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Management of hepatic epithelioid haemangio-endothelioma in children: what option?
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...
Autores principales: | , , , , , , , , |
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Formato: | Texto |
Lenguaje: | English |
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Nature Publishing Group
2004
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2409708/ https://www.ncbi.nlm.nih.gov/pubmed/15083175 http://dx.doi.org/10.1038/sj.bjc.6601720 |
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author | Sharif, K English, M Ramani, P Alberti, D Otte, J-B McKiernan, P Gosseye, S Jenney, M de Ville de Goyet, J |
author_facet | Sharif, K English, M Ramani, P Alberti, D Otte, J-B McKiernan, P Gosseye, S Jenney, M de Ville de Goyet, J |
author_sort | Sharif, K |
collection | PubMed |
description | 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. |
format | Text |
id | pubmed-2409708 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2004 |
publisher | Nature Publishing Group |
record_format | MEDLINE/PubMed |
spelling | pubmed-24097082009-09-10 Management of hepatic epithelioid haemangio-endothelioma in children: what option? Sharif, K English, M Ramani, P Alberti, D Otte, J-B McKiernan, P Gosseye, S Jenney, M de Ville de Goyet, J Br J Cancer Clinical 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. Nature Publishing Group 2004-04-19 2004-03-23 /pmc/articles/PMC2409708/ /pubmed/15083175 http://dx.doi.org/10.1038/sj.bjc.6601720 Text en Copyright © 2004 Cancer Research UK https://creativecommons.org/licenses/by/4.0/This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material.If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit https://creativecommons.org/licenses/by/4.0/. |
spellingShingle | Clinical Sharif, K English, M Ramani, P Alberti, D Otte, J-B McKiernan, P Gosseye, S Jenney, M de Ville de Goyet, J Management of hepatic epithelioid haemangio-endothelioma in children: what option? |
title | Management of hepatic epithelioid haemangio-endothelioma in children: what option? |
title_full | Management of hepatic epithelioid haemangio-endothelioma in children: what option? |
title_fullStr | Management of hepatic epithelioid haemangio-endothelioma in children: what option? |
title_full_unstemmed | Management of hepatic epithelioid haemangio-endothelioma in children: what option? |
title_short | Management of hepatic epithelioid haemangio-endothelioma in children: what option? |
title_sort | management of hepatic epithelioid haemangio-endothelioma in children: what option? |
topic | Clinical |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2409708/ https://www.ncbi.nlm.nih.gov/pubmed/15083175 http://dx.doi.org/10.1038/sj.bjc.6601720 |
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