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Hepatic haemangioendothelioma in adults: excellent outcome following liver transplantation

2004; Springer Science+Business Media; Volume: 17; Issue: 4 Linguagem: Inglês

10.1111/j.1432-2277.2004.tb00429.x

1432-2277

Jan Lerut, Giuseppe Orlando, Christine Sempoux, Olga Ciecarelli, Bernard E. Van Beers, E. Danse, Yves Horsmans, Jacques Rahier, Francine Roggen,

Tuberous Sclerosis Complex Research

Abstract Hepatic epithelioid haemangioendotheliomas (HEHEs) are rare, low-grade vascular tumours. Five adults with HEHEs and one adult with a vascular tumour showing combined features of haemangioma and haemangioendothelioma underwent liver transplantation. Two HEHE patients had extrahepatic metastases at the time of transplantation. Median survival time following diagnosis was 10.7 years (range 40 months to 195 months). One patient needed resection of a HEHE in the breast 13 years post-transplantation. All six patients are surviving free from disease 22 to 166 months after transplantation (median 77 months). One HEHE-patient who had been treated for 8 years for vertebral and cerebral localisations is free of disease without immunosuppression 56 months after transplantation. We can conclude that liver transplantation is a valuable treatment for hepatic haemangioendothelioma, even in cases of extrahepatic localisation of the disease.