Skip to main content

Advertisement

Log in

Hepatic hemangioendothelioma: CT, MR, and FDG-PET-CT in 67 patients—a bi-institutional comprehensive cancer center review

  • Oncology
  • Published:
European Radiology Aims and scope Submit manuscript

Abstract

Objective

To evaluate the imaging features of hepatic epithelioid hemangioendothelioma (HEH) on multiphasic CT, MR, and FDG-PET-CT.

Methods

Bi-institutional review identified 67 adults (mean age, 47 years; 23 M/44 F) with pathologically proven HEH and pretreatment multiphasic CT (n = 67) and/or MR (n = 30) and/or FDG-PET-CT (n = 13).

Results

HEHs were multifocal in 88% (59/67). Mean size of the dominant mass was 4.1 cm (range, 1.4–19 cm). The tumors were located in the peripheral, subcapsular regions of the liver in 96% (64/67). Capsular retraction was present in 81% (54/67 cases) and tumors were coalescent in 61% (41/67). HEH demonstrated peripheral ring enhancement on arterial phase imaging in 33% (21/64) and target appearance on the portal venous phase in 69% (46/67). Persistent peripheral enhancement on the delayed phase was seen in 49% (31/63). On MR, multilayered target appearance was seen on the T2-weighted sequences in 67% (20/30) and on the diffusion-weighted sequences in 61% (11/18). Target appearance on hepatobiliary phase of MRI was seen in 57% (4/7). On pre-therapy FDG-PET-CT, increased FDG uptake above the background liver parenchyma was seen in 62% (8/13).

Conclusion

HEHs typically manifest as multifocal, coalescent hepatic nodules in peripheral subcapsular location, with associated capsular retraction. Peripheral arterial ring enhancement and target appearance on portal venous phase are commonly seen on CT. Similarly, multilayered target appearance correlating with its histopathological composition is typically seen on multiple sequences of MR including T2-weighted, diffusion-weighted, and dynamic contrast-enhanced multiphasic MR.

Key Points

• Hepatic epithelioid hemangioendotheliomas manifest on CT and MR as multifocal, coalescent hepatic nodules in peripheral subcapsular location, with associated capsular retraction.

• Enhancement pattern on contrast-enhanced CT and MR can vary but peripheral ring enhancement on arterial phase and target appearance on portal venous phase are commonly seen.

• Retrospective two-center study showed that cross-sectional imaging may help in the diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

Abbreviations

CT:

Computed tomography

FDG-PET-CT:

Fluoro-deoxy-glucose positron emission tomography

HEH:

Hepatic epithelioid hemangioendothelioma

HIPAA:

Health Insurance and Portability and Accountability Act

MR:

Magnetic resonance imaging

References

  1. Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M (2014) Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor. Oncol Rev 8:259

    Article  Google Scholar 

  2. Woodall CE, Scoggins CR, Lewis AM, McMasters KM, Martin RC (2008) Hepatic malignant epithelioid hemangioendothelioma: a case report and review of the literature. Am Surg 74:64–68

    PubMed  Google Scholar 

  3. Mehrabi A, Kashfi A, Fonouni H et al (2006) Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer 107:2108–2121

    Article  Google Scholar 

  4. Uchimura K, Nakamuta M, Osoegawa M et al (2001) Hepatic epithelioid hemangioendothelioma. J Clin Gastroenterol 32:431–434

    Article  CAS  Google Scholar 

  5. Makhlouf HR, Ishak KG, Goodman ZD (1999) Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer 85:562–582

    Article  CAS  Google Scholar 

  6. Taratuto AL, Zurbriggen G, Sevlever G, Saccoliti M (1988) Epithelioid hemangioendothelioma of the central nervous system. Immunohistochemical and ultrastructural observations of a pediatric case. Pediatr Neurosci 14:11–14

    Article  CAS  Google Scholar 

  7. Studer LL, Selby DM (2018) Hepatic epithelioid hemangioendothelioma. Arch Pathol Lab Med 142:263–267

    Article  CAS  Google Scholar 

  8. Mascarelli PE, Iredell JR, Maggi RG, Weinberg G, Breitschwerdt EB (2011) Bartonella species bacteremia in two patients with epithelioid hemangioendothelioma. J Clin Microbiol 49:4006–4012

    Article  Google Scholar 

  9. Mistry AM, Gorden DL, Busler JF, Coogan AC, Kelly BS (2012) Diagnostic and therapeutic challenges in hepatic epithelioid hemangioendothelioma. J Gastrointest Cancer 43:521–525

    Article  Google Scholar 

  10. Woller SC, Boschert ME, Hutson WR (2005) Hepatic epithelioid hemangioendothelioma presenting as liver infarction. Clin Gastroenterol Hepatol 3:xx

    Article  Google Scholar 

  11. Walsh MM, Hytiroglou P, Thung SN et al (1998) Epithelioid hemangioendothelioma of the liver mimicking Budd-Chiari syndrome. Arch Pathol Lab Med 122:846–848

    CAS  PubMed  Google Scholar 

  12. Fan F, Yang X, Zhu B, Zhang Y (2013) Clinical and radiological characteristics of Chinese patients with hepatic epithelioid hemangioendothelioma. Ann Saudi Med 33:334–338

    Article  Google Scholar 

  13. Gan LU, Chang R, Jin H, Yang LI (2016) Typical CT and MRI signs of hepatic epithelioid hemangioendothelioma. Oncol Lett 11:1699–1706

    Article  CAS  Google Scholar 

  14. Kim EH, Rha SE, Lee YJ, Yoo IeR, Jung ES, Byun JY (2015) CT and MR imaging findings of hepatic epithelioid hemangioendotheliomas: emphasis on single nodular type. Abdom Imaging 40:500–509

  15. Lee JH, Jeong WK, Kim YK et al (2017) Magnetic resonance findings of hepatic epithelioid hemangioendothelioma: emphasis on hepatobiliary phase using Gd-EOB-DTPA. Abdom Radiol (NY) 42:2261–2271

    Article  Google Scholar 

  16. Miller WJ, Dodd GD 3rd, Federle MP, Baron RL (1992) Epithelioid hemangioendothelioma of the liver: imaging findings with pathologic correlation. AJR Am J Roentgenol 159:53–57

    Article  CAS  Google Scholar 

  17. Paolantonio P, Laghi A, Vanzulli A et al (2014) MRI of hepatic epithelioid hemangioendothelioma (HEH). J Magn Reson Imaging 40:552–558

    Article  Google Scholar 

  18. Zhou L, Cui MY, Xiong J et al (2015) Spectrum of appearances on CT and MRI of hepatic epithelioid hemangioendothelioma. BMC Gastroenterol 15:69

    Article  Google Scholar 

  19. Giardino A, Miller FH, Kalb B et al (2016) Hepatic epithelioid hemangioendothelioma: a report from three university centers. Radiol Bras 49:288–294

    Article  Google Scholar 

  20. Ganeshan D, Szklaruk J, Kaseb A, Kattan A, Elsayes KM (2018) Fibrolamellar hepatocellular carcinoma: multiphasic CT features of the primary tumor on pre-therapy CT and pattern of distant metastases. Abdom Radiol (NY) 43:3340–3348

    Article  Google Scholar 

  21. Lyburn ID, Torreggiani WC, Harris AC et al (2003) Hepatic epithelioid hemangioendothelioma: sonographic, CT, and MR imaging appearances. AJR Am J Roentgenol 180:1359–1364

    Article  Google Scholar 

  22. Mamone G, Miraglia R (2019) The “target sign” and the “lollipop sign” in hepatic epithelioid hemangioendothelioma. Abdom Radiol (NY) 44:1617–1620

    Article  Google Scholar 

  23. Alomari AI (2006) The lollipop sign: a new cross-sectional sign of hepatic epithelioid hemangioendothelioma. Eur J Radiol 59:460–464

    Article  Google Scholar 

  24. Dong Y, Wang WP, Cantisani V et al (2016) Contrast-enhanced ultrasound of histologically proven hepatic epithelioid hemangioendothelioma. World J Gastroenterol 22:4741–4749

    Article  Google Scholar 

  25. Lin J, Ji Y (2010) CT and MRI diagnosis of hepatic epithelioid hemangioendothelioma. Hepatobiliary Pancreat Dis Int 9:154–158

    PubMed  Google Scholar 

  26. Van Beers B, Roche A, Mathieu D et al (1992) Epithelioid hemangioendothelioma of the liver: MR and CT findings. J Comput Assist Tomogr 16:420–424

    Article  Google Scholar 

  27. Furui S, Itai Y, Ohtomo K et al (1989) Hepatic epithelioid hemangioendothelioma: report of five cases. Radiology 171:63–68

    Article  CAS  Google Scholar 

  28. Giancipoli RG, Monti S, Basturk O et al (2018) Complete metabolic response to therapy of hepatic epithelioid hemangioendothelioma evaluated with 18F-fluorodeoxyglucose positron emission tomography/contrast-enhanced computed tomography: a CARE case report. Medicine (Baltimore) 97:e12795

    Article  Google Scholar 

  29. Kitapci MT, Akkas BE, Gullu I, Sokmensuer C (2010) FDG-PET/CT in the evaluation of epithelioid hemangioendothelioma of the liver: the role of dual-time-point imaging. A case presentation and review of the literature. Ann Nucl Med 24:549–553

    Article  Google Scholar 

  30. Suga K, Kawakami Y, Hiyama A, Hori K (2009) F-18 FDG PET/CT monitoring of radiation therapeutic effect in hepatic epithelioid hemangioendothelioma. Clin Nucl Med 34:199–202

    Article  Google Scholar 

  31. Dong A, Dong H, Wang Y, Gong J, Lu J, Zuo C (2013) MRI and FDG PET/CT findings of hepatic epithelioid hemangioendothelioma. Clin Nucl Med 38:e66–e73

    Article  Google Scholar 

  32. Kim R, Lee JM, Shin CI et al (2016) Differentiation of intrahepatic mass-forming cholangiocarcinoma from hepatocellular carcinoma on gadoxetic acid-enhanced liver MR imaging. Eur Radiol 26:1808–1817

    Article  Google Scholar 

  33. Jeong HT, Kim MJ, Chung YE, Choi JY, Park YN, Kim KW (2013) Gadoxetate disodium-enhanced MRI of mass-forming intrahepatic cholangiocarcinomas: imaging-histologic correlation. AJR Am J Roentgenol 201:W603–W611

    Article  Google Scholar 

  34. Park HJ, Kim YK, Park MJ, Lee WJ (2013) Small intrahepatic mass-forming cholangiocarcinoma: target sign on diffusion-weighted imaging for differentiation from hepatocellular carcinoma. Abdom Imaging 38:793–801

    Article  Google Scholar 

  35. Mamone G, Marrone G, Caruso S et al (2015) Intrahepatic mass-forming cholangiocarcinoma: enhancement pattern on Gd-BOPTA-MRI with emphasis of hepatobiliary phase. Abdom Imaging 40:2313–2322

    Article  Google Scholar 

Download references

Funding

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

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Dhakshinamoorthy Ganeshan.

Ethics declarations

Guarantor

The scientific guarantor of this publication is Khaled Elsayes.

Conflict of interest

The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article.

Statistics and biometry

No complex statistical methods were necessary for this paper.

Informed consent

Written informed consent was waived by the Institutional Review Board.

Ethical approval

Institutional Review Board approval was obtained.

Methodology

• Retrospective

• Multicenter study

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Ganeshan, D., Pickhardt, P.J., Morani, A.C. et al. Hepatic hemangioendothelioma: CT, MR, and FDG-PET-CT in 67 patients—a bi-institutional comprehensive cancer center review. Eur Radiol 30, 2435–2442 (2020). https://doi.org/10.1007/s00330-019-06637-3

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00330-019-06637-3

Keywords

Navigation