Hepatic haemangioendothelioma in adults: excellent outcome following liver transplantation

Abstract

Hepatic epithelioid haemangioendotheliomas (HEHEs) are rare, low-grade vascular tumours. transplantation 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.

Hepatic epithelioid hemangioendothelioma: Long‐term results of surgical management

“…Our results are consistent with those previously published by Madariaga et al [17] who reported 71% actuarial patient survival and 60% actuarial disease-free survival at 5 years postOLT in 16 patients and those reported by Yokoyama et al [24] with an actuarial survival rates of 88% and 48% disease-free in eight patients treated by OLT at 5 years. Penn et al [22] also reported 7 out of 21 patients who developed HEHE recurrences and 43% posttransplantation survival rate at 5 years and in Lerut et al [25] study, an excellent outcome following LT was obtained in six patients with disease-free survival at a range of 22 months-14 years. More recently, results of the largest reported multicenter transplant series, including 59 patients, were found to be excellent with 1-, 5-, and 10 year patient survival rates of 93%, 83%, and 72%, respectively [26].…”

“…According to the reported experience in the revelant literature, limited extrahepatic disease, such as small pulmonary metastases should not be currently considered as an absolute contraindication to OLT [22,25].…”

“…Furethermore, because life expectancy for patients presenting with HEHE is potentially long and because extrahepatic tumor spreading does not appear to influence the prognosis after surgery [1,3,18,27], OLT should be even more seriously considered as a potential treatment for patients with HEHE even if an extrahepatic disease is present [22,25,26]. According to the reported experience in the revelant literature, limited extrahepatic disease, such as small pulmonary metastases should not be currently considered as an absolute contraindication to OLT [22,25].…”

Living Donor Liver Transplantation for Epithelioid Hemangioendothelioma: Report of a Case

“…Thus, with such wide biologic characteristics, it is difficult to evaluate the effectiveness of a particular treatment. Previous studies suggest that extrahepatic metastasis is not a prognostic factor after liver transplantation for EH; 2,4,[8][9][10][11][12]14 however, it is paradoxical that the presence of metastasis at transplantation would be associated with better post-transplant prognosis for a malignant disease.To re-evaluate the clinical effectiveness of liver transplantation for EH, we reviewed 13 studies 4,6-16 documenting long-term results after liver transplantation for EH. Of the 41 patients studied, including our patient Abstract Epithelioid hemangioendothelioma (EH) of the liver is a rare tumor, generally considered to have low-grade malignancy.…”

Management of synchronous vascular and ductal anomalies in living donor liver transplantation for hepatic epithelioid hemangioendothelioma

“…This is a rare, low-grade vascular malignancy whose therapeutic algorithm is far from standardized. 1,2 As demonstrated by different authors, [3][4][5][6] liver transplantation is a valuable treatment for hepatic epithelioid hemangioendothelioma, even in cases of extrahepatic metastasis. In our case, the 26-year-old donor and the 26-year-old recipient were twins (brother and sister, respectively).…”