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

• Published in: British journal of cancer Vol. 90; no. 8; pp. 1498 - 1501
• Main Authors: SHARIF, K, ENGLISH, M, RAMANI, P, ALBERTI, D, OTTE, J.-B, MCKIERNAN, P, GOSSEYE, S, JENNEY, M, DE VILLE DE GOYET, J
• Format: Journal Article
• Language: English
• Published: Basingstoke Nature Publishing Group 19-04-2004
• Subjects: Adolescent; Antineoplastic Agents, Alkylating - therapeutic use; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Child; Child, Preschool; Clinical; Female; Hemangioendothelioma - drug therapy; Hemangioendothelioma - pathology; Hemangioendothelioma - surgery; Humans; Ifosfamide - therapeutic use; Liver Neoplasms - drug therapy; Liver Neoplasms - pathology; Liver Neoplasms - surgery; Liver Transplantation; Male; Medical sciences; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Tumors; Antineoplastic agent; Actinomycin; Liver; Transplantation; Doxorubicin; Alkaloid; liver tumour hepatic epithelioid haemangio-endothelioma; Isomerases; Cancerology; Surgery; Graft; Tumor; Antimitotic; Child; Human; DNA topoisomerase (ATP-hydrolysing); actinomycin D; Digestive system; Enzyme; Ifosfamide; Enzyme inhibitor; Etoposide; Vincristine; Cisplatin; Podophyllotoxine derivatives; Alkylating agent; Oxazaphosphinane derivatives; Antibiotic; Chemotherapy; Treatment; Nitrogen mustard; adriamycin; Carboplatin; Anthracyclins; liver transplantation
• ISSN: 0007-0920; 1532-1827
• DOI: 10.1038/sj.bjc.6601720

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.