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Fibrolamellar Variant of Hepatocellular Carcinoma

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Multidisciplinary Treatment of Hepatocellular Carcinoma

Part of the book series: Recent Results in Cancer Research ((RECENTCANCER,volume 190))

Abstract

The fibrolamellar variant of hepatocellular carcinoma is a rare primary liver cancer occurring in adolescents and young adults without chronic liver disease or known risk factors. Histologically, it is defined by lamellar bands of fibrosis surrounding well-differentiated tumor cells. Radiologic imaging typically demonstrates a large, solitary mass with calcifications and a central scar. Lymph node metastases in the porta hepatis are frequently diagnosed upon presentation. More patients with fibrolamellar carcinoma are candidates for surgical resection than those with conventional hepatocellular carcinoma, owing to their young age and absence of cirrhosis. The most important prognostic factor is surgical resection, which results in 5-year overall survival rates ranging between 50 and 76 %. Despite complete surgical resection, relapse rates are high, and novel therapies are needed to prevent and treat recurrent disease.

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Author information

Authors and Affiliations

  1. Department of Surgical Oncology, Fox Chase Cancer Center, 333 Cottman Avenue, Philadelphia, PA, 19111, USA

    Yun Shin Chun

  2. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 1484, Houston, TX, 77030, USA

    Giuseppe Zimmitti

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  1. Yun Shin Chun
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  2. Giuseppe Zimmitti
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Correspondence to Yun Shin Chun .

Editor information

Editors and Affiliations

  1. M.D. Anderson Cancer Center, Dept. Surgical Oncology, University of Texas, Holcombe Blvd. 1515, Houston, 77030, Texas, USA

    Jean-Nicolas Vauthey

  2. , Dept. of Surgical Oncology, MD Anderson Cancer Center, Holcombe Boulevard, Unit 444 1515, Houston, 77030, USA

    Antoine Brouquet

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Chun, Y.S., Zimmitti, G. (2013). Fibrolamellar Variant of Hepatocellular Carcinoma. In: Vauthey, JN., Brouquet, A. (eds) Multidisciplinary Treatment of Hepatocellular Carcinoma. Recent Results in Cancer Research, vol 190. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-16037-0_7

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  • DOI: https://doi.org/10.1007/978-3-642-16037-0_7

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-16036-3

  • Online ISBN: 978-3-642-16037-0

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