Skip to main content
SpringerLink
Log in

Treatment and Prognosis of Hepatic Epithelioid Hemangioendothelioma: A SEER Database Analysis

  • Original Scientific Report
  • Published:
World Journal of Surgery Aims and scope Submit manuscript

Abstract

Background

Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor that can affect multiple organs. Little is known about the pathophysiology, clinical course and management of this disease. The aim of this study is to determine survival rates and elucidate the role of various prognostic factors and therapeutic modalities as compared to surgery on patients with HEH.

Methods

A retrospective analysis on patients diagnosed with HEH between 2004 and 2016 was performed utilizing the SEER database. Kaplan–Meier curves were constructed to determine overall and cancer-specific survival, and the log-rank test was used to compare between groups. To explore prognostic factors and treatment outcomes, univariable and multivariable Cox proportional hazard models were developed.

Results

A total of 353 patients with HEH (median age: 50.4 years) were identified. The most common surgery performed was liver resection (90.8%). One-year OS in the surgical group and non-surgical group was 86.6% and 61.0%, respectively, while 5-year OS was 75.2% and 37.4%, respectively. On multivariable analysis, surgery emerged as a favorable prognostic factor [HR (95%CI): 0.404 (0.215–0.758) p value = 0.005]. Age > 65 years [HR (95%CI): 2.548 (1.442–4.506) p value = 0.001] and tumor size > 10 cm [HR (95%CI): 2.401 (1.319–4.37) p value = 0.004] were shown to be poor survival prognostic factors.

Conclusion

HEH is a rare disease that is poorly understood. Surgical intervention is associated with improved survival rates. Multicenter prospective collaborations are needed to improve our limited knowledge about this neoplasm and determine the optimal treatment strategy.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Lau K, Massad M, Pollak C et al (2011) Clinical patterns and outcome in epithelioid hemangioendothelioma with or without pulmonary involvement: insights from an internet registry in the study of a rare cancer. Chest 140:1312–1318

    Article  Google Scholar 

  2. Weiss SW, Enzinger FM (1982) Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 50:970–981

    Article  CAS  Google Scholar 

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

    PubMed  PubMed Central  Google Scholar 

  4. Virarkar M, Saleh M, Diab R et al (2020) Hepatic Hemangioendothelioma: An update. World J Gastrointest Oncol 12:248–266

    Article  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. Frider B, Bruno A, Selser J et al (2005) Kasabach–Merrit syndrome and adult hepatic epithelioid hemangioendothelioma an unusual association. J Hepatol 42:282–283

    Article  Google Scholar 

  7. Lau A, Malangone S, Green M et al (2015) Combination capecitabine and bevacizumab in the treatment of metastatic hepatic epithelioid hemangioendothelioma. Ther Adv Med Oncol 7:229–236

    Article  CAS  Google Scholar 

  8. Cardinal J, de Vera ME, Marsh JW et al (2009) Treatment of hepatic epithelioid hemangioendothelioma: a single-institution experience with 25 cases. Arch Surg 144:1035–1039

    Article  Google Scholar 

  9. Bostancı EB, Karaman K, Turhan N et al (2014) Selective internal radiotherapy for hepatic epithelioid hemangioendothelioma. Turk J Gastroenterol Off J Turk Soc Gastroenterol 25(1):252–253

    Article  Google Scholar 

  10. Emad A, Fadel S, El Wakeel M et al (2020) Outcome of children treated for infantile hepatic hemangioendothelioma. J Pediatr Hematol Oncol 42:126–130

    Article  Google Scholar 

  11. Lakkis Z, Kim S, Delabrousse E et al (2013) Metronomic cyclophosphamide: an alternative treatment for hepatic epithelioid hemangioendothelioma. J Hepatol 58:1254–1257

    Article  CAS  Google Scholar 

  12. Sangro B, Iñarrairaegui M, Fernández-Ros N (2012) Malignant epithelioid hemangioendothelioma of the liver successfully treated with Sorafenib. Rare Tumors 4:e34

    Article  Google Scholar 

  13. 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 

  14. Lai Q, Feys E, Karam V et al (2017) Hepatic epithelioid hemangioendothelioma and adult liver transplantation: proposal for a prognostic score based on the analysis of the ELTR-ELITA registry. Transplantation 101:555–564

    Article  Google Scholar 

  15. Wang LR, Zhou JM, Zhao YM et al (2012) Clinical experience with primary hepatic epithelioid hemangioendothelioma: retrospective study of 33 patients. World J Surg 36:2677–2683https://doi.org/10.1007/s00268-012-1714-x

    Article  PubMed  Google Scholar 

  16. Gloeckler Ries LA, Reichman ME, Lewis DR et al (2003) Cancer survival and incidence from the Surveillance, Epidemiology, and End Results (SEER) program. The Oncologist 8:541–552

  17. Otrock ZK, Al-Kutoubi A, Kattar MM et al (2006) Spontaneous complete regression of hepatic epithelioid haemangioendothelioma. Lancet Oncol 7:439–441

    Article  Google Scholar 

  18. Kitaichi M, Nagai S, Nishimura K et al (1998) Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression. Eur Respir J 12:89–96

    Article  CAS  Google Scholar 

  19. Komatsu Y, Koizumi T, Yasuo M et al (2010) Malignant hepatic epithelioid hemangioendothelioma with rapid progression and fatal outcome. Intern Med 49:1149–1153

    Article  Google Scholar 

  20. Boukerrouche A (2017) Management of giant liver hemangioma. J Gastroenterol Dig Dis 2(3):12–16

    Google Scholar 

  21. Groeschl RT, Miura JT, Oshima K et al (2014) Does histology predict outcome for malignant vascular tumors of the liver? J Surg Oncol 109:483–486

    Article  Google Scholar 

  22. Lerut JP, Orlando G, Adam R et al (2007) The place of liver transplantation in the treatment of hepatic epitheloid hemangioendothelioma: report of the European liver transplant registry. Annal Surg 246:949–957 (discussion 957)

    Article  Google Scholar 

  23. Rodriguez JA, Becker NS, O’Mahony CA et al (2008) Long-term outcomes following liver transplantation for hepatic hemangioendothelioma: the UNOS experience from 1987 to 2005. J Gastrointest Surg Offi J Soc Surg Aliment Tract 12:110–116

    Article  Google Scholar 

  24. Nudo CG, Yoshida EM, Bain VG et al (2008) Liver transplantation for hepatic epithelioid hemangioendothelioma: the Canadian multicentre experience. Can J Gastroenterol. https://doi.org/10.1155/2008/418485

    Article  PubMed  PubMed Central  Google Scholar 

  25. Mosoia L, Mabrut JY, Adham M et al (2008) Hepatic epithelioid hemangioendothelioma: Long-term results of surgical management. J Surg Oncol 98:432–437

    Article  Google Scholar 

  26. Grotz TE, Nagorney D, Donohue J et al (2010) Hepatic epithelioid haemangioendothelioma: is transplantation the only treatment option? HPB (Oxford) 12:546–553

    Article  Google Scholar 

  27. Thomas RM, Aloia TA, Truty MJ et al (2014) Treatment sequencing strategy for hepatic epithelioid haemangioendothelioma. HPB (Oxford) 16:677–685

    Article  Google Scholar 

  28. Krasnodębski M, Grąt M, Morawski M et al (2020) Hepatic Epithelioid Hemangioendothelioma: A Rare Disease With Favorable Outcomes After Liver Transplantation. Transplant Proc 52:2447–2449

  29. Noh OK, Kim SS, Yang MJ et al (2020) Treatment and prognosis of hepatic epithelioid hemangioendothelioma based on SEER data analysis from 1973 to 2014. Hepatobiliary Pancreat Dis Int HBPD INT 19:29–35

    Article  Google Scholar 

  30. Lazăr DC, Avram MF, Romoșan I et al (2019) Malignant hepatic vascular tumors in adults: characteristics, diagnostic difficulties and current management. World J Clin Oncol 10:110–135

    Article  Google Scholar 

  31. Bachmann R, Genin B, Bugmann P et al (2003) Selective hepatic artery ligation for hepatic haemangioendothelioma: case report and review of the literature. Eur J Pediatr Surg Offi J Austrian Assoc Pediatr Surg 13:280–284

    CAS  Google Scholar 

  32. Ben-Haim M, Roayaie S, Ye MQ et al (1999) Hepatic epithelioid hemangioendothelioma: resection or transplantation, which and when? Liver Transpl Surg Off Publ Am Assoc Study Liver Dis Int Liver Transpl Soc 5:526–531

    CAS  Google Scholar 

  33. National Cancer Institute (2021) Trametinib in Treating Patients With Epithelioid Hemangioendothelioma That is Metastatic, Locally Advanced, or Cannot Be Removed by Surgery (ClinicalTrials.gov Identifier NCT03148275). Retrieved from https://clinicaltrials.gov/ct2/show/NCT03148275

  34. Massachusetts General Hospital (2017) Eribulin in Angiosarcoma and Epithelioid Hemangioendothelioma (EHE) (ClinicalTrials.gov Identifier: NCT03331250). Retrieved from https://clinicaltrials.gov/ct2/show/NCT03331250

Download references

Author information

Authors and Affiliations

  1. Department of Surgery, American University of Beirut Medical Center, Beirut, Lebanon

    Mohamad A. Chahrour, Hussein H. Khachfe & Rudy El-Asmar

  2. Department of Surgery, Johns Hopkins University, Baltimore, MD, USA

    Joseph R. Habib

  3. Department of Radiation Oncology, Mayo Clinic, Jacksonville, FL, USA

    Omran Saifi

  4. Division of General Surgery, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates

    Faek R. Jamali

Authors
  1. Mohamad A. Chahrour
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Hussein H. Khachfe
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Joseph R. Habib
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Rudy El-Asmar
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Omran Saifi
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Faek R. Jamali
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Faek R. Jamali.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

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

Chahrour, M.A., Khachfe, H.H., Habib, J.R. et al. Treatment and Prognosis of Hepatic Epithelioid Hemangioendothelioma: A SEER Database Analysis. World J Surg 45, 2886–2894 (2021). https://doi.org/10.1007/s00268-021-06165-6

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00268-021-06165-6

Search

Navigation