Aggressive intracranial fibromatosis: case report Fibromatose agressiva intracraniana: relato de caso

Fibromatosis is a locally aggressive, proliferative fibroblastic lesion affecting musculoaponeurotic structures, most often in the limbs and trunk. Intracranial fibromatosis is extremely rare and requires aggressive treatment to prevent recurrence. Case Description - We present the case of a 20 year...

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Main Authors: Oswaldo Inácio de Tella Jr, Luciano Ricardo França Silva, João Norberto Stavale, Marco Antonio Herculano, Manoel Antonio de Paiva Neto, Celso Agner
Format: Article
Language:English
Published: Thieme Revinter Publicações 2006-06-01
Series:Arquivos de Neuro-Psiquiatria
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Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2006000300031
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author Oswaldo Inácio de Tella Jr
Luciano Ricardo França Silva
João Norberto Stavale
Marco Antonio Herculano
Manoel Antonio de Paiva Neto
Celso Agner
author_facet Oswaldo Inácio de Tella Jr
Luciano Ricardo França Silva
João Norberto Stavale
Marco Antonio Herculano
Manoel Antonio de Paiva Neto
Celso Agner
author_sort Oswaldo Inácio de Tella Jr
collection DOAJ
description Fibromatosis is a locally aggressive, proliferative fibroblastic lesion affecting musculoaponeurotic structures, most often in the limbs and trunk. Intracranial fibromatosis is extremely rare and requires aggressive treatment to prevent recurrence. Case Description - We present the case of a 20 year old woman with aggressive skull base fibromatosis. The lesion extended through the sphenoid, ethmoid sinus and nasal cavity, destroying the right roof of the orbit and penetrating in anterior skull base. A combined anterior craniofacial approach was performed; complete resection with surgical margin was impossible due to the localization of the tumor and relation to important neurovascular structures. Complete resection with surgical margin is often impossible because of its widely infiltrative nature. Radiotherapy and chemotherapy are often required to improve local control of the lesion.<br>Fibromatose é lesão fibroblástica proliferativa com agressividade localizada, afetando as estruturas músculo-aponeuróticas e acometendo, mais freqüentemente, os membros e tronco. A fibromatose intracraniana é rara e requer tratamento agressivo para evitar recidiva. Descrevemos uma paciente de 20 anos portadora de fibromatose agressiva de base de crânio. A lesão se estendia através da cavidade nasal, seios esfenoidal e etmoidal, destruindo o teto da órbita direita e invadindo a porção anterior da base do crânio. A paciente foi submetida a uma via craniofacial anterior combinada, não sendo possível uma ressecção completa devido ao envolvimento de estruturas neurovasculares. A ressecção completa é, freqüentemente, impossível devido sua natureza infiltrativa. Radioterapia e quimioterapia são indicadas para melhorar o controle da lesão.
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series Arquivos de Neuro-Psiquiatria
spelling doaj-art-4d8656b41e924f339e3f0add694366f62025-08-20T02:38:43ZengThieme Revinter PublicaçõesArquivos de Neuro-Psiquiatria0004-282X1678-42272006-06-01642b51651910.1590/S0004-282X2006000300031Aggressive intracranial fibromatosis: case report Fibromatose agressiva intracraniana: relato de casoOswaldo Inácio de Tella JrLuciano Ricardo França SilvaJoão Norberto StavaleMarco Antonio HerculanoManoel Antonio de Paiva NetoCelso AgnerFibromatosis is a locally aggressive, proliferative fibroblastic lesion affecting musculoaponeurotic structures, most often in the limbs and trunk. Intracranial fibromatosis is extremely rare and requires aggressive treatment to prevent recurrence. Case Description - We present the case of a 20 year old woman with aggressive skull base fibromatosis. The lesion extended through the sphenoid, ethmoid sinus and nasal cavity, destroying the right roof of the orbit and penetrating in anterior skull base. A combined anterior craniofacial approach was performed; complete resection with surgical margin was impossible due to the localization of the tumor and relation to important neurovascular structures. Complete resection with surgical margin is often impossible because of its widely infiltrative nature. Radiotherapy and chemotherapy are often required to improve local control of the lesion.<br>Fibromatose é lesão fibroblástica proliferativa com agressividade localizada, afetando as estruturas músculo-aponeuróticas e acometendo, mais freqüentemente, os membros e tronco. A fibromatose intracraniana é rara e requer tratamento agressivo para evitar recidiva. Descrevemos uma paciente de 20 anos portadora de fibromatose agressiva de base de crânio. A lesão se estendia através da cavidade nasal, seios esfenoidal e etmoidal, destruindo o teto da órbita direita e invadindo a porção anterior da base do crânio. A paciente foi submetida a uma via craniofacial anterior combinada, não sendo possível uma ressecção completa devido ao envolvimento de estruturas neurovasculares. A ressecção completa é, freqüentemente, impossível devido sua natureza infiltrativa. Radioterapia e quimioterapia são indicadas para melhorar o controle da lesão.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2006000300031fibromatose intracranianabase do crâniovia craniofacialintracranial fibromatosisskull basecraniofacial approach
spellingShingle Oswaldo Inácio de Tella Jr
Luciano Ricardo França Silva
João Norberto Stavale
Marco Antonio Herculano
Manoel Antonio de Paiva Neto
Celso Agner
Aggressive intracranial fibromatosis: case report Fibromatose agressiva intracraniana: relato de caso
Arquivos de Neuro-Psiquiatria
fibromatose intracraniana
base do crânio
via craniofacial
intracranial fibromatosis
skull base
craniofacial approach
title Aggressive intracranial fibromatosis: case report Fibromatose agressiva intracraniana: relato de caso
title_full Aggressive intracranial fibromatosis: case report Fibromatose agressiva intracraniana: relato de caso
title_fullStr Aggressive intracranial fibromatosis: case report Fibromatose agressiva intracraniana: relato de caso
title_full_unstemmed Aggressive intracranial fibromatosis: case report Fibromatose agressiva intracraniana: relato de caso
title_short Aggressive intracranial fibromatosis: case report Fibromatose agressiva intracraniana: relato de caso
title_sort aggressive intracranial fibromatosis case report fibromatose agressiva intracraniana relato de caso
topic fibromatose intracraniana
base do crânio
via craniofacial
intracranial fibromatosis
skull base
craniofacial approach
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2006000300031
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