A 38-year-old Malayan gharial (Tomistoma schlegelii) with a 2-week history of anorexia was found dead and presented for post-mortem examination. Numerous white firm nodules of various sizes were found on the surface of the liver, both left and right kidneys, the spleen and the serosa of the intestinal tract. All masses had similar microscopical appearance and were diagnosed as metastasizing fibrolamellar hepatocellular carcinoma. Immunohistochemically, the tumour cells did not react with antibodies specific for pan-cytokeratin, vimentin or HepPar-1. The anti-HepPar-1 and anti-pan-cytokeratin antibodies also did not react with normal hepatocytes or exocrine pancreatic cells. This is the first description of fibrolamellar hepatocellular carcinoma with metastases in a crocodilian.
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http://dx.doi.org/10.1016/j.jcpa.2017.06.007 | DOI Listing |
Rev Esp Enferm Dig
January 2025
Oncología Médica, Hospital Universitario de Burgos, ESPAÑA.
We present the case of a 21-year-old male diagnosed with stage IV fibrolamellar hepatocellular carcinoma, studied by the Oncomine Comprehensive Assay genomic sequencing panel with the finding of the DNAJB1-PRKACA fusion and treated with a combination of chemotherapy and immunotherapy based on cisplatin, 5-fluorouracil, adriamycin and nivolumab.
View Article and Find Full Text PDFMod 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 PDFCancers (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 PDFBMJ 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 PDFBMJ 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.
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