Epithelioid Hemangioendothelioma of the Liver

Beers, Bernard Van; Roche, Alain; Mathieu, Didier; Menu, Y.; Delos, Monique; Otte, Jean-Bernard; Lalonde, Lucie; Pringot, Jacques

doi:10.1097/00004728-199205000-00014

Cited by 79 publications

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“…Furui et al [12], reported capsular retraction in one of five cases of epithelioid hemangioendothelioma of the liver. Van Beers et al [13] found retraction of the liver capsule in two of five cases of this tumor. In the series by Miller et al [14], this finding was found in nine of 13 cases.…”

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confidence: 81%

MR imaging of intrahepatic cholangiocarcinoma

et al. 1995

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confidence: 81%

MR imaging of intrahepatic cholangiocarcinoma

et al. 1995

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“…It may be found incidentally but around half of patients present with upper abdominal pain or discomfort; rare presentations include jaundice, Budd -Chiari-like syndrome or liver failure. Radiological features (CT or MRI) vary: HEHE may present as a large liver mass (one case in this series), but more often the tumour is diffuse with multiple peripheral hepatic lesions with capsular retraction, peripheral contrast enhancement and evidence of multiple calcifications (Van Beers et al, 1992). The latter lesion type has been considered as a progression, with time, of the former unifocal mass type.…”

Section: Discussionmentioning

confidence: 93%

Management of hepatic epithelioid haemangio-endothelioma in children: what option?

et al. 2004

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Hepatic epithelioid haemangio-endothelioma (HEHE) is an endothelium-derived tumour of low-to-medium grade malignancy. It is predominantly seen in adults and is unresponsive to chemotherapy. Liver transplantation is an accepted indication when the tumour is unresectable. Hepatic epithelioid haemangio-endothelioma is very rare in children and results after transplantation are not reported. The aim of this study is to review the experience of three European centres in the management of HEHE in children. A retrospective review of all paediatric patients with HEHE managed in three European centres is presented. Five children were identified. Four had unresectable tumours. The first had successful resection followed by chemotherapy and is alive, without disease 3 years after diagnosis. One child died of sepsis and one of tumour recurrence in the graft and lungs 2 and 5 months, respectively, after transplant. Two children who had progressive disease with ifosfamide-based chemotherapy have had a reduction in clinical symptoms and stabilisation of disease up to 18 and 24 months after the use of platinum-based chemotherapy. HEHE seems more aggressive in children than reported in adults and the curative role of transplantation must be questioned. Ifosfamide-based chemotherapy was not effective. Further studies are necessary to confirm if HEHE progression in children may be influenced by platinum-based chemotherapy. Hepatic epithelioid haemangio-endothelioma (HEHE) is a rare tumour of vascular origin. Hepatic epithelioid haemangio-endothelioma is a tumour of low-to-intermediate grade malignancy and is distinct from the benign tumour 'infantile haemangioendothelioma' and the malignant tumour 'angiosarcoma'. Although the usual clinical course is characterised by both a very slow progression and a malignant behaviour with local recurrence and distant metastasis, its natural behaviour has also been described as unpredictable (Dietze et al, 1989;Makhlouf et al, 1999). Prolonged survival without treatment (Läuffer et al, 1996;Makhlouf et al, 1999;Uchimura et al, 2001) and even spontaneous clearance has been seen, while in others the tumour has progressed rapidly.In the absence of effective chemotherapy strategies, tumour resection is the only management option. Many cases present at diagnosis with multifocal hepatic disease or large, widespread tumours and conventional liver surgery is not possible. Since HEHE progression is usually very slow, liver transplantation has been proposed as a reasonable option in these cases (Scoazec et al, 1988;Madariaga et al, 1995). The 5-year actuarial survival following liver transplantation for HEHE in adults is as high as 71% (disease-free survival ¼ 60%) (Madariaga et al, 1995).Most reports have been in adults and a female predominance has been seen. In children, HEHE is extremely rare and management is usually as for adults; however, very little information is available in literature about the disease characteristics and outcome in paediatric age range. For the purpose of this retrospective r...

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“…Focal calcifications are found in 20% of tumours [16,3,171. The internal architecture of the tumours is best depicted on T2-weighted MRI [17]. Angiography reveals only moderate vascularisation, with displacement of marginal vessels [3, 171. Due to the non-specific evolution and the rarity of this tumour, diagnosis may be difficult.…”

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confidence: 99%

Hepatic haemangioendothelioma in adults: excellent outcome following liver transplantation

et al. 2004

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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.

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Epithelioid Hemangioendothelioma of the Liver

Cited by 79 publications

References 0 publications

MR imaging of intrahepatic cholangiocarcinoma

MR imaging of intrahepatic cholangiocarcinoma

Management of hepatic epithelioid haemangio-endothelioma in children: what option?

Hepatic haemangioendothelioma in adults: excellent outcome following liver transplantation

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