Advances in fibrolamellar hepatocellular carcinoma: A review Journal Article


Authors: Lim, I. I. P.; Farber, B. A.; LaQuaglia, M. P.
Article 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.
Keywords: cancer chemotherapy; cancer surgery; survival rate; unclassified drug; overall survival; clinical feature; pathogenesis; review; cancer recurrence; fluorouracil; liver cell carcinoma; liver transplantation; multimodality cancer therapy; alpha interferon; gemcitabine; cancer radiotherapy; lymph node dissection; epidermal growth factor receptor; protein; surgical approach; folinic acid; comorbidity; liver resection; alternative medicine; oxaliplatin; fibroblast growth factor receptor 1; antiestrogen; alpha fetoprotein; rare disease; intraoperative radiotherapy; recurrence free survival; liver tumors; cancer prognosis; human; fibrolamellar hepatocellular carcinoma; protein dnajb1; protein prkaca
Journal Title: European Journal of Pediatric Surgery
Volume: 24
Issue: 6
ISSN: 0939-7248
Publisher: Georg Thieme Verlag Kg  
Date Published: 2014-12-01
Start Page: 461
End Page: 466
Language: English
DOI: 10.1055/s-0034-1396420
PROVIDER: scopus
PUBMED: 25486412
DOI/URL:
Notes: Export Date: 2 February 2015 -- Source: Scopus
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  1. Benjamin Asher Farber
    12 Farber