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

Abstract

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...

The Challenges of Vasoformative Tumors of the Liver in Children

“…The signet-ring cell-like features in a myxohyaline stroma may suggest metastatic carcinoma, especially in the presence of multiple hepatic lesions. Hepatic transplantation has been used with some success in the management of this tumor [22].…”

The Role of Liver Transplantation in the Management of Paediatric Liver Tumours

“…It may behave more aggressively in children. 12,53 Most HEHE are large and diffuse and unresectable by partial hepatectomy. There is no consistently effective chemotherapy.…”

“…[7][8][9][10][11][12][13][14][15][16] There are few reports of these relatively high-risk approaches in children and their utility is limited. However, these techniques are sometimes useful when dealing with massive benign tumours or when liver transplantation is not an option for malignant lesions.…”

Hepatic Epithelioid Hemangioendothelioma in an Eight Year old - A Case Report

“…They have a very slow progression yet a malignant behaviour with local recurrence and distant metastasis [7]. Histological features including pleomorphism, atypia and mitotic activity do not correlate with disease severity or predict outcome.…”

“…Benefit from chemotherapy, radiotherapy and interferon remains unclear and unsatisfactory till date [2]. The tumour is said to pursue a more aggressive clinical course in children with limited role of liver transplantation [7].…”