ATYPICAL FIBROXANTHOMA: CLINICOPATHOLOGICAL REVIEW

Detalhes bibliográficos
Autor(a) principal: António, Ana Marta
Data de Publicação: 2015
Outros Autores: Alves, João, Matos, Diogo, Goulão, João, Bártolo, Elvira
Tipo de documento: Artigo
Idioma: por
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: https://doi.org/10.29021/spdv.73.1.345
Resumo: Introduction: Atypical fibroxanthoma (AFX) is a rare cutaneous tumor without a completely defined histogenesis which frequently develops on head and neck of elderly people. Its diagnosis is histological, of exclusion with other tumors with identical morphology. Despite its classical benign behavior some rare cases reporting distant metastasis classified it on intermediate malignancy.Material and methods: We preformed a retrospective analysis with purpose of reviewing the AFX diagnosis made in our department between 2008 and 2014. We analyzed demographic, clinical, histological and immunohistochemical data and also surgical treatment and recurrence.Results: We obtained 12 cases of AFX, 11 males and 1 female, the youngest was 25 years old and the others had a median age of 76 years. The lesions were nodules or tumors with 1.9cm median size, all of them located on the head. Histology revealed well defined lesions with atypical pleomorphic cells and frequent solar elastosis, classified as mix variant (spindle and histiocytic cells) in all cases. Immunohistochemical study showed positivity for vimentin and negativity for cytokeratins and S100 protein in all cases and CD10 and CD99 were frequently positive. Surgical excision was performed in 10 cases with 10% rate of recurrence during 3 years of median follow-up.Conclusions: These results were similar with other published series. This tumor is clinically unspecified and histologically undifferentiated and so immunohistochemistry is essential for diagnosis, however, for now, available makers are not sufficiently specific. Despite its good prognosis in most cases clinical surveillance is recommended.
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spelling ATYPICAL FIBROXANTHOMA: CLINICOPATHOLOGICAL REVIEWFIBROXANTOMA ATÍPICO: REVISÃO CLÍNICO-PATOLÓGICAAtypical fibroxanthomaHistiocytomamalignant fibrousFibroxantoma atípicoFibrohistiocitoma maligno.Introduction: Atypical fibroxanthoma (AFX) is a rare cutaneous tumor without a completely defined histogenesis which frequently develops on head and neck of elderly people. Its diagnosis is histological, of exclusion with other tumors with identical morphology. Despite its classical benign behavior some rare cases reporting distant metastasis classified it on intermediate malignancy.Material and methods: We preformed a retrospective analysis with purpose of reviewing the AFX diagnosis made in our department between 2008 and 2014. We analyzed demographic, clinical, histological and immunohistochemical data and also surgical treatment and recurrence.Results: We obtained 12 cases of AFX, 11 males and 1 female, the youngest was 25 years old and the others had a median age of 76 years. The lesions were nodules or tumors with 1.9cm median size, all of them located on the head. Histology revealed well defined lesions with atypical pleomorphic cells and frequent solar elastosis, classified as mix variant (spindle and histiocytic cells) in all cases. Immunohistochemical study showed positivity for vimentin and negativity for cytokeratins and S100 protein in all cases and CD10 and CD99 were frequently positive. Surgical excision was performed in 10 cases with 10% rate of recurrence during 3 years of median follow-up.Conclusions: These results were similar with other published series. This tumor is clinically unspecified and histologically undifferentiated and so immunohistochemistry is essential for diagnosis, however, for now, available makers are not sufficiently specific. Despite its good prognosis in most cases clinical surveillance is recommended.Introdução: O fibroxantoma atípico (FXA) é um tumor raro de histogénese indeterminada mais frequente em idosos nas áreas fotoexpostas da cabeça e pescoço. Trata-se de um tumor indiferenciado sendo o seu diagnóstico histológico de exclusão. Apesar de classicamente indolente, os casos descritos associados a metastização atribuem-lhe uma malignidade intermédia.Material e métodos: Foi objetivo deste estudo retrospetivo rever e caracterizar os casos de FXA diagnosticados no nosso serviço entre 2008 e 2014. Foram avaliadas variáveis demográficas, clínicas, dados histológicos, imunohistoquímicos, terapêutica cirúrgica e recidiva.Resultados: Foram diagnosticados 12 casos de FXA, 11 do sexo masculino e 1 do sexo feminino, o mais novo com 25 anos e os restantes com idade média de 76 anos. Apresentaram-se como nódulos/tumores com diâmetro médio de 1,9cm, todos localizados na cabeça. Histologicamente revelaram-se lesões bem delimitadas com grande atipia citológica e elastose solar frequente, todas classificadas na variante mista (células fusiformes e histiocitárias). Na imunohistoquímica verificou-se positividade para a vimentina e negatividade para as citoqueratinas e proteína S100 em todos os casos, sendo positivos o CD10 e CD99 na maioria dos casos. A terapêutica foi cirúrgica com excisão radical da lesão em 10 doentes, sendo a recidiva local de 10% num seguimento médio de 3 anos.Conclusões: Os resultados obtidos foram concordantes com outras séries da literatura. Dada a clínica inespecífica e tratando-se de um tumor histologicamente indiferenciado a imunohistoquímica é essencial, não existindo ainda marcadores suficientemente específicos. Apesar do bom prognóstico na maioria dos casos a vigilância destes doentes é recomendada.Sociedade Portuguesa de Dermatologia e Venereologia2015-06-06T00:00:00Zjournal articleinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://doi.org/10.29021/spdv.73.1.345oai:ojs.revista.spdv.com.pt:article/345Journal of the Portuguese Society of Dermatology and Venereology; Vol 73 No 1 (2015): Janeiro - Março; 71-77Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 73 n. 1 (2015): Janeiro - Março; 71-772182-24092182-2395reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://revista.spdv.com.pt/index.php/spdv/article/view/345https://doi.org/10.29021/spdv.73.1.345https://revista.spdv.com.pt/index.php/spdv/article/view/345/314António, Ana MartaAlves, JoãoMatos, DiogoGoulão, JoãoBártolo, Elvirainfo:eu-repo/semantics/openAccess2022-10-06T12:34:51Zoai:ojs.revista.spdv.com.pt:article/345Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:10:51.398112Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv ATYPICAL FIBROXANTHOMA: CLINICOPATHOLOGICAL REVIEW
FIBROXANTOMA ATÍPICO: REVISÃO CLÍNICO-PATOLÓGICA
title ATYPICAL FIBROXANTHOMA: CLINICOPATHOLOGICAL REVIEW
spellingShingle ATYPICAL FIBROXANTHOMA: CLINICOPATHOLOGICAL REVIEW
António, Ana Marta
Atypical fibroxanthoma
Histiocytoma
malignant fibrous
Fibroxantoma atípico
Fibrohistiocitoma maligno.
title_short ATYPICAL FIBROXANTHOMA: CLINICOPATHOLOGICAL REVIEW
title_full ATYPICAL FIBROXANTHOMA: CLINICOPATHOLOGICAL REVIEW
title_fullStr ATYPICAL FIBROXANTHOMA: CLINICOPATHOLOGICAL REVIEW
title_full_unstemmed ATYPICAL FIBROXANTHOMA: CLINICOPATHOLOGICAL REVIEW
title_sort ATYPICAL FIBROXANTHOMA: CLINICOPATHOLOGICAL REVIEW
author António, Ana Marta
author_facet António, Ana Marta
Alves, João
Matos, Diogo
Goulão, João
Bártolo, Elvira
author_role author
author2 Alves, João
Matos, Diogo
Goulão, João
Bártolo, Elvira
author2_role author
author
author
author
dc.contributor.author.fl_str_mv António, Ana Marta
Alves, João
Matos, Diogo
Goulão, João
Bártolo, Elvira
dc.subject.por.fl_str_mv Atypical fibroxanthoma
Histiocytoma
malignant fibrous
Fibroxantoma atípico
Fibrohistiocitoma maligno.
topic Atypical fibroxanthoma
Histiocytoma
malignant fibrous
Fibroxantoma atípico
Fibrohistiocitoma maligno.
description Introduction: Atypical fibroxanthoma (AFX) is a rare cutaneous tumor without a completely defined histogenesis which frequently develops on head and neck of elderly people. Its diagnosis is histological, of exclusion with other tumors with identical morphology. Despite its classical benign behavior some rare cases reporting distant metastasis classified it on intermediate malignancy.Material and methods: We preformed a retrospective analysis with purpose of reviewing the AFX diagnosis made in our department between 2008 and 2014. We analyzed demographic, clinical, histological and immunohistochemical data and also surgical treatment and recurrence.Results: We obtained 12 cases of AFX, 11 males and 1 female, the youngest was 25 years old and the others had a median age of 76 years. The lesions were nodules or tumors with 1.9cm median size, all of them located on the head. Histology revealed well defined lesions with atypical pleomorphic cells and frequent solar elastosis, classified as mix variant (spindle and histiocytic cells) in all cases. Immunohistochemical study showed positivity for vimentin and negativity for cytokeratins and S100 protein in all cases and CD10 and CD99 were frequently positive. Surgical excision was performed in 10 cases with 10% rate of recurrence during 3 years of median follow-up.Conclusions: These results were similar with other published series. This tumor is clinically unspecified and histologically undifferentiated and so immunohistochemistry is essential for diagnosis, however, for now, available makers are not sufficiently specific. Despite its good prognosis in most cases clinical surveillance is recommended.
publishDate 2015
dc.date.none.fl_str_mv 2015-06-06T00:00:00Z
dc.type.driver.fl_str_mv journal article
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dc.identifier.uri.fl_str_mv https://doi.org/10.29021/spdv.73.1.345
oai:ojs.revista.spdv.com.pt:article/345
url https://doi.org/10.29021/spdv.73.1.345
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dc.relation.none.fl_str_mv https://revista.spdv.com.pt/index.php/spdv/article/view/345
https://doi.org/10.29021/spdv.73.1.345
https://revista.spdv.com.pt/index.php/spdv/article/view/345/314
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
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dc.publisher.none.fl_str_mv Sociedade Portuguesa de Dermatologia e Venereologia
publisher.none.fl_str_mv Sociedade Portuguesa de Dermatologia e Venereologia
dc.source.none.fl_str_mv Journal of the Portuguese Society of Dermatology and Venereology; Vol 73 No 1 (2015): Janeiro - Março; 71-77
Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 73 n. 1 (2015): Janeiro - Março; 71-77
2182-2409
2182-2395
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