Abstract
Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare liver malignancy in adolescents and young adults. Surgery is the mainstay of therapy for primary and metastatic disease. Most patients relapse, with development of both local and distant metastases. Brain metastases from solid tumors are rare in the pediatric and young adult population. Here, we document three patients with brain metastases from FLHCC, confirmed by histology and molecular characterization of the chimeric fusion DNAJB1-PRKACA, each necessitating neurosurgical intervention. These observations highlight the ability of FLHCC to metastasize to the brain and suggest the need for surveillance neuroimaging for patients with advanced-stage disease.
Keywords:
adolescent and young adult tumors; brain; fibrolamellar; liver; rare tumors; tumor biology; tumors.
© 2017 Wiley Periodicals, Inc.
Publication types
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Case Reports
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Research Support, N.I.H., Extramural
MeSH terms
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Adolescent
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Brain Neoplasms* / diagnostic imaging
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Brain Neoplasms* / genetics
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Brain Neoplasms* / secondary
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Brain Neoplasms* / surgery
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Carcinoma, Hepatocellular* / diagnostic imaging
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Carcinoma, Hepatocellular* / genetics
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Carcinoma, Hepatocellular* / metabolism
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Carcinoma, Hepatocellular* / surgery
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Cyclic AMP-Dependent Protein Kinase Catalytic Subunits / genetics
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Cyclic AMP-Dependent Protein Kinase Catalytic Subunits / metabolism
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Female
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HSP40 Heat-Shock Proteins / genetics
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HSP40 Heat-Shock Proteins / metabolism
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Humans
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Liver Neoplasms* / diagnostic imaging
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Liver Neoplasms* / genetics
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Liver Neoplasms* / metabolism
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Liver Neoplasms* / surgery
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Neoplasm Metastasis
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Neuroimaging*
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Neurosurgical Procedures*
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Oncogene Proteins, Fusion / genetics
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Oncogene Proteins, Fusion / metabolism
Substances
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DNAJB1 protein, human
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HSP40 Heat-Shock Proteins
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Oncogene Proteins, Fusion
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Cyclic AMP-Dependent Protein Kinase Catalytic Subunits
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PRKACA protein, human
Supplementary concepts
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Fibrolamellar hepatocellular carcinoma