Sclerosing epitelioid fibrosarcoma: case report and review of the literature
Autor(a) principal: | |
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Data de Publicação: | 2003 |
Outros Autores: | , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Revista Brasileira de Cancerologia (Online) |
Texto Completo: | https://rbc.inca.gov.br/index.php/revista/article/view/2075 |
Resumo: | The sclerosing epitelioid fibrossarcoma (SEF) is a malignant neoplasm made by nests and cords of small round cells, with clear cytoplasm, exuberant hyalinization and a high degree of necrosis. It seldom occurs in the limbs, trunk and neck. The SEF is a low degree tumor, and is reative to p53. Local recurrence is related to close resection margins. Differencial diagnosis includes nodular fascitis, desmoids tumor, fibrous hystiocitoma, synovial fibrossarcoma, clear cell sarcoma and alveolar rabdomiossarcoma. Tumor size, trunk localization and gender (male) compromises diagnosis. The autors relat a 44 years old patient case, with tumor in the middle anterior portion of the right forearm, and biopsy confirming SEF. Histology, clinical presentation and management options are discussed. |
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Sclerosing epitelioid fibrosarcoma: case report and review of the literatureFibrossarcoma epitelióide esclerosante: relato de caso.Fibrossarcoma Epitelióide EsclerosanteSarcoma de Partes MolesDiagnósticoCirurgiaSclerosing Epitelioid FibrossarcomaSoft Tissue SarcomaDiagnosisSurgeryThe sclerosing epitelioid fibrossarcoma (SEF) is a malignant neoplasm made by nests and cords of small round cells, with clear cytoplasm, exuberant hyalinization and a high degree of necrosis. It seldom occurs in the limbs, trunk and neck. The SEF is a low degree tumor, and is reative to p53. Local recurrence is related to close resection margins. Differencial diagnosis includes nodular fascitis, desmoids tumor, fibrous hystiocitoma, synovial fibrossarcoma, clear cell sarcoma and alveolar rabdomiossarcoma. Tumor size, trunk localization and gender (male) compromises diagnosis. The autors relat a 44 years old patient case, with tumor in the middle anterior portion of the right forearm, and biopsy confirming SEF. Histology, clinical presentation and management options are discussed.O fibrossarcoma epitelióide esclerosante (FEE) pode ser conceituado como uma neoplasia maligna constituída por ninhos e cordões de pequenas células arredondadas, poligonais e ovóides, de citoplasma claro e escasso, hialinização exuberante e alto grau de esclerose. Compromete mais freqüentemente extremidades, tronco e pescoço. O FEE é considerado um sarcoma de baixo grau e demonstra imunorreatividade ao P 53, sugerindo um papel de destaque deste fator. A recorrência local está relacionada com a ressecção com margem de segurança insuficiente. O diagnóstico diferencial é vasto e inclui tumores e proliferações fibrosas, como fasciíte nodular, miosite ossificante, desmóides, leiomioma hialinizante e histiocitoma fibroso, fibrossarcoma sinovial, sarcoma de células claras de tendões e aponeuroses, osteosarcoma de pequenas células, rabdomiosarcoma alveolar. Os tumores localizados no tronco, as lesões volumosas e em homens comprometem o diagnóstico.Os autores relatam um caso de um paciente de 44 anos, com tumoração localizada em face ântero-medial do braço direito, com evolução em 3 meses, cuja biópsia incisional revelou tratar-se de FEE. Serão discutidos aspectos anatomopatológicos, quadro clínico e tratamento instituído para o caso.INCA2003-12-30info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionRelato de Casoapplication/pdfhttps://rbc.inca.gov.br/index.php/revista/article/view/207510.32635/2176-9745.RBC.2003v49n4.2075Revista Brasileira de Cancerologia; Vol. 49 No. 4 (2003): Oct./Nov./Dec.; 221-225Revista Brasileira de Cancerologia; Vol. 49 Núm. 4 (2003): oct./nov./dic.; 221-225Revista Brasileira de Cancerologia; v. 49 n. 4 (2003): out./nov./dez.; 221-2252176-9745reponame:Revista Brasileira de Cancerologia (Online)instname:Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)instacron:INCAporhttps://rbc.inca.gov.br/index.php/revista/article/view/2075/1290Borba, Patrícia PaturyFiod, Nelson Jabour Gouveia, Gustavo de CastroNascimento, Marilene F. Rezende, José Francisco de info:eu-repo/semantics/openAccess2021-11-29T20:34:30Zoai:rbc.inca.gov.br:article/2075Revistahttps://rbc.inca.gov.br/index.php/revistaPUBhttps://rbc.inca.gov.br/index.php/revista/oairbc@inca.gov.br0034-71162176-9745opendoar:2021-11-29T20:34:30Revista Brasileira de Cancerologia (Online) - Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA)false |
dc.title.none.fl_str_mv |
Sclerosing epitelioid fibrosarcoma: case report and review of the literature Fibrossarcoma epitelióide esclerosante: relato de caso. |
title |
Sclerosing epitelioid fibrosarcoma: case report and review of the literature |
spellingShingle |
Sclerosing epitelioid fibrosarcoma: case report and review of the literature Borba, Patrícia Patury Fibrossarcoma Epitelióide Esclerosante Sarcoma de Partes Moles Diagnóstico Cirurgia Sclerosing Epitelioid Fibrossarcoma Soft Tissue Sarcoma Diagnosis Surgery |
title_short |
Sclerosing epitelioid fibrosarcoma: case report and review of the literature |
title_full |
Sclerosing epitelioid fibrosarcoma: case report and review of the literature |
title_fullStr |
Sclerosing epitelioid fibrosarcoma: case report and review of the literature |
title_full_unstemmed |
Sclerosing epitelioid fibrosarcoma: case report and review of the literature |
title_sort |
Sclerosing epitelioid fibrosarcoma: case report and review of the literature |
author |
Borba, Patrícia Patury |
author_facet |
Borba, Patrícia Patury Fiod, Nelson Jabour Gouveia, Gustavo de Castro Nascimento, Marilene F. Rezende, José Francisco de |
author_role |
author |
author2 |
Fiod, Nelson Jabour Gouveia, Gustavo de Castro Nascimento, Marilene F. Rezende, José Francisco de |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Borba, Patrícia Patury Fiod, Nelson Jabour Gouveia, Gustavo de Castro Nascimento, Marilene F. Rezende, José Francisco de |
dc.subject.por.fl_str_mv |
Fibrossarcoma Epitelióide Esclerosante Sarcoma de Partes Moles Diagnóstico Cirurgia Sclerosing Epitelioid Fibrossarcoma Soft Tissue Sarcoma Diagnosis Surgery |
topic |
Fibrossarcoma Epitelióide Esclerosante Sarcoma de Partes Moles Diagnóstico Cirurgia Sclerosing Epitelioid Fibrossarcoma Soft Tissue Sarcoma Diagnosis Surgery |
description |
The sclerosing epitelioid fibrossarcoma (SEF) is a malignant neoplasm made by nests and cords of small round cells, with clear cytoplasm, exuberant hyalinization and a high degree of necrosis. It seldom occurs in the limbs, trunk and neck. The SEF is a low degree tumor, and is reative to p53. Local recurrence is related to close resection margins. Differencial diagnosis includes nodular fascitis, desmoids tumor, fibrous hystiocitoma, synovial fibrossarcoma, clear cell sarcoma and alveolar rabdomiossarcoma. Tumor size, trunk localization and gender (male) compromises diagnosis. The autors relat a 44 years old patient case, with tumor in the middle anterior portion of the right forearm, and biopsy confirming SEF. Histology, clinical presentation and management options are discussed. |
publishDate |
2003 |
dc.date.none.fl_str_mv |
2003-12-30 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Relato de Caso |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://rbc.inca.gov.br/index.php/revista/article/view/2075 10.32635/2176-9745.RBC.2003v49n4.2075 |
url |
https://rbc.inca.gov.br/index.php/revista/article/view/2075 |
identifier_str_mv |
10.32635/2176-9745.RBC.2003v49n4.2075 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://rbc.inca.gov.br/index.php/revista/article/view/2075/1290 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
INCA |
publisher.none.fl_str_mv |
INCA |
dc.source.none.fl_str_mv |
Revista Brasileira de Cancerologia; Vol. 49 No. 4 (2003): Oct./Nov./Dec.; 221-225 Revista Brasileira de Cancerologia; Vol. 49 Núm. 4 (2003): oct./nov./dic.; 221-225 Revista Brasileira de Cancerologia; v. 49 n. 4 (2003): out./nov./dez.; 221-225 2176-9745 reponame:Revista Brasileira de Cancerologia (Online) instname:Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA) instacron:INCA |
instname_str |
Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA) |
instacron_str |
INCA |
institution |
INCA |
reponame_str |
Revista Brasileira de Cancerologia (Online) |
collection |
Revista Brasileira de Cancerologia (Online) |
repository.name.fl_str_mv |
Revista Brasileira de Cancerologia (Online) - Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA) |
repository.mail.fl_str_mv |
rbc@inca.gov.br |
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