title: Advances in fibrolamellar hepatocellular carcinoma: a review.
abstract: Fibrolamellar hepatocellular carcinoma is a rare primary liver tumor that often arises in the absence of cirrhosis or viral hepatitis. Compared with hepatocellular carcinoma, patients are typically younger with less comorbidities. Diagnosis is often multimodal and requires a high level of suspicion, as traditional liver pathology markers, such as serum α fetoprotein and transaminases, are often normal. Overall, patients respond well to surgical resection but recurrences are frequent, and alternative therapies, such as chemotherapy and radiation, are not well studied. Currently, there are no established chemotherapy regimens; there are only limited case reports of select agents, such as 5-fluorouracil with interferon-α and gemcitabine with oxaliplatin, used with varying degrees of success. Because little is known about this rare tumor, the development of serum markers and alternative therapies continues to be a challenge. A major advancement in the understanding of this rare disease is the discovery of a functional chimeric transcript incorporating DNAJB1 and PRKACA. This finding may finally provide the basis for specific diagnostic markers and chemotherapies that patients with this disease have long needed. Here, we present advances in the surgical treatment of fibrolamellar hepatocellular carcinoma, as well as recent data on its tumor biology and pathogenesis.
authors.affiliation: Department of Surgery, Pediatric Surgical Service, Memorial Sloan Kettering Cancer Center, New York, New York, United States.
authors.forename: Irene Isabel P
authors.initials: II
authors.lastname: Lim
authors.affiliation: Department of Surgery, Pediatric Surgical Service, Memorial Sloan Kettering Cancer Center, New York, New York, United States.
authors.forename: Benjamin A
authors.initials: BA
authors.lastname: Farber
authors.affiliation: Department of Surgery, Pediatric Surgical Service, Memorial Sloan Kettering Cancer Center, New York, New York, United States.
authors.forename: Michael P
authors.initials: MP
authors.lastname: LaQuaglia
mesh_terms: D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000235:genetics; D006528:Carcinoma, Hepatocellular / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D002648:Child; D054751:Cyclic AMP-Dependent Protein Kinase Catalytic Subunits / Q000235:genetics; D050956:HSP40 Heat-Shock Proteins / Q000235:genetics; D006801:Humans; D008113:Liver Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery
chemical_list: D014408:Biomarkers, Tumor; C494647:DNAJB1 protein, human; D050956:HSP40 Heat-Shock Proteins; D054751:Cyclic AMP-Dependent Protein Kinase Catalytic Subunits; C517757:PRKACA protein, human