Treatment of hepatic epithelioid hemangioendothelioma with liver transplantation

Abstract

Ten patients received liver transplants for unresectable epithelioid hemangioendothelioma (EHE). At the time of transplantation, four patients had microscopic metastases to the hilar lymph nodes, and one of the four also had metastases to a rib. The fifth patient had metastases to the lung, pleura, and diaphragm. The remaining five patients were believed to be free of metastatic disease. Two of these five patients died of metastatic disease at 3 and 16 months, respectively, after transplantation. Interestingly, all five patients with metastatic involvement are currently alive 40.6 +/- 22 months (mean +/- standard error of mean [SEM]) after transplantation, although one of these patients currently has metastatic disease to the lungs and mediastinum. Thus, the projected 5-year actuarial survival rate is 76%, with two patients at risk after the third year. In conclusion, liver transplantation is a reasonable procedure for bulky, otherwise unresectable, EHE even in the presence of metastatic disease.

Hepatic haemangioendothelioma in adults: excellent outcome following liver transplantation

“…Its unpredictable behaviour and prognosis raises controversy about the role of liver transplantation, especially in view of (a) the spontaneous, long-term survival [6,1,31; (b) the 28% to 45% incidence of extrahepatic, mostly pulmonary, osseous, peritoneal and/or lymph node disease [l, 3, 41; (c) the reported recurrent allograft disease [16,19,20,21,22,23,241.…”

Hepatic epithelioid hemangioendothelioma: Resection or transplantation, which and when?

“…3,9,10 Since its application in the treatment of primary liver malignancies, orthotopic liver transplantation (OLT) has been considered the treatment of choice for HEHE and has been associated with reasonable results. [11][12][13] The inability to predict the behavior of this tumor, and therefore its prognosis, as well as the limited availability of cadaveric livers, raises controversy over the indications, contraindications, and patient selection criteria for OLT in the treatment of HEHE. 14,15 …”

Hemangioendotelioma epitelioide hepático primario: un simulador de metástasis

“…En pacientes con lesiones únicas o escasas, la extirpación quirúrgica es el tratamiento de elección 3 . Los pacientes con lesiones múltiples o extensas, que son la mayoría, deben transplantarse, sin que la existencia de metástasis sea una contraindicación 24,25 . La quimioterapia y radioterapia no han influido significativamente en el curso de la enfermedad 3,4 .…”