Inflammatory Myofibroblastic Tumour of the Common Bile Duct
Autor(a) principal: | |
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Data de Publicação: | 2022 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16976 |
Resumo: | Inflammatory myofibroblastic tumour is a rare entity of indeterminate biological potential with a reduced tendency for recurrence and metastasis. Although it can arise from multiple organs, the bile duct is a very rare site of origin. We report the case of a 75-year-old asymptomatic male with elevated gamma-glutamyl transferase [1575 U/L (12 - 64 U/L)] and alkaline phosphatase [271 U/L (40 - 150 U/L)]. Computed tomography showed a 17 mm hypervascular lesion in the confluence of the right and left hepatic ducts, with bile duct ectasia and right liver lobe atrophy. The patient was initially managed as having a Klatskin tumour and underwent right hepatectomy. Histology showed a spindle cell proliferation with an inflammatory infiltrate of lymphocytes, plasma cells and collagen-rich stroma, consistent with an inflammatory myofibroblastic tumour. He was discharged 30 days after admission, and nine months later remains asymptomatic. His liver function tests have normalized and follow-up tests are unremarkable. |
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Inflammatory Myofibroblastic Tumour of the Common Bile DuctTumor Inflamatório Miofibroblástico da Via Biliar PrincipalBile Duct NeoplasmsHepatic DuctCommonHepatectomyKlatskin TumourLiver Function TestsDucto Hepático ComumHepatectomiaNeoplasias dos Ductos biliaresTestes de Função HepáticaTumor de KlatskinInflammatory myofibroblastic tumour is a rare entity of indeterminate biological potential with a reduced tendency for recurrence and metastasis. Although it can arise from multiple organs, the bile duct is a very rare site of origin. We report the case of a 75-year-old asymptomatic male with elevated gamma-glutamyl transferase [1575 U/L (12 - 64 U/L)] and alkaline phosphatase [271 U/L (40 - 150 U/L)]. Computed tomography showed a 17 mm hypervascular lesion in the confluence of the right and left hepatic ducts, with bile duct ectasia and right liver lobe atrophy. The patient was initially managed as having a Klatskin tumour and underwent right hepatectomy. Histology showed a spindle cell proliferation with an inflammatory infiltrate of lymphocytes, plasma cells and collagen-rich stroma, consistent with an inflammatory myofibroblastic tumour. He was discharged 30 days after admission, and nine months later remains asymptomatic. His liver function tests have normalized and follow-up tests are unremarkable.O tumor miofibroblástico inflamatório é uma entidade rara com comportamento biológico indeterminado e reduzido potencial de recorrência e metastização. Apesar de poder surgir em vários órgãos, as vias biliares constituem uma localização incomum. Descreve-se o caso de um homem de 75 anos, assintomático, com elevação da gama glutamil transferase [1575 U/L (12 - 64 U/L)] e fosfatase alcalina [271 U/L (40 - 150 U/L)], que realizou uma tomografia computorizada evidenciando uma lesão hipervascular com 17 mm na confluência dos ductos biliares, com ectasia das vias biliares intra-hepáticas à direita, e atrofia do lobo hepático correspondente. O caso foi abordado inicialmente como um tumor de Klatskin e realizada hepatectomia direita. A histologia relevou uma proliferação de células fusiformes com infiltrado inflamatório de linfócitos, plasmócitos e estroma rico em colágeno, compatível com tumor miofibroblástico inflamatório. Após nove meses, o doente permanece assintomático, com testes de função hepática com valores normais e sem sinais imagiológicos de recidiva.Ordem dos Médicos2022-02-07info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16976oai:ojs.www.actamedicaportuguesa.com:article/16976Acta Médica Portuguesa; Vol. 35 No. 10 (2022): October; 765-769Acta Médica Portuguesa; Vol. 35 N.º 10 (2022): Outubro; 765-7691646-07580870-399Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16976https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16976/6546Direitos de Autor (c) 2022 Acta Médica Portuguesahttp://creativecommons.org/licenses/by-nc/4.0info:eu-repo/semantics/openAccessAlmeida e Sousa, MatildeCarvalho, AnaMega, RaquelBilhim, Tiago2022-12-20T11:07:59Zoai:ojs.www.actamedicaportuguesa.com:article/16976Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:20:53.009614Repositório 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 |
Inflammatory Myofibroblastic Tumour of the Common Bile Duct Tumor Inflamatório Miofibroblástico da Via Biliar Principal |
title |
Inflammatory Myofibroblastic Tumour of the Common Bile Duct |
spellingShingle |
Inflammatory Myofibroblastic Tumour of the Common Bile Duct Almeida e Sousa, Matilde Bile Duct Neoplasms Hepatic Duct Common Hepatectomy Klatskin Tumour Liver Function Tests Ducto Hepático Comum Hepatectomia Neoplasias dos Ductos biliares Testes de Função Hepática Tumor de Klatskin |
title_short |
Inflammatory Myofibroblastic Tumour of the Common Bile Duct |
title_full |
Inflammatory Myofibroblastic Tumour of the Common Bile Duct |
title_fullStr |
Inflammatory Myofibroblastic Tumour of the Common Bile Duct |
title_full_unstemmed |
Inflammatory Myofibroblastic Tumour of the Common Bile Duct |
title_sort |
Inflammatory Myofibroblastic Tumour of the Common Bile Duct |
author |
Almeida e Sousa, Matilde |
author_facet |
Almeida e Sousa, Matilde Carvalho, Ana Mega, Raquel Bilhim, Tiago |
author_role |
author |
author2 |
Carvalho, Ana Mega, Raquel Bilhim, Tiago |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Almeida e Sousa, Matilde Carvalho, Ana Mega, Raquel Bilhim, Tiago |
dc.subject.por.fl_str_mv |
Bile Duct Neoplasms Hepatic Duct Common Hepatectomy Klatskin Tumour Liver Function Tests Ducto Hepático Comum Hepatectomia Neoplasias dos Ductos biliares Testes de Função Hepática Tumor de Klatskin |
topic |
Bile Duct Neoplasms Hepatic Duct Common Hepatectomy Klatskin Tumour Liver Function Tests Ducto Hepático Comum Hepatectomia Neoplasias dos Ductos biliares Testes de Função Hepática Tumor de Klatskin |
description |
Inflammatory myofibroblastic tumour is a rare entity of indeterminate biological potential with a reduced tendency for recurrence and metastasis. Although it can arise from multiple organs, the bile duct is a very rare site of origin. We report the case of a 75-year-old asymptomatic male with elevated gamma-glutamyl transferase [1575 U/L (12 - 64 U/L)] and alkaline phosphatase [271 U/L (40 - 150 U/L)]. Computed tomography showed a 17 mm hypervascular lesion in the confluence of the right and left hepatic ducts, with bile duct ectasia and right liver lobe atrophy. The patient was initially managed as having a Klatskin tumour and underwent right hepatectomy. Histology showed a spindle cell proliferation with an inflammatory infiltrate of lymphocytes, plasma cells and collagen-rich stroma, consistent with an inflammatory myofibroblastic tumour. He was discharged 30 days after admission, and nine months later remains asymptomatic. His liver function tests have normalized and follow-up tests are unremarkable. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-02-07 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16976 oai:ojs.www.actamedicaportuguesa.com:article/16976 |
url |
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16976 |
identifier_str_mv |
oai:ojs.www.actamedicaportuguesa.com:article/16976 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16976 https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16976/6546 |
dc.rights.driver.fl_str_mv |
Direitos de Autor (c) 2022 Acta Médica Portuguesa http://creativecommons.org/licenses/by-nc/4.0 info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
Direitos de Autor (c) 2022 Acta Médica Portuguesa http://creativecommons.org/licenses/by-nc/4.0 |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Ordem dos Médicos |
publisher.none.fl_str_mv |
Ordem dos Médicos |
dc.source.none.fl_str_mv |
Acta Médica Portuguesa; Vol. 35 No. 10 (2022): October; 765-769 Acta Médica Portuguesa; Vol. 35 N.º 10 (2022): Outubro; 765-769 1646-0758 0870-399X reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
instname_str |
Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1817551988485783552 |