Hepatic Hemangiosarcoma: An Absolute Contraindication to Liver Transplantation-The European Liver Transplant Registry Experience

1 Starzl Abdominal Transplant Unit, University Hospitals St. Luc, Brussels, Belgium. 2 Centre Hépatobiliaire Paul Brousse, Paris, France. 3 Liver Unit, Queen Elizabeth Hospital, Birmingham, UK. 4 Department of Transplantation, Karolinska University Hospital, Huddinge, Stockholm, Sweden. 5 Department of Hepatobiliary Surgery and Liver Transplantation, UMC Groningen, the Netherlands. 6 Department of General and Transplantation Surgery, University Hospital of Essen, Essen, Germany. 7The Royal Free Sheimla Sherlock Liver Centre and University Department of Surgery, Royal Free Hospital and UCL, London, UK. 8 Department of Visceral and Transplantation Surgery, Inselspital, Bern, Switzerland. 9 Department of Lung and Liver Transplantation, Ospedali Riuniti, Bergamo, Italy. 10 Universitätsklinik, Innsbruck, Austria. 11 Department of Hepatobiliary and Liver Transplantation Surgery, Hospital Clinic I Provincial, Barcelona, Spain. 12 Service Chirurgie Digestive et Transplantations, CHU, Univ Nord-de-France, Lille, France. 13 ELT Registry, Paris, France. 14 Currently, Department of General Surgery, Wake Forest University School of Medicine, Winston-Salem, NC. 15 Address correspondence to: Prof. Jan Lerut, M.D., Ph.D., F.A.C.S., Starzl Abdominal Transplant Unit, University Hospitals St. Luc, Department of Abdominal Surgery and Transplantation, Université catholique de Louvain (UCL), Avenue Hippocrate 10, 1200 Brussels, Belgium.
Transplantation (Impact Factor: 3.83). 01/2013; 95(6). DOI: 10.1097/TP.0b013e318281b902
Source: PubMed


Liver transplantation (LT) is performed for hemangiosarcoma (HAS) despite disappointing results.

Retrospective study of 14 males and 8 females reported to the European Liver Transplant Registry. In view of the difficult differential diagnosis between HAS and hemangioendothelioma (HE), the study was deliberately restricted to the period 1986 to 2004 to allow comparison of clinical and biochemical behavior of HAS and HE liver recipients transplanted during the same time period.

Clinical signs, symptoms, and biochemical parameters differed significantly. Pre-LT diagnosis of HAS was made in only 5 of 16 (31%) biopsied patients. HE (7 patients) and hepatocellular cancer (2 patients) were confounding diagnoses leading to LT. Extrahepatic disease was present at time of LT in 4 (19%) patients. Giant invalidating tumor (5 HAS, 1 with Budd-Chiari syndrome [BCS], and 10 supposed epithelioid hemangioendothelioma, 1 with BCS), acute BCS of unknown origin (2 patients), chronic liver failure (4 patients), and solitary hepatocellular cancer (1 patient) were the main indications for LT. Overall survival was 7.2±2.6 months; no patient survived after 23 months. Recurrence was diagnosed after 5.0±2.6 months. Seventeen (77.2%) patients died of tumor recurrence, and the remaining 5 patients died of early infections.

HAS is an absolute contraindication to LT due to the poor outcome. When dealing with the difficult differential diagnosis between HAS and HE, futile LT can be avoided by taking into consideration their distinct clinical and biochemical behaviors as well as a 6-month wait-list observation period. This time period enables the evaluation of HAS disease progression without compromising prognosis of HE patients, thereby allowing to avoid organ wastage.

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