A review of hepatic epithelioid hemangioendothelioma-Analyzing patient characteristics and treatment strategies

J Surg Oncol. 2022 Dec;126(8):1423-1429. doi: 10.1002/jso.27066. Epub 2022 Aug 17.


Background: Hepatic epithelioid hemangioendothelioma (HEH) is a rare vascular tumor of unknown etiology and unpredictable natural history. To date, no large-scale studies have been published evaluating this disease due to its rare occurrence.

Methods: The National Cancer Database was reviewed between 2004 and 2016 to identify patients with HEH. Univariate analysis with overall survival (OS) was performed by Cox proportional hazards model. Kaplan-Meier method was used to create OS curves and compared using the log-rank test.

Results: We identified 229 patients with HEH. The majority of patients were female (61.1%), white (84.3%), and had a Charlson-Deyo score of 0 (75%). Chemotherapeutic intervention was seen in 26% of the patients while 33% received surgical intervention in the form of wedge/segmental liver resection (n = 27), hepatectomy lobectomy/extended lobectomy (n = 18), and liver transplant (n = 22). Five-year survival in surgical patients was 90.5%, 66.5% and 81%, respectively (p = 0.485). Age greater than 55 years (hazard ratio [HR], 2.78; p < 0.001), Asian ethnicity compared to white (HR, 2.84; p = 0.012), and a higher Charlson-Deyo score (score 1: HR, 2.28; p < 0.001 and score ≥2: HR, 2.76; p = 0.011) were associated with worse OS.

Conclusion: Treatment for HEH remains variable with only a third of the patients undergoing surgery. International collaboration is necessary to determine the optimal treatment for this rare disease.

Keywords: HEH; HEHE; epithelioid hemangioendothelioma.

Publication types

  • Review

MeSH terms

  • Female
  • Hemangioendothelioma, Epithelioid* / pathology
  • Hemangioendothelioma, Epithelioid* / surgery
  • Hepatectomy
  • Humans
  • Liver Neoplasms* / pathology
  • Liver Neoplasms* / surgery
  • Liver Transplantation*
  • Male
  • Middle Aged
  • Proportional Hazards Models