Hepatic epithelioid haemangioendothelioma: challenges in diagnosis and management

Background: Hepatic epithelioid haemangioendothelioma (HEH) is a rare, low grade malignant neoplasm of endothelial origin which is difficult to diagnose and has a variable outcome. We review five HEH cases from our centre with the aim of identifying clinical predictors of outcome and various therapeutic options.

Methods: A search was made on the WA Liver Transplant registry for cases with histologically confirmed HEH. Their medical records were reviewed. A literature search was conducted through Medline using terms to compare the results from this series with those of other series.

Results: Five patients were identified to have HEH. The mean age was 44.2years (range 34-53years). Four of five patients presented with dyspepsia and two patients had clinical evidence of portal hypertension with ascites. Two patients had radiologically diffuse disease and three patients had discrete nodular liver involvement. The mean duration from presentation of symptoms to diagnosis of HEH was 26.8months. Liver transplantation was performed in one patient with diffuse HEH who is alive with no disease recurrence at 3years. Three patients with radiologically stable disease followed with 6monthly surveillance imaging are currently alive and well. The median survival of all five patients was 5years (range 1.5-16years) at the time of follow up.

Conclusions: These results support the role of surveillance alone for patients with focal and radiologically stable disease. Patients with diffuse HEH with hepatic decompensation should be considered for transplantation. However, numbers are small and an international registry is required to make firm comparisons.