VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORT
Autor(a) principal: | |
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Data de Publicação: | 2023 |
Outros Autores: | , , , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) |
Texto Completo: | http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/986 |
Resumo: | Vena cava leiomyosarcoma is a rare neoplasm, usually divided into 3 types: Type I- below the renal vein, Type II- between the renal and hepatic veins and Type III - between hepatic veins and right atrium. In Type III venous drainage of the liver is compromised and may be the etiology of Budd Chiari Syndrome. Radiological imaging is very characteristic and may be the key to diagnosis. The aim of this case report is to present a 68-year-old patient, male, with severe abdominal pain associated with ascites, lower limb edema and portal hypertension compatible with Budd Chiari Syndrome. Tomography showed infiltrative image in the inferior vena cava pathway extending to the caudate lobe of the liver and histology showed low-grade leiomyosarcoma, confirmed by immunohistochemistry. Knowing the clinical and radiological picture of vena cava leiomyosarcoma allows doing the diagnosis and guiding the treatment and prognosis, reducing the economic and emotional costs. |
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VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORTLeiomiossarcoma de veia cava desenvolvendo síndrome de Budd-Chiari: relato de caso / Vena cava leiomyosarcoma developing Budd-Chiari Syndrome: a case report: RELATO DE CASO Leiomiosarcomaveia cava inferiorSíndrome de Budd Chiaridor abdominalneoplasia retroperitonealLeiomyosarcomainferior vena cavaBudd Chiari syndromeabdominal painretroperitoneal neoplasmsVena cava leiomyosarcoma is a rare neoplasm, usually divided into 3 types: Type I- below the renal vein, Type II- between the renal and hepatic veins and Type III - between hepatic veins and right atrium. In Type III venous drainage of the liver is compromised and may be the etiology of Budd Chiari Syndrome. Radiological imaging is very characteristic and may be the key to diagnosis. The aim of this case report is to present a 68-year-old patient, male, with severe abdominal pain associated with ascites, lower limb edema and portal hypertension compatible with Budd Chiari Syndrome. Tomography showed infiltrative image in the inferior vena cava pathway extending to the caudate lobe of the liver and histology showed low-grade leiomyosarcoma, confirmed by immunohistochemistry. Knowing the clinical and radiological picture of vena cava leiomyosarcoma allows doing the diagnosis and guiding the treatment and prognosis, reducing the economic and emotional costs.Introdução: O leiomiossarcoma de veia cava é uma neoplasia rara, dividida em três tipos: tipo I — abaixo da veia renal, tipo II — entre as veias renais e hepáticas e tipo III — entre veias hepáticas e átrio direito. No tipo III a drenagem venosa do fígado fica comprometida e pode ser a etiologia de síndrome de Budd-Chiari. A imagem radiológica é muito característica e pode ser a chave do diagnóstico. Objetivo: Relatar um caso de síndromede Budd-Chiari secundário a leiomiossarcoma de veia cava superior, discutindo os fatores envolvidos e a propedêutica adotada. Relato do caso: Paciente do sexo masculino, 68 anos, com dor abdominal de forte intensidade associado a ascite, edema de membros inferiores e hipertensão portal compatíveis com síndrome de Budd-Chiari. Tomografia evidenciou imagem infiltrativa no trajeto da veia cava inferior, com extensão paralobo caudado do fígado, e a histologia demonstrou leiomiossarcoma de baixo grau, confirmado por imuno-histoquímica. Conclusão: Conhecer o quadro clínico e radiológico de leiomiossarcoma de veia cava permite realizar o diagnóstico, orientar o tratamento e prognóstico, diminuindo os custos econômicos e emocionais.Palavras-chave: Leiomiossarcoma, Veia cava inferior, Síndrome de Budd-Chiari, Dor abdominal, Neoplasias retroperitoneais. ABSTRACT: Introduction: Vena cava leiomyosarcoma is a rare neoplasm, divided into three types: Type I — below the renal vein, Type II — between the renal and hepatic veins and Type III — between the hepatic veins and right atrium. In Type III, the venous drainage of the liver is compromised and may be the etiology of Budd-Chiari Syndrome. The radiological image is very characteristic and can be the key to diagnosis. Objective: To report a case of Budd-Chiari syndrome secondary to a leiomyosarcoma of the superior vena cava, discussing the factors involved and the work-up adopted. Case report: Male patient, 68 years old, with severe abdominal pain associated with ascites, lower limb edema and portal hypertension compatible with Budd-Chiari Syndrome. Tomography showed an infiltrative image in inferior vena cava extending to the caudate lobe of the liver and histology showed low-grade leiomyosarcoma, confirmed by immunohistochemistry. Conclusion: Knowing the clinical and radiological picture of vena cava leiomyosarcoma allows making the diagnosis, guiding the treatment and prognosis, reducing economic and emotional costs.Keywords: Leiomyosarcoma, Inferior vena cava, Budd-Chiari syndrome, Abdominal pain, Retroperitoneal neoplasms.Faculdade de Ciências Médicas da Santa Casa de São Paulo2023-10-24info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttp://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/98610.26432/1809-3019.2023.68.021Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo; V. 68 (2023): Jan/Dez; 1 of 51809-30190101-6067reponame:Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online)instname:Faculdade de Ciências Médicas da Santa Casa de São Pauloinstacron:FCMSCSPporhttp://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/986/1219Copyright (c) 2023 Mariana Brasileiro Vieira Puppo, Felício Stenio Schuenck Rozete, Rovena Campana Tardin, Paula Morette de Oliveira, Mateus Andre Rueda-Dantas, Layni Storch, Danielle Cristina Filgueira Alves Batista, Ludmilla Ventura Lirio, Kethleen Gomes Wandekoken, Caroline Alcure Pinto, João Eugênio Loureiro Lopes, Luciana Lofego Gonçalves, Maria da Penha Zago-Gomeshttps://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessPuppo, Mariana Brasileiro VieiraRozete, Felício Stenio SchuenckTardin, Rovena CampanaOliveira, Paula Morette de Rueda-Dantas, Mateus AndreStorch, LayniBatista, Danielle Cristina Filgueira AlvesLirio, Ludmilla VenturaWandekoken, Kethleen GomesPinto, Caroline AlcureLopes, João Eugênio LoureiroGonçalves, Luciana LofegoZago-Gomes, Maria da Penha2023-10-24T17:44:35Zoai:ojs2.arquivosmedicos.fcmsantacasasp.edu.br:article/986Revistahttp://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSPONGhttp://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/oaiarquivosmedicos@fcmsantacasasp.edu.br||1809-30190101-6067opendoar:2023-10-24T17:44:35Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) - Faculdade de Ciências Médicas da Santa Casa de São Paulofalse |
dc.title.none.fl_str_mv |
VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORT Leiomiossarcoma de veia cava desenvolvendo síndrome de Budd-Chiari: relato de caso / Vena cava leiomyosarcoma developing Budd-Chiari Syndrome: a case report: RELATO DE CASO |
title |
VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORT |
spellingShingle |
VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORT Puppo, Mariana Brasileiro Vieira Leiomiosarcoma veia cava inferior Síndrome de Budd Chiari dor abdominal neoplasia retroperitoneal Leiomyosarcoma inferior vena cava Budd Chiari syndrome abdominal pain retroperitoneal neoplasms |
title_short |
VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORT |
title_full |
VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORT |
title_fullStr |
VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORT |
title_full_unstemmed |
VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORT |
title_sort |
VENA CAVA LEIOMYOSARCOMA DEVELOPING BUDD CHIARI SYNDROME: CASE REPORT |
author |
Puppo, Mariana Brasileiro Vieira |
author_facet |
Puppo, Mariana Brasileiro Vieira Rozete, Felício Stenio Schuenck Tardin, Rovena Campana Oliveira, Paula Morette de Rueda-Dantas, Mateus Andre Storch, Layni Batista, Danielle Cristina Filgueira Alves Lirio, Ludmilla Ventura Wandekoken, Kethleen Gomes Pinto, Caroline Alcure Lopes, João Eugênio Loureiro Gonçalves, Luciana Lofego Zago-Gomes, Maria da Penha |
author_role |
author |
author2 |
Rozete, Felício Stenio Schuenck Tardin, Rovena Campana Oliveira, Paula Morette de Rueda-Dantas, Mateus Andre Storch, Layni Batista, Danielle Cristina Filgueira Alves Lirio, Ludmilla Ventura Wandekoken, Kethleen Gomes Pinto, Caroline Alcure Lopes, João Eugênio Loureiro Gonçalves, Luciana Lofego Zago-Gomes, Maria da Penha |
author2_role |
author author author author author author author author author author author author |
dc.contributor.author.fl_str_mv |
Puppo, Mariana Brasileiro Vieira Rozete, Felício Stenio Schuenck Tardin, Rovena Campana Oliveira, Paula Morette de Rueda-Dantas, Mateus Andre Storch, Layni Batista, Danielle Cristina Filgueira Alves Lirio, Ludmilla Ventura Wandekoken, Kethleen Gomes Pinto, Caroline Alcure Lopes, João Eugênio Loureiro Gonçalves, Luciana Lofego Zago-Gomes, Maria da Penha |
dc.subject.por.fl_str_mv |
Leiomiosarcoma veia cava inferior Síndrome de Budd Chiari dor abdominal neoplasia retroperitoneal Leiomyosarcoma inferior vena cava Budd Chiari syndrome abdominal pain retroperitoneal neoplasms |
topic |
Leiomiosarcoma veia cava inferior Síndrome de Budd Chiari dor abdominal neoplasia retroperitoneal Leiomyosarcoma inferior vena cava Budd Chiari syndrome abdominal pain retroperitoneal neoplasms |
description |
Vena cava leiomyosarcoma is a rare neoplasm, usually divided into 3 types: Type I- below the renal vein, Type II- between the renal and hepatic veins and Type III - between hepatic veins and right atrium. In Type III venous drainage of the liver is compromised and may be the etiology of Budd Chiari Syndrome. Radiological imaging is very characteristic and may be the key to diagnosis. The aim of this case report is to present a 68-year-old patient, male, with severe abdominal pain associated with ascites, lower limb edema and portal hypertension compatible with Budd Chiari Syndrome. Tomography showed infiltrative image in the inferior vena cava pathway extending to the caudate lobe of the liver and histology showed low-grade leiomyosarcoma, confirmed by immunohistochemistry. Knowing the clinical and radiological picture of vena cava leiomyosarcoma allows doing the diagnosis and guiding the treatment and prognosis, reducing the economic and emotional costs. |
publishDate |
2023 |
dc.date.none.fl_str_mv |
2023-10-24 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/986 10.26432/1809-3019.2023.68.021 |
url |
http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/986 |
identifier_str_mv |
10.26432/1809-3019.2023.68.021 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
http://arquivosmedicos.fcmsantacasasp.edu.br/index.php/AMSCSP/article/view/986/1219 |
dc.rights.driver.fl_str_mv |
https://creativecommons.org/licenses/by/4.0 info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
https://creativecommons.org/licenses/by/4.0 |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Faculdade de Ciências Médicas da Santa Casa de São Paulo |
publisher.none.fl_str_mv |
Faculdade de Ciências Médicas da Santa Casa de São Paulo |
dc.source.none.fl_str_mv |
Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo; V. 68 (2023): Jan/Dez; 1 of 5 1809-3019 0101-6067 reponame:Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) instname:Faculdade de Ciências Médicas da Santa Casa de São Paulo instacron:FCMSCSP |
instname_str |
Faculdade de Ciências Médicas da Santa Casa de São Paulo |
instacron_str |
FCMSCSP |
institution |
FCMSCSP |
reponame_str |
Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) |
collection |
Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) |
repository.name.fl_str_mv |
Arquivos Médicos dos Hospitais e da Faculdade de Ciências Médicas da Santa Casa de São Paulo (Online) - Faculdade de Ciências Médicas da Santa Casa de São Paulo |
repository.mail.fl_str_mv |
arquivosmedicos@fcmsantacasasp.edu.br|| |
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1831480502133981184 |