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Pediatr Blood Cancer ; 65(4)2018 04.
Article in English | MEDLINE | ID: mdl-29286561

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.


Subject(s)
Brain Neoplasms , Carcinoma, Hepatocellular , Liver Neoplasms , Neuroimaging , Neurosurgical Procedures , Adolescent , Brain Neoplasms/diagnostic imaging , Brain Neoplasms/genetics , Brain Neoplasms/secondary , Brain Neoplasms/surgery , Carcinoma, Hepatocellular/diagnostic imaging , Carcinoma, Hepatocellular/genetics , Carcinoma, Hepatocellular/metabolism , Carcinoma, Hepatocellular/surgery , Cyclic AMP-Dependent Protein Kinase Catalytic Subunits/genetics , Cyclic AMP-Dependent Protein Kinase Catalytic Subunits/metabolism , Female , HSP40 Heat-Shock Proteins/genetics , HSP40 Heat-Shock Proteins/metabolism , Humans , Liver Neoplasms/diagnostic imaging , Liver Neoplasms/genetics , Liver Neoplasms/metabolism , Liver Neoplasms/surgery , Neoplasm Metastasis , Oncogene Proteins, Fusion/genetics , Oncogene Proteins, Fusion/metabolism
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