Giant cell angioblastoma in an adult: a unique presentation

Giant cell angioblastoma is a very rare, locally destructive vascular tumor of intermediate malignancy without metastatic potential. There are only a few cases reported in the literature exclusively in the soft tissue of children. For the first time, we report on an adult patient with a giant cell a...

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Main Authors: Bruno Fuchs, Yvonne Nussbaumer-Ochsner, Beata Bode-Lesniewska, Susanna Crivelli-Ochsner
Format: Article
Language:English
Published: SAGE Publishing 2013-07-01
Series:Rare Tumors
Subjects:
Online Access:http://www.pagepress.org/journals/index.php/rt/article/view/4706
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author Bruno Fuchs
Yvonne Nussbaumer-Ochsner
Beata Bode-Lesniewska
Susanna Crivelli-Ochsner
author_facet Bruno Fuchs
Yvonne Nussbaumer-Ochsner
Beata Bode-Lesniewska
Susanna Crivelli-Ochsner
author_sort Bruno Fuchs
collection DOAJ
description Giant cell angioblastoma is a very rare, locally destructive vascular tumor of intermediate malignancy without metastatic potential. There are only a few cases reported in the literature exclusively in the soft tissue of children. For the first time, we report on an adult patient with a giant cell angioblastoma in the popliteal fossa. The therapy included tumor resection with favorable clinical, oncological and functional outcome. Due to its locally destructive nature, surgery remains the mainstay of treatment. Histologically, giant cell angioblastoma is comprised of nodular aggre- gates of histiocytoid cells arranged around bland angiomatous spaces. Because of insufficient available data in regard to the definition of the entity, diagnostic criteria and its biological potential, it is not included in the new World Health Organization classification of tumors of soft tissue and bone. The differential diagnosis includes plexiform fibrohistiocytic tumor, myofibroma and giant cell fibroblastoma.
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spelling doaj.art-905ce51ebaeb46dcba846d1e9adc56e12022-12-21T23:11:43ZengSAGE PublishingRare Tumors2036-36052036-36132013-07-0153e27e2710.4081/rt.2013.e27Giant cell angioblastoma in an adult: a unique presentationBruno FuchsYvonne Nussbaumer-OchsnerBeata Bode-LesniewskaSusanna Crivelli-OchsnerGiant cell angioblastoma is a very rare, locally destructive vascular tumor of intermediate malignancy without metastatic potential. There are only a few cases reported in the literature exclusively in the soft tissue of children. For the first time, we report on an adult patient with a giant cell angioblastoma in the popliteal fossa. The therapy included tumor resection with favorable clinical, oncological and functional outcome. Due to its locally destructive nature, surgery remains the mainstay of treatment. Histologically, giant cell angioblastoma is comprised of nodular aggre- gates of histiocytoid cells arranged around bland angiomatous spaces. Because of insufficient available data in regard to the definition of the entity, diagnostic criteria and its biological potential, it is not included in the new World Health Organization classification of tumors of soft tissue and bone. The differential diagnosis includes plexiform fibrohistiocytic tumor, myofibroma and giant cell fibroblastoma.http://www.pagepress.org/journals/index.php/rt/article/view/4706giant cell, angioblastoma, vascular tumor, soft-tissue tumor, intermediate malignancy
spellingShingle Bruno Fuchs
Yvonne Nussbaumer-Ochsner
Beata Bode-Lesniewska
Susanna Crivelli-Ochsner
Giant cell angioblastoma in an adult: a unique presentation
Rare Tumors
giant cell, angioblastoma, vascular tumor, soft-tissue tumor, intermediate malignancy
title Giant cell angioblastoma in an adult: a unique presentation
title_full Giant cell angioblastoma in an adult: a unique presentation
title_fullStr Giant cell angioblastoma in an adult: a unique presentation
title_full_unstemmed Giant cell angioblastoma in an adult: a unique presentation
title_short Giant cell angioblastoma in an adult: a unique presentation
title_sort giant cell angioblastoma in an adult a unique presentation
topic giant cell, angioblastoma, vascular tumor, soft-tissue tumor, intermediate malignancy
url http://www.pagepress.org/journals/index.php/rt/article/view/4706
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AT beatabodelesniewska giantcellangioblastomainanadultauniquepresentation
AT susannacrivelliochsner giantcellangioblastomainanadultauniquepresentation