Objective  To investigate the clinical efficacy and therapeutic progress of orthotopic liver transplantation for the treatment of hepatic epithelioid hemangioendothelioma (EHE).

  Methods  Clinical data of 2 patients diagnosed with hepatic EHE were retrospectively analyzed. One patient (case 1) was diagnosed with multiple hepatic EHE complicated with multiple infarction lesions of the spleen, and underwent orthotopic liver transplantation combined with splenectomy. The other case (case 2) was diagnosed with multiple hepatic EHE and received orthotopic liver transplantation alone. Literature review was performed. Pathological characteristics, clinical efficacy of liver transplantation and clinical prognosis of hepatic EHE patients were analyzed.

  Results  Two patients successfully underwent surgery and were discharged postoperatively. The diagnosis of hepatic EHE was confirmed by pathological examination and case 1 was complicated with EHE of the spleen. For case 1, tacrolimus was replaced by sirolimus at postoperative 1 month. At postoperative 4 months, capecitabine was orally administered (chemotherapy) for EHE recurrence. At 6 months after surgery, the patient was diagnosed with recurrent hepatic EHE complicated with multiple bone metastases, and was orally administered with sorafenib. At postoperative 7 months, the patient died from cachexia and liver failure. Case 2 was followed up until the submission date (8 months after surgery). No postoperative complications and tumor recurrence were observed. Previous studies had demonstrated that surgical resection was the primary therapy of hepatic EHE. Liver transplantation was highly recommended for patients with multiple unresectable hepatic EHE and extra-hepatic lesions. Moreover, chemotherapy, percutaneous puncture combined with transcatheter arterial chemoembolization and anti-angiogenesis treatment exerted certain clinical efficacy.

  Conclusions  Surgical resection remains the primary therapy of hepatic EHE. For patients with multiple intrahepatic EHE, liver transplantation is considered as the optimal treatment. Much attention should be diverted to the prevention and treatment of recurrent hepatic EHE following liver transplantation, aiming to improve the clinical efficacy.