Allograft hepatitis after liver transplantation for epithelioid haemangioendothelioma

Primary hepatic epitheloid heamangioendothelioma (EHE) is a rare neoplasm of vascular origin. We present a retrospective study of 6 patients with EHE treated by liver transplantation that were monitored clinically for more than 3 years and had protocol biopsy samples taken at 1, 3, 5, 7, and 10 years posttransplant. None of the patients suffered from any form of viral or autoimmune hepatitis before or after the transplantation. Two patients had lung metastases detected by preoperative imaging. All tumours showed factor VIII, CD31, and CD34 strong positive staining. In 5 of the 6 transplant recipients the protocol graft biopsies showed chronic non-specific hepatitis with slowly progressive periportal fibrosis that appeared during the 3rd post-transplant year. The septal fibrosis was diagnosed in the 6th and 10th year after transplantation. Liver tests did not reflect either the presence or the degree of inflammation or fibrosis and have remained normal. In retrospect, we consider that our recipients most probably developed alloantigen dependent inflammatory and fibrotic damage to their liver grafts. All six recipients are still alive for a median survival time of 95.1 month (range 44 months to 132 months), with good graft function, and without recurrence of the tumor. The lung metastases in 2 of the 6 patients have remained unchanged for 10 and 12 years retrospectively.