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.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Edmondson HA (1956) Differential diagnosis of tumors and tumor-like lesions of liver in infancy and childhood. AMA J Dis Child 91:168–186
Craig JR, Peters RL, Edmondson HA, Omata M (1980) Fibrolamellar carcinoma of the liver: a tumor of adolescents and young adults with distinctive clinico-pathologic features. Cancer 46:372–379
Berman MM, Libbey NP, Foster JH (1980) Hepatocellular carcinoma. Polygonal cell type with fibrous stroma–an atypical variant with a favorable prognosis. Cancer 46:1448–1455
Torbenson M (2007) Review of the clinicopathologic features of fibrolamellar carcinoma. Adv Anat Pathol 14:217–223
El-Serag HB, Davila JA (2004) Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population-based study. Hepatology 39:798–803
Okuda K (2002) Natural history of hepatocellular carcinoma including fibrolamellar and hepato-cholangiocarcinoma variants. J Gastroenterol Hepatol 17:401–405
Payne CM, Nagle RB, Paplanus SH, Graham AR (1986) Fibrolamellar carcinoma of liver: a primary malignant oncocytic carcinoid? Ultrastruct Pathol 10:539–552
Ward SC, Huang J, Tickoo SK, Thung SN, Ladanyi M, Klimstra DS (2010) Fibrolamellar carcinoma of the liver exhibits immunohistochemical evidence of both hepatocyte and bile duct differentiation. Mod Pathol 23:1180–1190
Agarwal VR, Takayama K, Van Wyk JJ, Sasano H, Simpson ER, Bulun SE (1998) Molecular basis of severe gynecomastia associated with aromatase expression in a fibrolamellar hepatocellular carcinoma. J Clin Endocrinol Metab 83:1797–1800
Muramori K, Taguchi S, Taguchi T, Kohashi K, Furuya K, Tokuda K, Ishii E (2011) High aromatase activity and overexpression of epidermal growth factor receptor in fibrolamellar hepatocellular carcinoma in a child. J Pediatr Hematol Oncol 33:e195–197
Pinna AD, Iwatsuki S, Lee RG, Todo S, Madariaga JR, Marsh JW, Casavilla A, Dvorchik I, Fung JJ, Starzl TE (1997) Treatment of fibrolamellar hepatoma with subtotal hepatectomy or transplantation. Hepatology 26:877–883
Kanai T, Takabayashi T, Kawano Y, Kuramochi S, Miyazawa N (2004) A case of postoperative recurrence of fibrolamellar hepatocellular carcinoma with increased vitamin B12 binding capacity in a young Japanese female. Jpn J Clin Oncol 34:346–351
Waxman S, Gilbert HS (1973) A tumor-related vitamin B12 binding protein in adolescent hepatoma. N Engl J Med 289:1053–1056
Paradinas FJ, Melia WM, Wilkinson ML, Portmann B, Johnson PJ, Murray-Lyon IM, Williams R (1982) High serum vitamin B12 binding capacity as a marker of the fibrolamellar variant of hepatocellular carcinoma. Br Med J (Clin Res Ed) 285:840–842
Lildballe DL, Nguyen KQ, Poulsen SS, Nielsen HO, Nexo E (2011) Haptocorrin as marker of disease progression in fibrolamellar hepatocellular carcinoma. Eur J Surg Oncol 37:72–79
Ward SC, Waxman S (2011) Fibrolamellar carcinoma: a review with focus on genetics and comparison to other malignant primary liver tumors. Semin Liver Dis 31:61–70
Ehrenfried JA, Zhou Z, Thompson JC, Evers BM (1994) Expression of the neurotensin gene in fetal human liver and fibrolamellar carcinoma. Ann Surg 220:484–489; discussion 489–491
McLarney JK, Rucker PT, Bender GN, Goodman ZD, Kashitani N, Ros PR (1999) Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation. Radiographics 19:453–471
Chung YE, Park MS, Park YN, Lee HJ, Seok JY, Yu JS, Kim MJ (2009) Hepatocellular carcinoma variants: radiologic-pathologic correlation. Am J Roentgenol 193:W7–13
Ichikawa T, Federle MP, Grazioli L, Madariaga J, Nalesnik M, Marsh W (1999) Fibrolamellar hepatocellular carcinoma: imaging and pathologic findings in 31 recent cases. Radiology 213:352–361
El-Gazzaz G, Wong W, El-Hadary MK, Gunson BK, Mirza DF, Mayer AD, Buckels JA, McMaster P (2000) Outcome of liver resection and transplantation for fibrolamellar hepatocellular carcinoma. Transpl Int 13(Suppl 1):S406–409
Maniaci V, Davidson BR, Rolles K, Dhillon AP, Hackshaw A, Begent RH, Meyer T (2009) Fibrolamellar hepatocellular carcinoma: prolonged survival with multimodality therapy. Eur J Surg Oncol 35:617–621
Stipa F, Yoon SS, Liau KH, Fong Y, Jarnagin WR, D’Angelica M, Abou-Alfa G, Blumgart LH, DeMatteo RP (2006) Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer 106:1331–1338
Spence RA, Rosen A, Krige JE, Blumgart RL, Temple-Camp CR, Terblanche J (1987) Unresectable fibrolamellar hepatocellular carcinoma treated with intra-arterial lipiodolised doxorubicin. A case report. S Afr Med J 72:701–703
Patt YZ, Hassan MM, Lozano RD, Brown TD, Vauthey JN, Curley SA, Ellis LM (2003) Phase II trial of systemic continuous fluorouracil and subcutaneous recombinant interferon Alfa-2b for treatment of hepatocellular carcinoma. J Clin Oncol 21:421–427
Katzenstein HM, Krailo MD, Malogolowkin MH, Ortega JA, Qu W, Douglass EC, Feusner JH, Reynolds M, Quinn JJ, Newman K, Finegold MJ, Haas JE, Sensel MG, Castleberry RP, Bowman LC (2003) Fibrolamellar hepatocellular carcinoma in children and adolescents. Cancer 97:2006–2012
Hemming AW, Langer B, Sheiner P, Greig PD, Taylor BR (1997) Aggressive surgical management of fibrolamellar hepatocellular carcinoma. J Gastrointest Surg 1:342–346
Dhingra S, Li W, Tan D, Zenali M, Zhang H, Brown RE (2010) Cell cycle biology of fibrolamellar hepatocellular carcinoma. Int J Clin Exp Pathol 3:792–797
Buckley AF, Burgart LJ, Kakar S (2006) Epidermal growth factor receptor expression and gene copy number in fibrolamellar hepatocellular carcinoma. Hum Pathol 37:410–414
Orsatti G, Hytiroglou P, Thung SN, Ishak KG, Paronetto F (1997) Lamellar fibrosis in the fibrolamellar variant of hepatocellular carcinoma: a role for transforming growth factor beta. Liver 17:152–156
Schoedel KE, Tyner VZ, Kim TH, Michalopoulos GK, Mars WM (2003) HGF, MET, and matrix-related proteases in hepatocellular carcinoma, fibrolamellar variant, cirrhotic and normal liver. Mod Pathol 16:14–21
Zenali MJ, Tan D, Li W, Dhingra S, Brown RE (2010) Stemness characteristics of fibrolamellar hepatocellular carcinoma: immunohistochemical analysis with comparisons to conventional hepatocellular carcinoma. Ann Clin Lab Sci 40:126–134
Tanaka K, Honna T, Kitano Y, Kuroda T, Morikawa N, Matsuda H, Kawashima N, Matsuoka K, Miyauchi J (2005) Combined fibrolamellar carcinoma and cholangiocarcinoma exhibiting biphenotypic antigen expression: a case report. J Clin Pathol 58:884–887
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2013 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
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
Download citation
DOI: https://doi.org/10.1007/978-3-642-16037-0_7
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-16036-3
Online ISBN: 978-3-642-16037-0
eBook Packages: MedicineMedicine (R0)