Fibrolamellar Carcinoma Questions & Answers

1. Smith M, Tomboc PJ, Markovich B. Fibrolamellar Hepatocellular Carcinoma. Exp Clin Immunogenet. 2022 Jan. 7 (1):1-84. [QxMD MEDLINE Link]. [Full Text].

2. Honeyman JN, Simon EP, Robine N, Chiaroni-Clarke R, Darcy DG, Lim II, et al. Detection of a recurrent DNAJB1-PRKACA chimeric transcript in fibrolamellar hepatocellular carcinoma. Science. 2014 Feb 28. 343 (6174):1010-4. [QxMD MEDLINE Link]. [Full Text].

3. Cornella H, Alsinet C, Sayols S, et al. Unique genomic profile of fibrolamellar hepatocellular carcinoma. Gastroenterology. 2015 Apr. 148 (4):806-18.e10. [QxMD MEDLINE Link]. [Full Text].

4. Tomasini MD, Wang Y, Karamafrooz A, Li G, Beuming T, Gao J, et al. Conformational Landscape of the PRKACA-DNAJB1 Chimeric Kinase, the Driver for Fibrolamellar Hepatocellular Carcinoma. Sci Rep. 2018 Jan 15. 8 (1):720. [QxMD MEDLINE Link]. [Full Text].

5. Graham RP, Jin L, Knutson DL, Kloft-Nelson SM, Greipp PT, Waldburger N, et al. DNAJB1-PRKACA is specific for fibrolamellar carcinoma. Mod Pathol. 2015 Jun. 28 (6):822-9. [QxMD MEDLINE Link]. [Full Text].

6. Griffith OL, Griffith M, Krysiak K, Magrini V, Ramu A, Skidmore ZL, et al. A genomic case study of mixed fibrolamellar hepatocellular carcinoma. Ann Oncol. 2016 Jun. 27 (6):1148-54. [QxMD MEDLINE Link]. [Full Text].

7. Riggle KM, Turnham R, Scott JD, Yeung RS, Riehle KJ. Fibrolamellar Hepatocellular Carcinoma: Mechanistic Distinction From Adult Hepatocellular Carcinoma. Pediatr Blood Cancer. 2016 Jul. 63 (7):1163-7. [QxMD MEDLINE Link]. [Full Text].

8. Dinh TA, Vitucci EC, Wauthier E, Graham RP, Pitman WA, Oikawa T, et al. Comprehensive analysis of The Cancer Genome Atlas reveals a unique gene and non-coding RNA signature of fibrolamellar carcinoma. Sci Rep. 2017 Mar 17. 7:44653. [QxMD MEDLINE Link]. [Full Text].

9. Kakar S, Burgart LJ, Batts KP, et al. Clinicopathologic features and survival in fibrolamellar carcinoma: comparison with conventional hepatocellular carcinoma with and without cirrhosis. Mod Pathol. 2005 Nov. 18(11):1417-23. [QxMD MEDLINE Link].

10. Mayo SC, Mavros MN, Nathan H, Cosgrove D, Herman JM, Kamel I, et al. Treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma: a national perspective. J Am Coll Surg. 2014 Feb. 218(2):196-205. [QxMD MEDLINE Link].

11. Weeda VB, Murawski M, McCabe AJ, Maibach R, Brugières L, Roebuck D, et al. Fibrolamellar variant of hepatocellular carcinoma does not have a better survival than conventional hepatocellular carcinoma--results and treatment recommendations from the Childhood Liver Tumour Strategy Group (SIOPEL) experience. Eur J Cancer. 2013 Aug. 49 (12):2698-704. [QxMD MEDLINE Link]. [Full Text].

12. Edmondson HA. Differential diagnosis of tumors and tumor-like lesions of liver in infancy and childhood. AMA J Dis Child. 1956 Feb. 91(2):168-86. [QxMD MEDLINE Link].

13. Craig JR, Peters RL, Edmondson HA, et al. Fibrolamellar carcinoma of the liver: a tumor of adolescents and young adults with distinctive clinico-pathologic features. Cancer. 1980 Jul 15. 46(2):372-9. [QxMD MEDLINE Link].

14. Berman MM, Libbey NP, Foster JH. Hepatocellular carcinoma. Polygonal cell type with fibrous stroma--an atypical variant with a favorable prognosis. Cancer. 1980 Sep 15. 46(6):1448-55. [QxMD MEDLINE Link].

15. Farhi DC, Shikes RH, Murari PJ, et al. Hepatocellular carcinoma in young people. Cancer. 1983 Oct 15. 52(8):1516-25. [QxMD MEDLINE Link].

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18. Torbenson M. Review of the clinicopathologic features of fibrolamellar carcinoma. Adv Anat Pathol. 2007 May. 14(3):217-23. [QxMD MEDLINE Link].

19. Darcy DG, Chiaroni-Clarke R, Murphy JM, Honeyman JN, Bhanot U, LaQuaglia MP, et al. The genomic landscape of fibrolamellar hepatocellular carcinoma: whole genome sequencing of ten patients. Oncotarget. 2015 Jan 20. 6 (2):755-70. [QxMD MEDLINE Link]. [Full Text].

20. El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population-based study. Hepatology. 2004 Mar. 39(3):798-803. [QxMD MEDLINE Link].

21. Arista-Nasr J, Gutierrez-Villalobos L, Nuncio J, et al. Fibrolamellar hepatocellular carcinoma in mexican patients. Pathol Oncol Res. 2002. 8(2):133-7. [QxMD MEDLINE Link].

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23. Haas JE, Muczynski KA, Krailo M, et al. Histopathology and prognosis in childhood hepatoblastoma and hepatocarcinoma. Cancer. 1989 Sep 1. 64(5):1082-95. [QxMD MEDLINE Link].

24. Katzenstein HM, Krailo MD, Malogolowkin MH, et al. Fibrolamellar hepatocellular carcinoma in children and adolescents. Cancer. 2003 Apr 15. 97(8):2006-12. [QxMD MEDLINE Link].

25. Nagorney DM, Adson MA, Weiland LH, et al. Fibrolamellar hepatoma. Am J Surg. 1985 Jan. 149(1):113-9. [QxMD MEDLINE Link].

26. Sempokuya T, Forlemu A, Azawi M, Silangcruz K, Khoury N, Ma J, et al. Survival characteristics of fibrolamellar hepatocellular carcinoma: A Surveillance, Epidemiology, and End Results database study. World J Clin Oncol. 2022 May 24. 13 (5):352-365. [QxMD MEDLINE Link]. [Full Text].

27. Groeschl RT, Miura JT, Wong RK, Bloomston M, Lidsky ML, Clary BM, et al. Multi-institutional analysis of recurrence and survival after hepatectomy for fibrolamellar carcinoma. J Surg Oncol. 2014 Sep. 110(4):412-5. [QxMD MEDLINE Link].

28. Saab S, Yao F. Fibrolamellar hepatocellular carcinoma. Case reports and a review of the literature. Dig Dis Sci. 1996 Oct. 41(10):1981-5. [QxMD MEDLINE Link].

29. Hemming AW, Langer B, Sheiner P, et al. Aggressive surgical management of fibrolamellar hepatocellular carcinoma. J Gastrointest Surg. 1997 Jul-Aug. 1(4):342-6. [QxMD MEDLINE Link].

30. El-Gazzaz G, Wong W, El-Hadary MK, et al. Outcome of liver resection and transplantation for fibrolamellar hepatocellular carcinoma. Transpl Int. 2000. 13 Suppl 1:S406-9. [QxMD MEDLINE Link].

31. Kanai T, Takabayashi T, Kawano Y, et al. A case of postoperative recurrence of fibrolamellar hepatocellular carcinoma with increased vitamin B12 binding capacity in a young Japanese female. Jpn J Clin Oncol. 2004 Jun. 34(6):346-51. [QxMD MEDLINE Link].

32. Debray D, Pariente D, Fabre M, et al. Fibrolamellar hepatocellular carcinoma: report of a case mimicking a liver abscess. J Pediatr Gastroenterol Nutr. 1994 Nov. 19(4):468-72. [QxMD MEDLINE Link].

33. Soyer P, Roche A, Levesque M. Fibrolamellar hepatocellular carcinoma presenting with obstructive jaundice. A report of two cases. Eur J Radiol. 1991 Nov-Dec. 13(3):196-8. [QxMD MEDLINE Link].

34. Eckstein RP, Bambach CP, Stiel D, et al. Fibrolamellar carcinoma as a cause of bile duct obstruction. Pathology. 1988 Oct. 20(4):326-31. [QxMD MEDLINE Link].

35. Agarwal VR, Takayama K, Van Wyk JJ, et al. Molecular basis of severe gynecomastia associated with aromatase expression in a fibrolamellar hepatocellular carcinoma. J Clin Endocrinol Metab. 1998 May. 83(5):1797-800. [QxMD MEDLINE Link].

36. McCloskey JJ, Germain-Lee EL, Perman JA, et al. Gynecomastia as a presenting sign of fibrolamellar carcinoma of the liver. Pediatrics. 1988 Sep. 82(3):379-82. [QxMD MEDLINE Link].

37. Buetow PC, Pantongrag-Brown L, Buck JL, et al. Focal nodular hyperplasia of the liver: radiologic-pathologic correlation. Radiographics. 1996 Mar. 16(2):369-88. [QxMD MEDLINE Link].

38. Farhi DC, Shikes RH, Silverberg SG. Ultrastructure of fibrolamellar oncocytic hepatoma. Cancer. 1982 Aug 15. 50(4):702-9. [QxMD MEDLINE Link].

39. Saul SH, Titelbaum DS, Gansler TS, et al. The fibrolamellar variant of hepatocellular carcinoma. Its association with focal nodular hyperplasia. Cancer. 1987 Dec 15. 60(12):3049-55. [QxMD MEDLINE Link].

40. Imkie M, Myers SA, Li Y, et al. Fibrolamellar hepatocellular carcinoma arising in a background of focal nodular hyperplasia: a report of 2 cases. J Reprod Med. 2005 Aug. 50(8):633-7. [QxMD MEDLINE Link].

41. Vecchio FM, Fabiano A, Ghirlanda G, et al. Fibrolamellar carcinoma of the liver: the malignant counterpart of focal nodular hyperplasia with oncocytic change. Am J Clin Pathol. 1984 Apr. 81(4):521-6. [QxMD MEDLINE Link].

42. Okada K, Kim YI, Nakashima K, et al. Fibrolamellar hepatocellular carcinoma coexistent with a hepatocellular carcinoma of common type: report of a case. Surg Today. 1993. 23(7):626-31. [QxMD MEDLINE Link].

43. Okano A, Hajiro K, Takakuwa H, et al. Fibrolamellar carcinoma of the liver with a mixture of ordinary hepatocellular carcinoma: a case report. Am J Gastroenterol. 1998 Jul. 93(7):1144-5. [QxMD MEDLINE Link].

44. Seitz G, Zimmermann A, Friess H, Büchler MW. Adult-type hepatocellular carcinoma in the center of a fibrolamellar hepatocellular carcinoma. Hum Pathol. 2002 Jul. 33(7):765-9. [QxMD MEDLINE Link].

45. Morise Z, Sugioka A, Mizoguchi Y, et al. Fibrolamellar carcinoma of the liver in a Japanese hepatitis B virus carrier. J Gastroenterol Hepatol. 2005 Jul. 20(7):1136-8. [QxMD MEDLINE Link].

46. Stipa F, Yoon SS, Liau KH, et al. Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer. 2006 Mar 15. 106(6):1331-8. [QxMD MEDLINE Link].

47. Yamamoto H, Watanabe K, Nagata M, et al. Transformation of fibrolamellar carcinoma to common hepatocellular carcinoma in the recurrent lesions of the rectum and the residual liver: a case report. Jpn J Clin Oncol. 1999 Sep. 29(9):445-7. [QxMD MEDLINE Link].

48. Chang YC, Dai YC, Chow NH. Fibrolamellar hepatocellular carcinoma with a recurrence of classic hepatocellular carcinoma: a case report and review of Oriental cases. Hepatogastroenterology. 2003 Sep-Oct. 50(53):1637-40. [QxMD MEDLINE Link].

49. Tanaka K, Honna T, Kitano Y, et al. Combined fibrolamellar carcinoma and cholangiocarcinoma exhibiting biphenotypic antigen expression: a case report. J Clin Pathol. 2005 Aug. 58(8):884-7. [QxMD MEDLINE Link].

50. Ichikawa T, Federle MP, Grazioli L, et al. Fibrolamellar hepatocellular carcinoma: pre- and posttherapy evaluation with CT and MR imaging. Radiology. 2000 Oct. 217(1):145-51. [QxMD MEDLINE Link].

51. Paradinas FJ, Melia WM, Wilkinson ML, et al. High serum vitamin B12 binding capacity as a marker of the fibrolamellar variant of hepatocellular carcinoma. Br Med J (Clin Res Ed). 1982 Sep 25. 285(6345):840-2. [QxMD MEDLINE Link].

52. Sheppard KJ, Bradbury DA, Davies JM, et al. High serum vitamin B12 binding capacity as a marker of the fibrolamellar variant of hepatocellular carcinoma. Br Med J (Clin Res Ed). 1983 Jan 1. 286(6358):57. [QxMD MEDLINE Link].

53. Collier NA, Weinbren K, Bloom SR, et al. Neurotensin secretion by fibrolamellar carcinoma of the liver. Lancet. 1984 Mar 10. 1(8376):538-40. [QxMD MEDLINE Link].

54. Brandt DJ, Johnson CD, Stephens DH, et al. Imaging of fibrolamellar hepatocellular carcinoma. AJR Am J Roentgenol. 1988 Aug. 151(2):295-9. [QxMD MEDLINE Link].

55. Corrigan K, Semelka RC. Dynamic contrast-enhanced MR imaging of fibrolamellar hepatocellular carcinoma. Abdom Imaging. 1995 Mar-Apr. 20(2):122-5. [QxMD MEDLINE Link].

56. Gabata T, Matsui O, Kadoya M, et al. Delayed MR imaging of the liver: correlation of delayed enhancement of hepatic tumors and pathologic appearance. Abdom Imaging. 1998 May-Jun. 23(3):309-13. [QxMD MEDLINE Link].

57. Soyer P, Roche A, Levesque M, et al. CT of fibrolamellar hepatocellular carcinoma. J Comput Assist Tomogr. 1991 Jul-Aug. 15(4):533-8. [QxMD MEDLINE Link].

58. Soyer P, Bluemke DA, Vissuzaine C, et al. CT of hepatic tumors: prevalence and specificity of retraction of the adjacent liver capsule. AJR Am J Roentgenol. 1994 May. 162(5):1119-22. [QxMD MEDLINE Link].

59. Blachar A, Federle MP, Ferris JV, et al. Radiologists' performance in the diagnosis of liver tumors with central scars by using specific CT criteria. Radiology. 2002 May. 223(2):532-9. [QxMD MEDLINE Link].

60. Titelbaum DS, Hatabu H, Schiebler ML, et al. Fibrolamellar hepatocellular carcinoma: MR appearance. J Comput Assist Tomogr. 1988 Jul-Aug. 12(4):588-91. [QxMD MEDLINE Link].

61. Hamrick-Turner JE, Shipkey FH, Cranston PE. Fibrolamellar hepatocellular carcinoma: MR appearance mimicking focal nodular hyperplasia. J Comput Assist Tomogr. 1994 Mar-Apr. 18(2):301-4. [QxMD MEDLINE Link].

62. Hui MS, Choi WM, Perng HL, et al. Fibrolamellar hepatocellular carcinoma--an atypical MR manifestation. Hepatogastroenterology. 1998 Mar-Apr. 45(20):514-7. [QxMD MEDLINE Link].

63. Adam A, Gibson RN, Soreide O, et al. The radiology of fibrolamellar hepatoma. Clin Radiol. 1986 Jul. 37(4):355-8. [QxMD MEDLINE Link].

64. Miller WJ, Federle MP, Campbell WL. Diagnosis and staging of hepatocellular carcinoma: comparison of CT and sonography in 36 liver transplantation patients. AJR Am J Roentgenol. 1991 Aug. 157(2):303-6. [QxMD MEDLINE Link].

65. Stevens WR, Johnson CD, Stephens DH, et al. Fibrolamellar hepatocellular carcinoma: stage at presentation and results of aggressive surgical management. AJR Am J Roentgenol. 1995 May. 164(5):1153-8. [QxMD MEDLINE Link].

66. Stevens WR, Johnson CD, Stephens DH, et al. CT findings in hepatocellular carcinoma: correlation of tumor characteristics with causative factors, tumor size, and histologic tumor grade. Radiology. 1994 May. 191(2):531-7. [QxMD MEDLINE Link].

67. Trojan J, Schroeder O, Raedle J, et al. Fluorine-18 FDG positron emission tomography for imaging of hepatocellular carcinoma. Am J Gastroenterol. 1999 Nov. 94(11):3314-9. [QxMD MEDLINE Link].

68. von Falck C, Rodt T, Shin HO, et al. F-18 FDG PET imaging of fibrolamellar hepatocellular carcinoma. Clin Nucl Med. 2008 Sep. 33(9):633-4. [QxMD MEDLINE Link].

69. Liu S, Wah Chan K, Tong J, Wang Y, Wang B, Qiao L. PET-CT scan is a valuable modality in the diagnosis of fibrolamellar hepatocellular carcinoma: a case report and a summary of recent literature. QJM. 2011 Jun. 104(6):477-83. [QxMD MEDLINE Link].

70. Crowe A, Knight CS, Jhala D, Bynon SJ, Jhala NC. Diagnosis of metastatic fibrolamellar hepatocellular carcinoma by endoscopic ultrasound-guided fine needle aspiration. Cytojournal. 2011 Jan 31. 8:2. [QxMD MEDLINE Link]. [Full Text].

71. Suen KC, Magee JF, Halparin LS, et al. Fine needle aspiration cytology of fibrolamellar hepatocellular carcinoma. Acta Cytol. 1985 Sep-Oct. 29(5):867-72. [QxMD MEDLINE Link].

72. Davenport RD. Cytologic diagnosis of fibrolamellar carcinoma of the liver by fine-needle aspiration. Diagn Cytopathol. 1990. 6(4):275-9. [QxMD MEDLINE Link].

73. Perez-Guillermo M, Masgrau NA, García-Solano J, et al. Cytologic aspect of fibrolamellar hepatocellular carcinoma in fine-needle aspirates. Diagn Cytopathol. 1999 Sep. 21(3):180-7. [QxMD MEDLINE Link].

74. Kojiro M. Histopathology of liver cancers. Best Pract Res Clin Gastroenterol. 2005 Feb. 19(1):39-62. [QxMD MEDLINE Link].

75. Mansouri D, Van Nhieu JT, Couanet D, et al. Fibrolamellar hepatocellular carcinoma: a case report with cytological features in a sixteen-year-old girl. Diagn Cytopathol. 2006 Aug. 34(8):568-71. [QxMD MEDLINE Link].

76. Kassahun WT. Contemporary management of fibrolamellar hepatocellular carcinoma: diagnosis, treatment, outcome, prognostic factors, and recent developments. World J Surg Oncol. 2016 May 23. 14 (1):151. [QxMD MEDLINE Link]. [Full Text].

77. Pinna AD, Iwatsuki S, Lee RG, et al. Treatment of fibrolamellar hepatoma with subtotal hepatectomy or transplantation. Hepatology. 1997 Oct. 26(4):877-83. [QxMD MEDLINE Link].

78. Ichikawa T, Federle MP, Grazioli L, et al. Fibrolamellar hepatocellular carcinoma: imaging and pathologic findings in 31 recent cases. Radiology. 1999 Nov. 213(2):352-61. [QxMD MEDLINE Link].

79. Pan Y, Wang ZM, Mou LJ, et al. Focal nodular hyperplasia of the liver: pathological analysis of 11 cases. Hepatobiliary Pancreat Dis Int. 2004 May. 3(2):199-203. [QxMD MEDLINE Link].

80. Berman MA, Burnham JA, Sheahan DG. Fibrolamellar carcinoma of the liver: an immunohistochemical study of nineteen cases and a review of the literature. Hum Pathol. 1988 Jul. 19(7):784-94. [QxMD MEDLINE Link].

81. Vasdev N, Nayak NC. CD99 expression in hepatocellular carcinoma: an immunohistochemical study in the fibrolamellar and common variant of the tumour. Indian J Pathol Microbiol. 2003 Oct. 46(4):625-9. [QxMD MEDLINE Link].

82. Polychronidis G, Murtha-Lemekhova A, Fuchs J, Karathanasi E, Hoffmann K. A Multidisciplinary Approach to the Management of Fibrolamellar Carcinoma: Current Perspectives and Future Prospects. Onco Targets Ther. 2022. 15:1095-1103. [QxMD MEDLINE Link]. [Full Text].

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Michael A Choti, MD, MBA, FACS Chief of Surgery, Banner MD Anderson Cancer Center

Michael A Choti, MD, MBA, FACS is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Surgeons, Americas Hepato-Pancreato-Biliary Association, American Society of Clinical Oncology, American Surgical Association, Association for Academic Surgery, International Hepato-Pancreato-Biliary Association, Society for Surgery of the Alimentary Tract, Society of Surgical Oncology, Society of University Surgeons

Disclosure: Nothing to disclose.

Rajesh Ramanathan, MD Surgical Oncologist, Banner MD Anderson Cancer Center, Arizona

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

N Joseph Espat, MD, MS, FACS Harold J Wanebo Professor of Surgery, Assistant Dean of Clinical Affairs, Boston University School of Medicine; Chairman, Department of Surgery, Director, Adele R Decof Cancer Center, Roger Williams Medical Center

N Joseph Espat, MD, MS, FACS is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American College of Surgeons, American Medical Association, American Society for Parenteral and Enteral Nutrition, American Society of Clinical Oncology, Americas Hepato-Pancreato-Biliary Association, Association for Academic Surgery, Central Surgical Association, Chicago Medical Society, International Hepato-Pancreato-Biliary Association, Pancreas Club, Sigma Xi, The Scientific Research Honor Society, Society for Leukocyte Biology, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, Society of Surgical Oncology, Society of University Surgeons, Southeastern Surgical Congress, Southern Medical Association, Surgical Infection Society

Disclosure: Nothing to disclose.

Lodovico Balducci, MD Professor, Oncology Fellowship Director, Department of Internal Medicine, Division of Adult Oncology, H Lee Moffitt Cancer Center and Research Institute, University of South Florida Morsani College of Medicine

Lodovico Balducci, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association for Cancer Research, American College of Physicians, American Geriatrics Society, American Society of Hematology, New York Academy of Sciences, American Society of Clinical Oncology, Southern Society for Clinical Investigation, International Society for Experimental Hematology, American Federation for Clinical Research, American Society of Breast Disease

Disclosure: Nothing to disclose.

Hari Nathan, MD House Staff, Department of Surgery, The Johns Hopkins Hospital; Postdoctoral Fellow, Department of Surgery, The Johns Hopkins University School of Medicine

Hari Nathan, MD is a member of the following medical societies: American College of Surgeons, Americas Hepato-Pancreato-Biliary Association, Association for Academic Surgery, Society for Surgery of the Alimentary Tract

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