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

• Type of Material: Article
• Authors: Pranay S Ajay, Emory University; Vasileios Tsagkalidis, University of Rochester; Anthony Casabianca, University of Rochester; Paul R Burchard, University of Rochester; Alexa D Melucci, University of Rochester; Alexander Chacon, University of Rochester; Subir Goyal, Emory University; Jeffrey Switchenko, Emory University; David Kooby, Emory University; Darren R Carpizo, University of Rochester; Mihir Shah, Emory University
• Language: English
• Date: 2022-08-17
• Publisher: WILEY
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2022 Wiley Periodicals LLC.
• Final Published Version (URL): http://dx.doi.org/10.1002/jso.27066
• Title of Journal or Parent Work: JOURNAL OF SURGICAL ONCOLOGY
• Volume: 126
• Issue: 8
• Start Page: 1423
• End Page: 1429
• Keywords: EXPERIENCE; HEH; Oncology; GROWTH; OUTCOMES; HEHE; Life Sciences & Biomedicine; epithelioid hemangioendothelioma; Science & Technology; LIVER-TRANSPLANTATION; Surgery

Abstract

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.