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Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumor

Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, al...

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Autores principales: Sardaro, Angela, Bardoscia, Lilia, Petruzzelli, Maria Fonte, Portaluri, Maurizio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: PAGEPress Publications, Pavia, Italy 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4419652/
https://www.ncbi.nlm.nih.gov/pubmed/25992243
http://dx.doi.org/10.4081/oncol.2014.259
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author Sardaro, Angela
Bardoscia, Lilia
Petruzzelli, Maria Fonte
Portaluri, Maurizio
author_facet Sardaro, Angela
Bardoscia, Lilia
Petruzzelli, Maria Fonte
Portaluri, Maurizio
author_sort Sardaro, Angela
collection PubMed
description Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers), and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease.
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spelling pubmed-44196522015-05-19 Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumor Sardaro, Angela Bardoscia, Lilia Petruzzelli, Maria Fonte Portaluri, Maurizio Oncol Rev Review Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers), and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease. PAGEPress Publications, Pavia, Italy 2014-10-13 /pmc/articles/PMC4419652/ /pubmed/25992243 http://dx.doi.org/10.4081/oncol.2014.259 Text en ©Copyright A. Sardaro et al. http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review
Sardaro, Angela
Bardoscia, Lilia
Petruzzelli, Maria Fonte
Portaluri, Maurizio
Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumor
title Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumor
title_full Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumor
title_fullStr Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumor
title_full_unstemmed Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumor
title_short Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumor
title_sort epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4419652/
https://www.ncbi.nlm.nih.gov/pubmed/25992243
http://dx.doi.org/10.4081/oncol.2014.259
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