Exploring the Paucity of the Diagnosis: Epithelioid Hemangioendothelioma of the Liver

Abstract

Intoduction: Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor of unpredictable natural history. No large-scale studies have been published evaluating this disease due to its rare occurrence. Method: The National Cancer Database was reviewed between 2004-2016 to identify patients undergoing non-palliative therapy. We stratified the cohort by surgical intervention as wedge/segmental resection, lobectomy/extended lobectomy and transplant. Univariate analysis was performed to identify variables associated with survival. Results: We identified 229 patients with HEH. Median age at diagnosis was 55 years. Majority were female (61.1%) and white (84.3%). Majority received no chemotherapy (70.8%), received their care at an academic center (58%) and had Charlson-Deyo score of 0 (75%). Only one-third underwent surgery as part of their treatment. 27 underwent wedge/segmental liver resection,18 underwent hepatic lobectomy/extended lobectomy and 22 underwent liver transplant. 30-day and 90-day mortality was 3%. There was no difference in survival irrespective of the extent of liver resection. Age greater than 55 years,Asian race compared to White and a higher Charlson-Deyo score compared to score zero were associated with worse OS. Patients receiving care at an academic/research program or comprehensive community cancer program had significantly improved survival (HR 0.39,p=0.008 and HR 0.45,p=0.037 respectively) compared to care at an integrated network cancer program. Conclusions: This desctiptive study represents a large proportion of patients with HEH. Only a third of the patients underwent surgery as treatment for this disease. Older age, higher Charlson-Deyo score and Asian race predicted a worse survival. Receiving care an at academic center predicted an improved survival.