Ultraschall Med 2021; 42(03): 306-313
DOI: 10.1055/a-1110-7124
Original Article

Imaging Features of Fibrolamellar Hepatocellular Carcinoma with Contrast-Enhanced Ultrasound

Bildgebungsmerkmale des fibrolamellären hepatozellulären Karzinoms im kontrastverstärkten Ultraschall
Yi Dong
1   Ultrasound, Zhongshan-Hospital Fudan-University, Shanghai, China
,
Wen-Ping Wang
1   Ultrasound, Zhongshan-Hospital Fudan-University, Shanghai, China
,
Feng Mao
1   Ultrasound, Zhongshan-Hospital Fudan-University, Shanghai, China
,
Qi Zhang
1   Ultrasound, Zhongshan-Hospital Fudan-University, Shanghai, China
,
Daohui Yang
1   Ultrasound, Zhongshan-Hospital Fudan-University, Shanghai, China
,
Andrea Tannapfel
2   Institut für Pathologie, Ruhr-Universität Bochum, Germany
,
Maria Franca Meloni
3   Casa di Cura Igea Interventional Radiology Department Milano, Italy
,
Holger Neye
4   Department of Internal Medicine II, Helios Hospital Emil von Behring, Berlin, Germany
,
Dirk-André Clevert
5   Interdisciplinary Ultrasound-Center, Department of Radiology, University Hospital Munich, München, Germany
,
Christoph F. Dietrich
6   Department Allgemeine Innere Medizin, Kliniken Hirslanden Beau Site, Salem und Permanence, Switzerland
› Author Affiliations

Abstract

Purpose Fibrolamellar hepatocellular carcinoma (f-HCC) is a rare primary liver tumor. Imaging plays an important role in diagnosis. The aim of this retrospective study was to analyze contrast-enhanced ultrasound (CEUS) features of histologically proven f-HCC in comparison to benign focal nodular hyperplasia (FNH).

Materials & Methods 16 patients with histologically proven f-HCC lesions and 30 patients with FNH lesions were retrospectively reviewed regarding CEUS features to determine the malignant or benign nature of the focal liver lesions (FLL). Five radiologists assessed the CEUS enhancement pattern and came to a consensus using the EFSUMB (European Federation of Societies for Ultrasound in Medicine and Biology) guideline criteria.

Results Fibrolamellar hepatocellular carcinoma manifested as a single and huge FLL. On CEUS, f-HCC showed heterogeneous hyperenhancement in the arterial phase and hypoenhancement (16/16, 100 %) in the portal venous and late phases (PVLP) as a sign of malignancy. In contrast to the hypoenhancement of f-HCC in the PVLP, all patients with FNH showed hyperenhancement as the most distinctive feature (P < 0.01). 8 f-HCC lesions showed a central scar as an unenhanced area (8/16, 50.0 %), which could also be detected in 53.3 % (16/30) of FNH lesions (P > 0.05).

Conclusion By analyzing the hypoenhancement in the PVLP, CEUS imaging reliably diagnosed f-HCC as a malignant FLL. CEUS also showed differentiation between f-HCC and FNH lesions, showing similar non-enhanced central scars, whereas f-HCC lesions showed peripheral hyperenhancement in the arterial phase and early washout in the PVLP.

Zusammenfassung

Ziel Das fibrolamelläre hepatozelluläre Karzinom (f-HCC) ist ein seltener primärer Lebertumor. Die Bildgebung spielt bei der Diagnose eine wichtige Rolle. Ziel dieser retrospektiven Studie war es, die Merkmale im kontrastverstärkten Ultraschall (CEUS) bei histologisch nachgewiesenem f-HCC im Vergleich zur benignen fokalen nodulären Hyperplasie (FNH) zu analysieren.

Material und Methoden 16 Patienten mit histologisch nachgewiesenen f-HCC-Läsionen und 30 Patienten mit FNH-Läsionen wurden retrospektiv auf CEUS-Merkmale untersucht, um die maligne oder benigne Natur der fokalen Leberläsionen (FLL) zu bestimmen. 5 Radiologen bewerteten das CEUS-Enhancement-Muster und kamen zu einem Konsens unter Verwendung der Kriterien der EFSUMB (European Federation of Societies for Ultrasound in Medicine and Biology) -Richtlinien.

Ergebnisse Das fibrolamelläre Leberzellkarzinom manifestierte sich als eine einzige und große FLL. Im CEUS zeigte das f-HCC ein heterogenes Hyperenhancement in der arteriellen Phase und ein Hypoenhancement (16/16, 100 %) in der portalvenösen und späten Phase (PVLP) als Marker für Malignität. Im Gegensatz zum Hypoenhancement des f-HCC in der PVLP zeigten alle Patienten mit FNH ein Hyperenhancement als auffälligstes Merkmal (p < 0,01). 8 f-HCC-Läsionen zeigten eine zentrale Narbe als Bereich ohne Anreicherung (8/16; 50,0 %), welche auch bei 53,3 % (16/30) der FNH-Läsionen nachgewiesen werden konnte (p > 0,05).

Schlussfolgerung Durch die Untersuchung des Hypoenhancements in der PVLP konnte die CEUS-Bildgebung f-HCC zuverlässig als maligne FLL diagnostizieren. Außerdem konnte CEUS zwischen f-HCC und FNH-Läsionen differenzieren und zeigte ähnliche zentrale Narben ohne Anreicherung, wobei f-HCC-Läsionen ein peripheres Hyperenhancement in der arteriellen Phase und ein frühes Wash-out in der PVLP zeigten.



Publication History

Received: 06 June 2019

Accepted: 23 December 2019

Article published online:
26 February 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • References

  • 1 Edmondson HA. Differential diagnosis of tumors and tumor-like lesions of liver in infancy and childhood. AMA journal of diseases of children 1956; 91: 168-186
  • 2 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; 46: 372-379
  • 3 Bosman FT, Carneiro F, Hruban RH. et al. WHO Classification of Tumours of the Digestive System. In World Health Organization Classifiation of Tumours. Lyon, France: International Agency for Research on Cancer; 2010
  • 4 Graham RP. Fibrolamellar Carcinoma: What Is New and Why It Matters. Surgical pathology clinics 2018; 11: 377-387
  • 5 Andersen JB. Fibrolamellar hepatocellular carcinoma: a rare but distinct type of liver cancer. Gastroenterology 2015; 148: 707-710
  • 6 Kassahun WT. Contemporary management of fibrolamellar hepatocellular carcinoma: diagnosis, treatment, outcome, prognostic factors, and recent developments. World journal of surgical oncology 2016; 14: 151
  • 7 Dietrich CF, Tannapfel A, Jang HJ. et al. Ultrasound Imaging of Hepatocellular Adenoma Using the New Histology Classification. Ultrasound Med Biol 2019; 45: 1-10
  • 8 Dietrich CF. Contrast-Enhanced Ultrasound of Benign Focal Liver Lesions. Ultraschall in Med 2019; 40: 12-29
  • 9 Dietrich CF, Averkiou M, Nielsen MB. et al. How to perform Contrast-Enhanced Ultrasound (CEUS). Ultrasound Int Open 2018; 4: E2-E15
  • 10 Dong Y, Teufel A, Trojan J. et al. Contrast enhanced ultrasound in mixed hepatocellular cholangiocarcinoma: Case series and review of the literature. Dig Liver Dis 2018; 50: 401-407
  • 11 Strobel D, Seitz K, Blank W. et al. Contrast-enhanced ultrasound for the characterization of focal liver lesions--diagnostic accuracy in clinical practice (DEGUM multicenter trial). Ultraschall in Med 2008; 29: 499-505
  • 12 Ganeshan D, Szklaruk J, Kundra V. et al. Imaging features of fibrolamellar hepatocellular carcinoma. Am J Roentgenol American journal of roentgenology 2014; 202: 544-552
  • 13 Rousseau C, Ronot M, Sibileau E. et al. Central element in liver masses, helpful, or pitfall?. Abdominal imaging 2015; 40: 1904-1925
  • 14 McLarney JK, Rucker PT, Bender GN. et al. Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation. Radiographics: a review publication of the Radiological Society of North America, Inc 1999; 19: 453-471
  • 15 Claudon M, Dietrich CF, Choi BI. et al. Guidelines and good clinical practice recommendations for contrast enhanced ultrasound (CEUS) in the liver--update 2012: a WFUMB-EFSUMB initiative in cooperation with representatives of AFSUMB, AIUM, ASUM, FLAUS and ICUS. Ultraschall in Med 2013; 34: 11-29
  • 16 Dietrich CF, Ignee A, Trojan J. et al. Improved characterisation of histologically proven liver tumours by contrast enhanced ultrasonography during the portal venous and specific late phase of SHU 508A. Gut 2004; 53: 401-405
  • 17 Chiorean L, Cantisani V, Jenssen C. et al. Focal masses in a non-cirrhotic liver: The additional benefit of CEUS over baseline imaging. Eur J Radiol 2015; 84: 1636-1643
  • 18 Liu S, Chan KW, Wang B. et al Fibrolamellar hepatocellular carcinoma. The American journal of gastroenterology 2009; 104: 2617-2624 ; quiz 2625
  • 19 Ichikawa T, Federle MP, Grazioli L. et al. Fibrolamellar hepatocellular carcinoma: pre- and posttherapy evaluation with CT and MR imaging. Radiology 2000; 217: 145-151
  • 20 Lin CC, Yang HM. Fibrolamellar Carcinoma: A Concise Review. Archives of pathology & laboratory medicine 2018; 142: 1141-1145
  • 21 Ichikawa T, Federle MP, Grazioli L. et al. Fibrolamellar hepatocellular carcinoma: imaging and pathologic findings in 31 recent cases. Radiology 1999; 213: 352-361
  • 22 Lee YJ, Lee JM, Lee JS. et al. Hepatocellular carcinoma: diagnostic performance of multidetector CT and MR imaging-a systematic review and meta-analysis. Radiology 2015; 275: 97-109
  • 23 Semelka RC, Nimojan N, Chandana S. et al. MRI features of primary rare malignancies of the liver: A report from four university centres. European radiology 2018; 28: 1529-1539
  • 24 Terzis I, Haritanti A, Economou I. Fibrolamellar hepatocellular carcinoma: a case report with distinct radiological features. Journal of gastrointestinal cancer 2010; 41: 2-5
  • 25 Mandry D, Bressenot A, Galloy MA. et al. Contrast-enhanced ultrasound in fibro-lamellar hepatocellular carcinoma: a case report. Ultraschall in Med 2007; 28: 547-552
  • 26 Chiorean L, Cui XW, Tannapfel A. et al. Benign liver tumors in pediatric patients – Review with emphasis on imaging features. World J Gastroenterol 2015; 21: 8541-8561
  • 27 Meloni MF, Galimberti S, Dietrich CF. et al. Microwave ablation of hepatic tumors with a third generation system: locoregional efficacy in a prospective cohort study with intermediate term follow-up. Z Gastroenterol 2016; 54: 541-547
  • 28 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; 223: 532-539