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Abdominal Radiology

, Volume 43, Issue 12, pp 3340–3348 | Cite as

Fibrolamellar hepatocellular carcinoma: multiphasic CT features of the primary tumor on pre-therapy CT and pattern of distant metastases

  • Dhakshinamoorthy Ganeshan
  • Janio Szklaruk
  • Ahmed Kaseb
  • Ahmad Kattan
  • Khaled M. Elsayes
Article

Abstract

Objective

The aim of our study is to describe the multiphasic CT features of fibrolamellar hepatocellular carcinomas (FLHCCs) and pattern of distant metastases.

Materials and methods

33 patients (mean age 28 years: 17M/16F) with pathologically confirmed FLHCC and pre-treatment multiphasic CT were included in the study. Two abdominal radiologists evaluated the multiphasic CT imaging features of these 33 FLHCC patients in consensus.

Results

Solitary hepatic mass was seen in 67% (22/33). Mean tumor size was 11.3 cm (range 4.6–22 cm). Tumor was well-defined in 64% (21/33). In the pre-contrast CT, 91% (30/33) were hypoattenuating. Central stellate scar was present in 73% (24/33). In FLHCC having central stellate scar, calcification within the central scar was seen in 88% (21/24). In the hepatic arterial phase, 82% (27/33) were hyperattenuating relative to liver. In the portal venous phase, 36% (12/33) were hyperattenuating, 46% (15/33) were isoattenuating, and 18% (6/33) were hypoattenuating. In the delayed phase images, 24% (8/23) were hyperattenuating, 67% (22/33) were isoattenuating, and 9% (3/33) were hypoattenuating. Delayed enhancement of the central stellate scar was seen in 12% (4/33). Distant metastases were seen in 54% (18/33). The most common site of metastases was lungs and was seen in 89% (16/18) of the patients with metastatic disease.

Conclusion

FLHCC typically manifests as a large, solitary mass demonstrating heterogeneous hypervascular enhancement in the arterial phase. Most tend to be isoattenuating in delayed phase. Central stellate scar with internal calcification is a useful imaging feature that can help in the diagnosis of FLHCC. Lungs are the most common site of distant metastases in FLHCC.

Keywords

Fibrolamellar HCC CT Imaging Metastases 

Notes

Compliance with ethical standards

Funding

This study was supported by MD Anderson Cancer Center Support Grant No. NIH/NCI P30 CA016672 from the National Cancer Institute, National Institutes of Health.

Conflict of interest

All authors confirm that there are no relevant conflicts of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the Ethical Standards of the Institutional and/or National Research Committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Being a retrospective study, formal consent was not required. This article does not contain any studies with animals performed by any of the authors.

Informed consent

IRB approval was obtained for a waiver of Informed Consent, because this is a retrospective chart review that involves no diagnostic or therapeutic intervention, as well as no direct patient contact and no patient identifiers. A consent waiver was granted by the Institutional Review Board.

References

  1. 1.
    El-Serag HB, Davila JA (2004) Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population-based study. Hepatology 39(3):798–803CrossRefGoogle Scholar
  2. 2.
    Berman MA, Burnham JA, Sheahan DG (1988) Fibrolamellar carcinoma of the liver: an immunohistochemical study of nineteen cases and a review of the literature. Hum Pathol 19(7):784–794CrossRefGoogle Scholar
  3. 3.
    Mavros MN, Mayo SC, Hyder O, Pawlik TM (2012) A systematic review: treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma. J Am Coll Surg 215(6):820–830CrossRefGoogle Scholar
  4. 4.
    Mayo SC, Mavros MN, Nathan H, et al. (2014) Treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma: a national perspective. J Am Coll Surg 218(2):196–205CrossRefGoogle Scholar
  5. 5.
    Do RK, McErlean A, Ang CS, DeMatteo RP, Abou-Alfa GK (1040) CT and MRI of primary and metastatic fibrolamellar carcinoma: a case series of 37 patients. Br J Radiol 2014(87):20140024Google Scholar
  6. 6.
    Ichikawa T, Federle MP, Grazioli L, Marsh W (2000) Fibrolamellar hepatocellular carcinoma: pre- and posttherapy evaluation with CT and MR imaging. Radiology 217(1):145–151CrossRefGoogle Scholar
  7. 7.
    Ichikawa T, Federle MP, Grazioli L, et al. (1999) Fibrolamellar hepatocellular carcinoma: imaging and pathologic findings in 31 recent cases. Radiology 213(2):352–361CrossRefGoogle Scholar
  8. 8.
    McLarney JK, Rucker PT, Bender GN, et al. (1999) Fibrolamellar carcinoma of the liver: radiologic–pathologic correlation. Radiographics 19(2):453–471CrossRefGoogle Scholar
  9. 9.
    Blachar A, Federle MP, Ferris JV, et al. (2002) Radiologists’ performance in the diagnosis of liver tumors with central scars by using specific CT criteria. Radiology 223(2):532–539CrossRefGoogle Scholar
  10. 10.
    Brancatelli G, Federle MP, Grazioli L, et al. (2001) Focal nodular hyperplasia: CT findings with emphasis on multiphasic helical CT in 78 patients. Radiology 219(1):61–68CrossRefGoogle Scholar
  11. 11.
    Brandt DJ, Johnson CD, Stephens DH, Weiland LH (1988) Imaging of fibrolamellar hepatocellular carcinoma. Am J Roentgenol 151(2):295–299CrossRefGoogle Scholar
  12. 12.
    Friedman AC, Lichtenstein JE, Goodman Z, et al. (1985) Fibrolamellar hepatocellular carcinoma. Radiology 157(3):583–587CrossRefGoogle Scholar
  13. 13.
    Ganeshan D, Szklaruk J, Kundra V, et al. (2014) Imaging features of fibrolamellar hepatocellular carcinoma. Am J Roentgenol 202(3):544–552CrossRefGoogle Scholar
  14. 14.
    Stevens WR, Johnson CD, Stephens DH, Nagorney DM (1995) Fibrolamellar hepatocellular carcinoma: stage at presentation and results of aggressive surgical management. Am J Roentgenol 164(5):1153–1158CrossRefGoogle Scholar
  15. 15.
    Vilgrain V, Boulos L, Vullierme MP, et al. (2000) Imaging of atypical hemangiomas of the liver with pathologic correlation. Radiographics 20(2):379–397CrossRefGoogle Scholar
  16. 16.
    Vivekanandan P, Torbenson M (2008) Epigenetic instability is rare in fibrolamellar carcinomas but common in viral-associated hepatocellular carcinomas. Mod Pathol 21(6):670–675CrossRefGoogle Scholar
  17. 17.
    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(1):61–70CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Diagnostic Imaging, Body Imaging Section, Unit 1473The University of Texas MD Anderson Cancer CenterHoustonUSA
  2. 2.Department of Gastrointestinal Medical OncologyThe University of Texas MD Anderson Cancer CenterHoustonUSA

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