like tumors have been reported showing other alterations involving PKA. To better understand FLC pathogenesis and the relationships among FLC, FLC-like, and other liver tumors, we performed a massive multi-omics analysis.
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