Background : Hepatic Epithelioid Haemagioendothelioma (HEHE) is a rare liver tumour arising from vascular tissue. Given its rarity there is little data regarding its treatment and outcomes.

Methods : This case series examines all patients identified with HEHE treated at the Royal Perth hospital hepatopancreaticobiliary surgery unit between 2010 and 2022. Variables presented are patient characteristics, disease characteristics, treatment details and outcomes.

Results : In this analysis 4 patients with HEHE were identified. Of the patients 1 was female and 3 patients were male. Presenting symptoms were predominantly abdominal pain, no patients presented with jaundice or ascites. Extrahepatic deposits were identified in 1 patient, located in the right lung. Primary resection was performed in 3 patients, best supportive care was offered to the patient with metastatic disease. All resected patients had unifocal, localised disease. Resection margins of 2mm, 8mm and 10mm were achieved. Tumour recurrence occurred in 2 patients. Recurrence occurred early in 1 patient at 15 months post resection both locally and distant. Recurrence occurred late at 5 years in another patient at a local site only. The late recurrence underwent further resection where a margin of 25mm was achieved. Of the resected patients 1 patient died 2 years following early recurrence. The remaining resected patients remain alive 15 months and 6 years following their original resection.

Conclusions : HEHE is a rare tumour which can be locally aggressive. Surgery appears to be a viable treatment option for patients with localised unifocal disease. We hope that this case series add to the body of available literature.