Inflammatory Myofibroblastic Tumour of the Common Bile Duct

Detalhes bibliográficos
Autor(a) principal: Almeida e Sousa, Matilde
Data de Publicação: 2022
Outros Autores: Carvalho, Ana, Mega, Raquel, Bilhim, Tiago
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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spelling 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
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dc.type.driver.fl_str_mv info:eu-repo/semantics/article
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dc.identifier.uri.fl_str_mv https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/16976
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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
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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)
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reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
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